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Review article
Rare Tumors of the Rectum. Narrative Review
Neoplasias de recto poco frecuentes. Revisión de conjunto
José Errasti Alustizaa,
Corresponding author
, Eloy Espín Basanyb, Ángel Reina Duartec
a Unidad de Coloproctología, Servicio de Cirugía General, Hospital Universitario de Álava, Universidad del País Vasco, Vitoria-Gasteiz, Spain
b Unidad de Coloproctología, Servicio de Cirugía General, Hospital Universitario Valle de Hebron, Universidad Autónoma de Barcelona, Barcelona, Spain
c Unidad de Cirugía Colorrectal, Complejo Hospitalario Torrecárdenas, Almería, Spain
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We used PubMed to review the literature from 1997 to 2012 using the key words related to a colorectal location stated at the beginning of this article&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0030">Neuroendocrine Neoplasms</span><p id="par0020" class="elsevierStylePara elsevierViewall">Neuroendocrine neoplasms are epithelial and present neuroendocrine differentiation&#46;<a class="elsevierStyleCrossRef" href="#bib0045"><span class="elsevierStyleSup">9</span></a> They can be located in different organs&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">They are classified<a class="elsevierStyleCrossRefs" href="#bib0050"><span class="elsevierStyleSup">10&#44;11</span></a> by their degree of differentiation &#40;well or poorly differentiated&#41; and their histologic grade &#40;G1&#44; G2 and G3&#41;&#44; based on the number of mitoses and the Ki67 index&#46;<a class="elsevierStyleCrossRefs" href="#bib0045"><span class="elsevierStyleSup">9&#44;12&#8211;14</span></a> Three different groups are defined&#58; neuroendocrine tumors &#40;NET&#41;&#44; neuroendocrine carcinoma &#40;NEC&#41;&#44; and mixed adenoneuroendocrine carcinoma &#40;MANEC&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;12</span></a></p><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0035">Neuroendocrine Tumors</span><p id="par0030" class="elsevierStylePara elsevierViewall">NET are well-differentiated neuroendocrine neoplasms made up of tumor cells that express neuroendocrine markers &#40;chromogranin A&#44; synaptophysin&#41; &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; and hormones&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Cellular atypia and proliferative activity are low&#46; By definition&#44; they are grade G1 or G2 tumors&#46; This category includes lesions that were previously called &#8220;carcinoid tumors&#8221;&#44; a denomination that is now criticized and is no longer included in the gastrointestinal NET classifications&#44; but is still widely used&#46; The rectal location represents 18&#37; of all NET and 27&#37; of all digestive tract tumors&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;15</span></a></p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0035" class="elsevierStylePara elsevierViewall">The annual incidence of rectal NET&#44; according to the SEER&#44;<a class="elsevierStyleCrossRef" href="#bib0025"><span class="elsevierStyleSup">5</span></a> is 0&#46;86 per 100<span class="elsevierStyleHsp" style=""></span>000&#44; which has experienced a large increase in recent decades&#46;<a class="elsevierStyleCrossRef" href="#bib0035"><span class="elsevierStyleSup">7</span></a> The incidence is higher in Asians&#44;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> and mean age is 56&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a></p><p id="par0040" class="elsevierStylePara elsevierViewall">NET usually present as small polypoid lesions or submucosal nodules&#46;<a class="elsevierStyleCrossRef" href="#bib0020"><span class="elsevierStyleSup">4</span></a> 45&#37; measure 10<span class="elsevierStyleHsp" style=""></span>mm or less&#44; while only 17&#37; measure more than 20<span class="elsevierStyleHsp" style=""></span>mm&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> These tumors are frequently asymptomatic<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18</span></a> or accompanied by mild symptoms such as bleeding&#44; tenesmus or discomfort&#46;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">17&#44;18</span></a></p><p id="par0045" class="elsevierStylePara elsevierViewall">49&#37; of the NET only affect the mucosa and submucosa&#44; 24&#37; infiltrate the muscularis propria&#44; and 15&#37; extend to the perirectal fat&#46;<a class="elsevierStyleCrossRef" href="#bib0085"><span class="elsevierStyleSup">17</span></a> 75&#37;&#8211;85&#37; are located in the rectal wall&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Tumor size and lymphovascular invasions are risk factors for lymph node involvement&#46;<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">16&#44;17&#44;19&#44;20</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">Liver metastases become more frequent as tumor size increases&#46;<a class="elsevierStyleCrossRef" href="#bib0080"><span class="elsevierStyleSup">16</span></a> These are present in 1&#46;7&#37; of the NET&#8804;1<span class="elsevierStyleHsp" style=""></span>cm&#44; in 15&#37; of those between 1 and 2<span class="elsevierStyleHsp" style=""></span>cm and in 50&#37; of those &#62;2<span class="elsevierStyleHsp" style=""></span>cm&#46;<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">20</span></a></p><p id="par0055" class="elsevierStylePara elsevierViewall">The majority of NET are diagnosed endoscopically&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Endorectal ultrasound seems to be the best method for assessing the size and invasion depth of these tumors&#46;<a class="elsevierStyleCrossRefs" href="#bib0080"><span class="elsevierStyleSup">16&#44;18&#44;20&#44;21</span></a> In NET measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm and without risk factors&#44; no further studies are necessary&#46; MRI and CT are indicated in larger tumors in order to study the pelvis and to rule out liver metastasis&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> OctreoScan seems to have high sensitivity&#44; but it is not often used<a class="elsevierStyleCrossRef" href="#bib0100"><span class="elsevierStyleSup">20</span></a>&#59; it is mainly used in cases with suspected metastatic disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Less than 1&#37; of the colorectal NET produce serotonin or other hormones&#59; therefore&#44; the routine analysis of serotonin and 5-HIAA is not recommended&#46;<a class="elsevierStyleCrossRef" href="#bib0090"><span class="elsevierStyleSup">18</span></a> Chromogranin A may be elevated and is useful as a tumor marker in the follow-up of surgically treated patients in stages <span class="elsevierStyleSmallCaps">II</span>and <span class="elsevierStyleSmallCaps">III</span> or in metastatic disease&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;12&#44;18</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Tumor size predicts its behavior and the type of treatment necessary&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;16&#44;17</span></a> Other factors must also be taken into account&#44; such as invasion of the muscularis propria&#44; lymphovascular invasion&#44; atypia&#44; and mitotic rate&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;20&#44;22</span></a> The treatment of a localized NET is complete resection&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#8211;14</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">NET measuring less than 1<span class="elsevierStyleHsp" style=""></span>cm can be treated with local resection<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18&#44;19&#44;21</span></a> as they present a risk of less than 3&#37; for lymph node metastasis&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> Previously&#44; infiltration of the muscularis propria needs to be ruled out with ultrasound&#46;<a class="elsevierStyleCrossRef" href="#bib0115"><span class="elsevierStyleSup">23</span></a> Resection can be performed with standard &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41; or dual-channel endoscopic ultrasound&#44;<a class="elsevierStyleCrossRefs" href="#bib0110"><span class="elsevierStyleSup">22&#8211;27</span></a> through transanal surgery&#44;<a class="elsevierStyleCrossRefs" href="#bib0085"><span class="elsevierStyleSup">17&#44;21</span></a> or even with band ligation&#46;<a class="elsevierStyleCrossRef" href="#bib0140"><span class="elsevierStyleSup">28</span></a></p><p id="par0075" class="elsevierStylePara elsevierViewall">The treatment of NET from 1 to 2<span class="elsevierStyleHsp" style=""></span>cm in diameter is not clear because between 10&#37; and 15&#37; will have lymph node metastasis&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18</span></a> Local resection is indicated in cases where no involvement of the muscularis propria and lymph nodes has been detected by ultrasound<a class="elsevierStyleCrossRefs" href="#bib0090"><span class="elsevierStyleSup">18&#44;21</span></a> and the mitotic rate is low&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a> If atypia and high mitotic rate are identified&#44; radical surgery should be considered&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0080" class="elsevierStylePara elsevierViewall">Tumors that are more than 2<span class="elsevierStyleHsp" style=""></span>cm in size have a risk of between 60&#37; and 80&#37; for lymph node metastasis&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18</span></a> NET larger than 2<span class="elsevierStyleHsp" style=""></span>cm&#44; with invasion of the muscularis propria or lymph node involvement should be treated with either anterior resection of the rectum or abdominoperineal resection&#44; depending on the distance to the anal margin&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;18</span></a> There is no evidence for adjuvant treatment after surgery&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p><p id="par0085" class="elsevierStylePara elsevierViewall">Curative surgery should be proposed in patients with operable liver metastasis&#44; since 5-year survival reaches 60&#37;&#8211;80&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">29</span></a> Subsequent adjuvant treatment is not recommended&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">29</span></a> Liver transplantation can be carried out in selected cases in which surgical removal is not possible&#46;<a class="elsevierStyleCrossRef" href="#bib0145"><span class="elsevierStyleSup">29</span></a></p><p id="par0090" class="elsevierStylePara elsevierViewall">In metastatic disease&#44; long-acting release &#40;LAR&#41; octreotide and interferon-&#945; have been used&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;18&#44;21&#44;29</span></a> Recently&#44; radiotherapy has been applied with peptide receptor radionuclide therapy using somatostatin analogs&#44; providing responses of 30&#37; in patients with tumors that express somatostatin receptors&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;18</span></a> On rare occasions&#44; chemotherapy is indicated in NET G1 or G2&#46; When used due to disease progression&#44; streptozotocin is most often administered in combination with 5-fluorouracil&#177;doxorubicin&#44; but the response is less than 25&#37;&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;12</span></a></p><p id="par0095" class="elsevierStylePara elsevierViewall">Five-year survival is 91&#37; in localized disease&#44; 49&#37; in regional disease and 32&#37; in metastatic disease&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p><p id="par0100" class="elsevierStylePara elsevierViewall">Follow-up is not necessary in NET&#60;1<span class="elsevierStyleHsp" style=""></span>cm that present with no other data for poor prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0065"><span class="elsevierStyleSup">13</span></a> In the remainder&#44; follow-up includes endorectal ultrasound&#44; rectoscopy&#44; MRI or CT&#44; and chromogranin A<span class="elsevierStyleSup">13</span> for 10 years&#46;<a class="elsevierStyleCrossRef" href="#bib0060"><span class="elsevierStyleSup">12</span></a></p></span><span id="sec0020" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0040">Neuroendocrine Carcinomas and Mixed Adenoneuroendocrine Carcinomas</span><p id="par0105" class="elsevierStylePara elsevierViewall">NEC are poorly differentiated high-grade malignant neoplasms of tumor cells that express neuroendocrine markers &#40;chromogranin A&#44; synaptophysin&#41; and have marked cellular atypia&#44; frequent necrosis and high proliferative activity&#46;<a class="elsevierStyleCrossRefs" href="#bib0060"><span class="elsevierStyleSup">12&#44;30</span></a> NEC and MANEC are G3 tumors by definition&#46; There are 2 categories of NEC&#58; small-cell and large-cell&#46; Due to their histologic characteristics&#44; they behave much more aggressively than NET&#46;<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">31&#44;32</span></a></p><p id="par0110" class="elsevierStylePara elsevierViewall">The annual incidence of these colorectal carcinomas is 2 cases per 1<span class="elsevierStyleHsp" style=""></span>000<span class="elsevierStyleHsp" style=""></span>000 inhabitants&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a> Symptoms are similar to those of rectal adenocarcinomas&#44; but they differ because many have already metastasized at diagnosis &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>a and b&#41; and have a poorer prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0160"><span class="elsevierStyleSup">32</span></a> Mean survival is 11 months&#46;<a class="elsevierStyleCrossRefs" href="#bib0155"><span class="elsevierStyleSup">31&#44;33</span></a></p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0115" class="elsevierStylePara elsevierViewall">In NEC&#44; chromogranin A is usually negative&#44; but neuron-specific enolase can be used as a marker&#46;<a class="elsevierStyleCrossRef" href="#bib0075"><span class="elsevierStyleSup">15</span></a></p><p id="par0120" class="elsevierStylePara elsevierViewall">There is no standardized treatment&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> The usual treatment is surgical&#58; amputation or anterior resection depending on the location&#44; with total mesorectal excision&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> Nonetheless&#44; it seems that surgery alone is curative on few occasions&#44; so adjuvant chemotherapy is recommended in most cases&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> The same chemotherapy is used as in neuroendocrine lung cancer&#44; which is a combination of cisplatin or carboplatin and ectoposide&#46;<a class="elsevierStyleCrossRefs" href="#bib0035"><span class="elsevierStyleSup">7&#44;29&#44;30</span></a> Radiotherapy can be indicated in cases at risk for local recurrence&#46;<a class="elsevierStyleCrossRef" href="#bib0150"><span class="elsevierStyleSup">30</span></a> There have been cases described with good evolution after chemotherapy alone&#44; without surgery&#46;<a class="elsevierStyleCrossRef" href="#bib0170"><span class="elsevierStyleSup">34</span></a></p></span></span><span id="sec0025" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0045">Sarcomas</span><p id="par0125" class="elsevierStylePara elsevierViewall">Up until the 1990s&#44; most intestinal mesenchymal tumors were leiomyomas or leiomyosarcomas&#44; but with the development of immunohistochemistry techniques it was seen that the majority of these tumors belonged to a different group&#58; gastrointestinal stromal tumor &#40;GIST&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0175"><span class="elsevierStyleSup">35&#44;36</span></a></p><span id="sec0030" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0050">Gastrointestinal Stromal Tumors</span><p id="par0130" class="elsevierStylePara elsevierViewall">GIST originate in the interstitial cells of Cajal&#44; which are intestinal pacemakers&#46; They are characterized by having specific markers&#44; such as CD117 &#40;c-KIT&#41; in more than 95&#37; and CD34 in 70&#37;&#44; which differentiate them from leiomyomas and leiomyosarcomas&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">37</span></a> The annual incidence is estimated at 1&#46;5 per 100<span class="elsevierStyleHsp" style=""></span>000&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> The most common locations are the stomach and small bowel&#59; however&#44; 10&#37; of GIST are situated in the rectum&#46;<a class="elsevierStyleCrossRef" href="#bib0175"><span class="elsevierStyleSup">35</span></a></p><p id="par0135" class="elsevierStylePara elsevierViewall">GIST are submucosal tumors&#44; most of which measure between 4 and 15<span class="elsevierStyleHsp" style=""></span>cm&#46; They can present central necrosis and ulcerate to the rectal lumen&#46; The most frequent symptoms are tenesmus and rectal bleeding&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">38</span></a></p><p id="par0140" class="elsevierStylePara elsevierViewall">Diagnosis is reached with CT&#44; MRI&#44; and endorectal ultrasound&#46; Rectal GIST are visualized as eccentric masses with well-defined tumor margins &#40;<a class="elsevierStyleCrossRef" href="#fig0015">Fig&#46; 3</a>&#41;&#44; which can have areas of hemorrhage or necrosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">39&#44;40</span></a> MRI seems to be the preferred test for rectal localization&#46;<a class="elsevierStyleCrossRef" href="#bib0205"><span class="elsevierStyleSup">41</span></a> Ultrasound is able to confirm that the tumor originates in the muscle wall and not in the mucosa&#46;<a class="elsevierStyleCrossRef" href="#bib0200"><span class="elsevierStyleSup">40</span></a> The histologic diagnosis can be difficult with unaffected mucosa&#46;<a class="elsevierStyleCrossRef" href="#bib0190"><span class="elsevierStyleSup">38</span></a> In cases of extensive or risky surgeries&#44; or in cases of doubtful diagnoses with processes that require other treatments&#44; endoscopic ultrasound biopsy is recommended&#44; which has a success rate of 80&#37;&#8211;90&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0210"><span class="elsevierStyleSup">42</span></a></p><elsevierMultimedia ident="fig0015"></elsevierMultimedia><p id="par0145" class="elsevierStylePara elsevierViewall">Standard treatment of localized GIST is surgical resection with free margins&#44;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;36&#44;43&#44;44</span></a> and&#44; as lymph node metastases are infrequent&#44;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;37</span></a> lymph node dissection is not required if the nodes are clinically negative&#46; These tumors have a pseudocapsule and are friable&#44; so they should be manipulated with care to avoid rupture&#44;<a class="elsevierStyleCrossRefs" href="#bib0185"><span class="elsevierStyleSup">37&#44;42&#44;43</span></a> which would worsen the prognosis&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">45</span></a></p><p id="par0150" class="elsevierStylePara elsevierViewall">In the rectum&#44; the size and location of the GIST determine the type of surgery&#46; Small tumors can be treated with local resection&#44; involving either the abdominal or transanal approaches&#46;<a class="elsevierStyleCrossRef" href="#bib0230"><span class="elsevierStyleSup">46</span></a> There should be no tumor cells in the resection margins&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> It is not clear whether asymptomatic tumors &#60;1<span class="elsevierStyleHsp" style=""></span>cm should be removed or have strict follow-up and only be resected if they increase in size&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;42</span></a> Large tumors&#44; which are generally larger than 5<span class="elsevierStyleHsp" style=""></span>cm&#44; are usually treated with anterior resection and without the need for mesorectal excision&#44; or by means of abdominoperineal resection&#46;<a class="elsevierStyleCrossRefs" href="#bib0190"><span class="elsevierStyleSup">38&#44;44&#44;47&#44;48</span></a> There have also been reports of large tumors removed using the transacral<a class="elsevierStyleCrossRefs" href="#bib0245"><span class="elsevierStyleSup">49&#44;50</span></a> or transvaginal<a class="elsevierStyleCrossRef" href="#bib0255"><span class="elsevierStyleSup">51</span></a> approaches&#46;</p><p id="par0155" class="elsevierStylePara elsevierViewall">The most important prognostic factors are tumor size&#44; number of mitoses&#44; and their location&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;36&#44;43&#44;52&#44;53</span></a> Rectal tumors have a poorer prognosis than gastric lesions&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;45</span></a> Furthermore&#44; tumor rupture is another adverse factor&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">45</span></a></p><p id="par0160" class="elsevierStylePara elsevierViewall">GIST are resistant to chemotherapy but sensitive to imatinib&#44; a tyrosine-kinase inhibitor that has demonstrated important clinical benefits in patients with advanced disease or recurrence&#46;<a class="elsevierStyleCrossRef" href="#bib0185"><span class="elsevierStyleSup">37</span></a> It is also effective for increasing survival in patients at risk in an adjuvant treatment&#46;<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">54&#8211;56</span></a></p><p id="par0165" class="elsevierStylePara elsevierViewall">Disease-free survival after 5&#44; 10&#44; and 15 years is 70&#37;&#44; 63&#37;&#44; and 60&#37;&#44; respectively&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">45</span></a> Adjuvant therapy is indicated in patients at risk for recurrence&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;43</span></a> In rectal GIST&#62;5<span class="elsevierStyleHsp" style=""></span>cm with any number of mitoses&#47;50 fields&#44; in those of any size with &#62;5<span class="elsevierStyleHsp" style=""></span>mitoses&#47;50 fields or in those with rupture&#44;<a class="elsevierStyleCrossRefs" href="#bib0225"><span class="elsevierStyleSup">45&#44;57</span></a> the recommended dose is 400<span class="elsevierStyleHsp" style=""></span>mg&#47;day for 3 years&#46;<a class="elsevierStyleCrossRefs" href="#bib0270"><span class="elsevierStyleSup">54&#44;58</span></a></p><p id="par0170" class="elsevierStylePara elsevierViewall">Given the good response of GIST to imatinib&#44; it is being used as neoadjuvant treatment to be able to resect initially unresectable tumors and to avoid abdominoperineal resection in large distal tumors&#46;<a class="elsevierStyleCrossRefs" href="#bib0185"><span class="elsevierStyleSup">37&#44;42&#44;59&#8211;62</span></a> After neoadjuvant therapy decreases tumor size and increases resectability&#44;<a class="elsevierStyleCrossRefs" href="#bib0300"><span class="elsevierStyleSup">60&#44;62&#8211;64</span></a> there may even be a complete response&#46;<a class="elsevierStyleCrossRefs" href="#bib0310"><span class="elsevierStyleSup">62&#44;63&#44;65</span></a> These indications are not supported by randomized studies and are based on short series or isolated cases&#46; A recent multicenter study<a class="elsevierStyleCrossRef" href="#bib0220"><span class="elsevierStyleSup">44</span></a> confirmed that neoadjuvant treatment reduces tumor size and increases resectability&#44; but it does not avoid mutilating surgery&#59; it also concluded that surgery continues to be the treatment of choice in primary resectable GIST&#46; Before neoadjuvant therapy&#44; there should be histologic confirmation&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">66</span></a> The optimal duration of preoperative treatment is unknown&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">63&#44;66&#44;67</span></a> For some&#44; maximum tumor response is achieved after 3&#8211;6 months of treatment&#44;<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">40&#44;68</span></a> while others consider 6&#8211;12 months reasonable&#46;<a class="elsevierStyleCrossRef" href="#bib0315"><span class="elsevierStyleSup">63</span></a> Treatment should be maintained until the maximum response is reached&#44; defined by non-improvement between 2 CT or MRI studies&#46;<a class="elsevierStyleCrossRefs" href="#bib0315"><span class="elsevierStyleSup">63&#44;66&#44;67</span></a> The use of PET can predict the response to treatment 2 weeks after initiation since functional results become evident before morphological results&#46;<a class="elsevierStyleCrossRef" href="#bib0330"><span class="elsevierStyleSup">66</span></a></p><p id="par0175" class="elsevierStylePara elsevierViewall">For patients with inoperable metastatic tumors&#44; the standard treatment is imatinib&#46;<a class="elsevierStyleCrossRefs" href="#bib0200"><span class="elsevierStyleSup">40&#44;41&#44;68</span></a> Treatment should be continued indefinitely because its interruption is generally accompanied by rapid tumor progression&#46;<a class="elsevierStyleCrossRefs" href="#bib0030"><span class="elsevierStyleSup">6&#44;68</span></a> In cases of progression during treatment with imatinib&#44; other substances can be used such as second-line sunitinib or third-line regorafenib&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a></p><p id="par0180" class="elsevierStylePara elsevierViewall">There are no data to recommend a follow-up protocol in patients operated on due to localized GIST&#44; but it seems logical for the follow-up to be done in line with patient risk&#46;<a class="elsevierStyleCrossRef" href="#bib0030"><span class="elsevierStyleSup">6</span></a> Most recurrences arise within the first 5 years and rarely do so after 10 years&#46;<a class="elsevierStyleCrossRef" href="#bib0225"><span class="elsevierStyleSup">45</span></a></p></span><span id="sec0035" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0055">Other Sarcomas</span><p id="par0185" class="elsevierStylePara elsevierViewall">Soft tissue sarcomas and sarcomas of other organs &#40;excluding GIST&#41; have an estimated annual incidence of 4&#8211;5 per 100<span class="elsevierStyleHsp" style=""></span>000&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> Rectal sarcomas are very rare since gastrointestinal sarcomas represent 2&#46;6&#37; of all sarcomas and&#44; amongst these&#44; only 15&#37; are colorectal&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">69</span></a> There are numerous histologic subtypes as they are classified according to the cells of origin of the tissue&#46; In the rectum&#44; the most frequent type is leiomyosarcoma&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">69</span></a> The histologic grade of malignancy &#40;G1&#44; G2 or G3&#41; is determined by 3 parameters&#58; differentiation&#44; tumor necrosis&#44; and the number of mitoses&#46;<a class="elsevierStyleCrossRefs" href="#bib0040"><span class="elsevierStyleSup">8&#44;70</span></a></p><p id="par0190" class="elsevierStylePara elsevierViewall">There is no clear etiology for these tumors&#44; but an increased risk for the appearance of sarcomas after ionizing radiation has already been identified&#46; They generally appear 7&#8211;10 years after radiotherapy&#46;<a class="elsevierStyleCrossRef" href="#bib0355"><span class="elsevierStyleSup">71</span></a> Leiomyosarcomas and angiosarcomas have been detected after pelvic radiotherapy&#46;<a class="elsevierStyleCrossRefs" href="#bib0360"><span class="elsevierStyleSup">72&#8211;75</span></a> Furthermore&#44; Kaposi&#39;s sarcoma has been associated with AIDS&#46;<a class="elsevierStyleCrossRef" href="#bib0380"><span class="elsevierStyleSup">76</span></a></p><p id="par0195" class="elsevierStylePara elsevierViewall">Leiomyosarcomas have a different origin than GIST&#58; they derive from the cells of the muscularis mucosae or the muscular propria&#46;<a class="elsevierStyleCrossRefs" href="#bib0180"><span class="elsevierStyleSup">36&#44;47</span></a> Immunohistochemistry studies show their positivity to smooth-muscle actin and desmin and negativity to CD117 and CD34&#44; which differentiates them from GIST&#46;<a class="elsevierStyleCrossRefs" href="#bib0235"><span class="elsevierStyleSup">47&#44;70</span></a> They frequently present as polypoid lesions measuring between 2 and 5<span class="elsevierStyleHsp" style=""></span>cm<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">47</span></a> &#40;<a class="elsevierStyleCrossRef" href="#fig0020">Fig&#46; 4</a>&#41;&#46; They are usually well-differentiated tumors with high mitotic activity&#44; but it seems that their prognosis can be better than GIST with a similar number of mitosis&#46;<a class="elsevierStyleCrossRef" href="#bib0235"><span class="elsevierStyleSup">47</span></a></p><elsevierMultimedia ident="fig0020"></elsevierMultimedia><p id="par0200" class="elsevierStylePara elsevierViewall">The histologic grade&#44; size&#44; and invasion of neighboring organs determine the prognosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0345"><span class="elsevierStyleSup">69&#44;70</span></a></p><p id="par0205" class="elsevierStylePara elsevierViewall">Surgical resection with free margins is the treatment of choice&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">69</span></a> Lymph node metastases are uncommon&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> It is not clear whether local resection is sufficient for small low-grade tumors&#46;<a class="elsevierStyleCrossRef" href="#bib0345"><span class="elsevierStyleSup">69</span></a> The indication for anterior resection or abdominoperineal resection is done according to the size and location of the tumor&#46; Radiotherapy can have results similar to those of the treatment of sarcomas of the extremities and it is recommended in high-grade tumors and those larger than 5<span class="elsevierStyleHsp" style=""></span>cm&#46;<a class="elsevierStyleCrossRef" href="#bib0385"><span class="elsevierStyleSup">77</span></a></p><p id="par0210" class="elsevierStylePara elsevierViewall">Recurrences can present as liver&#44; lung or local pelvic metastases&#46; Depending on their characteristics&#44; treatment may include surgical resection&#44; ablation&#44; radiotherapy or chemotherapy&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a> As there is no specific marker&#44; follow-up should be done with imaging tests&#46;<a class="elsevierStyleCrossRef" href="#bib0040"><span class="elsevierStyleSup">8</span></a></p></span></span><span id="sec0040" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0060">Lymphomas</span><p id="par0215" class="elsevierStylePara elsevierViewall">Gastrointestinal lymphomas are rare&#44; but the digestive tract is the most frequent non-lymph node location of non-Hodgkin lymphomas &#40;NHL&#41;&#46; Colorectal involvement is rarer than gastric or small bowel locations&#46; This lymphoma is considered primary when there is no systemic involvement&#44; meaning that there are no peripheral lymphadenopathies&#44; no mediastinal lymph node involvement&#44; normal peripheral blood and bone marrow biopsy studies&#44; lymphadenopathies located only in the proximity of the lesion&#44; and no involvement of the liver or spleen&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">78&#44;79</span></a></p><p id="par0220" class="elsevierStylePara elsevierViewall">Secondary rectal lymphoma is a generalized process with rectal involvement due to lymph node metastasis&#46; The differentiation between primary and secondary lymphoma is important because treatment and prognosis are different for each&#46; The treatment of secondary lymphoma is chemotherapy and five-year survival is 15&#37;&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0225" class="elsevierStylePara elsevierViewall">In the rectum&#44; all histological lymphoma subtypes may be present&#44; but the majority of primary lymphomas are B-cell NHL&#44;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#8211;81</span></a> with their different variations&#58; large B-cell&#44; mantle-cell&#44; follicular&#44; Burkitt&#44; and mucosa-associated lymphoid tissue &#40;MALT&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">78&#44;82&#44;83</span></a> The proportion of these different subtypes varies according to geographical region&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">79</span></a> T-cell NHL are more frequent in Asia than in Western countries&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;80</span></a></p><p id="par0230" class="elsevierStylePara elsevierViewall">Different factors have been involved in the genesis of gastrointestinal lymphomas&#59; these are generally associated with immunosuppression&#44; such as HIV infection&#44; inflammatory bowel disease&#44; organ transplantation or treatment with corticosteroids&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;80</span></a> They have also been related with infectious agents&#44; such as <span class="elsevierStyleItalic">Helicobacter pylori</span> and others&#46;<a class="elsevierStyleCrossRef" href="#bib0400"><span class="elsevierStyleSup">80</span></a></p><p id="par0235" class="elsevierStylePara elsevierViewall">Mean age at diagnosis is 55&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">78&#44;79</span></a> The most frequent symptoms are abdominal pain&#44; weight loss&#44; change in bowel habits&#44; and rectal bleeding&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;81</span></a></p><p id="par0240" class="elsevierStylePara elsevierViewall">Although in the colon it presents as a polypoid lesion &#40;possibly ulcerated&#41;&#44; stenosing mass&#44; segmental polyposis&#44; or nodularity of the mucosa&#44;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;80&#44;83</span></a> the most typical rectal presentation is a homogenous mass &#40;due to the concentric wall thickening&#41; with luminal stenosis&#46;<a class="elsevierStyleCrossRefs" href="#bib0195"><span class="elsevierStyleSup">39&#44;84</span></a> Suspicion of lymphoma is due the existence of large&#44; numerous lymphadenopathies&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">79</span></a> Ultrasound biopsy with immunohistochemistry provides the diagnosis&#44; although in many cases precise preoperative diagnosis can be difficult&#46;<a class="elsevierStyleCrossRefs" href="#bib0405"><span class="elsevierStyleSup">81&#44;85&#8211;87</span></a></p><p id="par0245" class="elsevierStylePara elsevierViewall">Due to the small number of patients and the various histological subtypes&#44; there is no standardized treatment for colorectal lymphomas&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;88</span></a> A combination of surgery and chemotherapy can be used&#44; reserving radiotherapy for certain cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0410"><span class="elsevierStyleSup">82&#44;83</span></a></p><p id="par0250" class="elsevierStylePara elsevierViewall">Oncologic surgical resection is the most common treatment for localized lymphomas<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">78&#44;79&#44;81&#8211;83</span></a> as it offers the possibility of a cure without adjuvant treatment and prevents complications such as bleeding&#44; obstruction or perforation&#46;<a class="elsevierStyleCrossRefs" href="#bib0410"><span class="elsevierStyleSup">82&#44;85</span></a></p><p id="par0255" class="elsevierStylePara elsevierViewall">Chemotherapy as an initial treatment is usually reserved for patients with locally advanced tumors<a class="elsevierStyleCrossRef" href="#bib0405"><span class="elsevierStyleSup">81</span></a> or disseminated disease&#46;<a class="elsevierStyleCrossRef" href="#bib0415"><span class="elsevierStyleSup">83</span></a> Adjuvant chemotherapy after surgery is recommended in aggressive lymphomas or advanced stages&#46;<a class="elsevierStyleCrossRefs" href="#bib0395"><span class="elsevierStyleSup">79&#44;85</span></a> The most widely used chemotherapy regime is CHOP &#40;cyclophosphamide&#44; doxorubicin&#44; vincristine&#44; and prednisone&#41;&#46;<a class="elsevierStyleCrossRefs" href="#bib0390"><span class="elsevierStyleSup">78&#44;82&#44;83&#44;85&#44;89</span></a> Results improve when an anti-CD 20 monoclonal antibody&#44; rituximab&#44; is added to this classic protocol &#40;R-CHOP&#41;&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">79</span></a></p><p id="par0260" class="elsevierStylePara elsevierViewall">Adjuvant radiotherapy could play a role in locoregional control after incomplete resection<a class="elsevierStyleCrossRefs" href="#bib0410"><span class="elsevierStyleSup">82&#44;83</span></a> or in cases of chemo-resistant tumors&#46;<a class="elsevierStyleCrossRef" href="#bib0395"><span class="elsevierStyleSup">79</span></a></p><p id="par0265" class="elsevierStylePara elsevierViewall">Rare rectal MALT lymphomas have been treated in many different ways&#46; In some <span class="elsevierStyleItalic">H&#46; pylori</span>-positive MALT&#44; antibiotic therapy has been used successfully alone<a class="elsevierStyleCrossRefs" href="#bib0450"><span class="elsevierStyleSup">90&#44;91</span></a>&#59; in other cases&#44; radiotherapy&#44; chemotherapy or surgery has been used&#46;<a class="elsevierStyleCrossRef" href="#bib0460"><span class="elsevierStyleSup">92</span></a></p><p id="par0270" class="elsevierStylePara elsevierViewall">Five-year survival of colorectal lymphomas is between 25&#37; and 57&#37;&#44; which is worse than gastric and small intestine lymphomas&#46;<a class="elsevierStyleCrossRef" href="#bib0410"><span class="elsevierStyleSup">82</span></a></p></span><span id="sec0045" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0065">Squamous-Cell Carcinoma</span><p id="par0275" class="elsevierStylePara elsevierViewall">Squamous-cell carcinoma is an uncommon tumor that is usually found in the esophagus and anus&#46; Rectal involvement is very rare&#46; In many cases&#44; supposed rectal squamous-cell carcinomas are&#44; in reality&#44; an extension of an anal carcinoma&#46;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">93</span></a> Occasionally&#44; they have a mixed histologic pattern and are called adenosquamous carcinoma&#46;<a class="elsevierStyleCrossRef" href="#bib0470"><span class="elsevierStyleSup">94</span></a></p><p id="par0280" class="elsevierStylePara elsevierViewall">Mean age is 57 years and this pathology is somewhat more frequent in women than in men&#46;<a class="elsevierStyleCrossRefs" href="#bib0465"><span class="elsevierStyleSup">93&#44;95</span></a> There are no clear risk factors&#44; but there has been an association with inflammatory diseases of the rectum&#44; human papilloma virus &#40;HPV&#41;&#44; and colorectal adenocarcinoma&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a></p><p id="par0285" class="elsevierStylePara elsevierViewall">Symptoms are similar to those of rectal adenocarcinoma&#44; and the most frequent is rectal bleeding&#46;<a class="elsevierStyleCrossRefs" href="#bib0465"><span class="elsevierStyleSup">93&#44;95</span></a> Endoscopic findings can be varied&#44; from a polypoid formation up to an ulcerative and stenosing tumor<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a>&#59; biopsy provides the diagnosis&#46; Occasionally&#44; there can be difficulties in distinguishing it from a poorly differentiated tumor&#44; but immunohistochemistry can define the lesion&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a> The most useful cytokeratins are CAM 5&#46;2&#44; AE1&#47;AE3&#44; and 34B12&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a></p><p id="par0290" class="elsevierStylePara elsevierViewall">For staging&#44; MRI &#40;<a class="elsevierStyleCrossRef" href="#fig0025">Fig&#46; 5</a>&#41;&#44; CT and endorectal ultrasound are used&#46; Squamous-cell carcinoma antigen is a tumor marker that is high in some patients&#46; It is not used for diagnosis but can be used for monitoring response or progression&#46;<a class="elsevierStyleCrossRefs" href="#bib0475"><span class="elsevierStyleSup">95&#44;96</span></a></p><elsevierMultimedia ident="fig0025"></elsevierMultimedia><p id="par0295" class="elsevierStylePara elsevierViewall">Traditionally&#44; the usual treatment has been surgery&#44; followed by adjuvant radiotherapy or chemotherapy in some cases&#46;<a class="elsevierStyleCrossRefs" href="#bib0485"><span class="elsevierStyleSup">97&#44;98</span></a> In the last decade&#44; given the good results of chemoradiotherapy in squamous-cell carcinoma of the anus&#44; this traditional approach has been questioned<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">99</span></a> and&#44; even though a standard treatment has not been established&#44;<a class="elsevierStyleCrossRef" href="#bib0465"><span class="elsevierStyleSup">93</span></a> there has been a tendency to modify this approach&#44; making chemotherapy the initial treatment for squamous-cell carcinoma of the rectum&#44; and reserving surgery for persistent tumors after treatment&#46;<a class="elsevierStyleCrossRefs" href="#bib0465"><span class="elsevierStyleSup">93&#44;96&#44;100&#44;101</span></a> The regimes used are the same that have been shown to be effective in squamous carcinoma of the anus&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a> Treatment entails a combination of mitomycin-C with 5-fluorouracil and radiotherapy at a minimum dose of 45&#8211;50<span class="elsevierStyleHsp" style=""></span>Gy&#46;<a class="elsevierStyleCrossRefs" href="#bib0510"><span class="elsevierStyleSup">102&#8211;104</span></a> The response to chemoradiotherapy is assessed 6&#8211;8 weeks after the end of treatment using rectoscopy with biopsy&#44; MRI or PET&#46;<a class="elsevierStyleCrossRefs" href="#bib0465"><span class="elsevierStyleSup">93&#44;99&#8211;101</span></a> If there is complete clinical and radiological response&#44; periodical follow-up is performed&#59; cases of tumor persistence should be re-evaluated after 4&#8211;6 weeks&#44; as recommended in anal cancer&#46;<a class="elsevierStyleCrossRef" href="#bib0520"><span class="elsevierStyleSup">104</span></a> Rescue surgery may be necessary&#44; which entails anterior resection or amputation&#44; depending on the tumor and patient characteristics&#46;<a class="elsevierStyleCrossRef" href="#bib0485"><span class="elsevierStyleSup">97</span></a></p><p id="par0300" class="elsevierStylePara elsevierViewall">There are few published series in which the initial treatment was chemoradiotherapy and they include few cases&#44; but between 66&#37; and 100&#37; showed complete response and did not require later surgery&#46;<a class="elsevierStyleCrossRefs" href="#bib0465"><span class="elsevierStyleSup">93&#44;96&#44;99&#8211;101</span></a> After complete response&#44; clinical follow-up should include rectal biopsies and radiological studies&#44; which become progressively spaced out over time&#46;<a class="elsevierStyleCrossRef" href="#bib0495"><span class="elsevierStyleSup">99</span></a></p><p id="par0305" class="elsevierStylePara elsevierViewall">Five-year survival is 50&#37; in stage <span class="elsevierStyleSmallCaps">II</span> and drops to 33&#37; when there is lymph node involvement&#46;<a class="elsevierStyleCrossRef" href="#bib0475"><span class="elsevierStyleSup">95</span></a></p></span><span id="sec0050" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0070">Conclusions</span><p id="par0310" class="elsevierStylePara elsevierViewall">The rarity of these tumors and their heterogenous origin&#44; treatment&#44; and prognosis mean that physicians may have difficulties in the management of these patients&#46; A multidisciplinary approach including pathologists&#44; radiologists&#44; oncologists&#44; radiotherapists&#44; and surgeons is recommended&#46;</p></span><span id="sec0055" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0075">Conflict of Interest</span><p id="par0315" class="elsevierStylePara elsevierViewall">The authors declare having no conflict of interests&#46;</p></span></span>"
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          "identificador" => "xres376563"
          "titulo" => "Abstract"
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          "titulo" => "Keywords"
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          "identificador" => "xres376562"
          "titulo" => "Resumen"
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        3 => array:2 [
          "identificador" => "xpalclavsec355685"
          "titulo" => "Palabras clave"
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        4 => array:2 [
          "identificador" => "sec0005"
          "titulo" => "Introduction"
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        5 => array:3 [
          "identificador" => "sec0010"
          "titulo" => "Neuroendocrine Neoplasms"
          "secciones" => array:2 [
            0 => array:2 [
              "identificador" => "sec0015"
              "titulo" => "Neuroendocrine Tumors"
            ]
            1 => array:2 [
              "identificador" => "sec0020"
              "titulo" => "Neuroendocrine Carcinomas and Mixed Adenoneuroendocrine Carcinomas"
            ]
          ]
        ]
        6 => array:3 [
          "identificador" => "sec0025"
          "titulo" => "Sarcomas"
          "secciones" => array:2 [
            0 => array:2 [
              "identificador" => "sec0030"
              "titulo" => "Gastrointestinal Stromal Tumors"
            ]
            1 => array:2 [
              "identificador" => "sec0035"
              "titulo" => "Other Sarcomas"
            ]
          ]
        ]
        7 => array:2 [
          "identificador" => "sec0040"
          "titulo" => "Lymphomas"
        ]
        8 => array:2 [
          "identificador" => "sec0045"
          "titulo" => "Squamous-Cell Carcinoma"
        ]
        9 => array:2 [
          "identificador" => "sec0050"
          "titulo" => "Conclusions"
        ]
        10 => array:2 [
          "identificador" => "sec0055"
          "titulo" => "Conflict of Interest"
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        11 => array:1 [
          "titulo" => "References"
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    ]
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    "fechaRecibido" => "2013-03-05"
    "fechaAceptado" => "2013-06-02"
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        0 => array:4 [
          "clase" => "keyword"
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            0 => "Rectal neoplasms"
            1 => "Lymphoma"
            2 => "Gastrointestinal stromal tumors"
            3 => "Sarcoma"
            4 => "Neuroendocrine tumors"
            5 => "Carcinoid tumor"
            6 => "Squamous cell carcinoma"
            7 => "Review literature"
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          "clase" => "keyword"
          "titulo" => "Palabras clave"
          "identificador" => "xpalclavsec355685"
          "palabras" => array:8 [
            0 => "C&#225;ncer de recto"
            1 => "Linfoma"
            2 => "Tumor del estroma gastrointestinal"
            3 => "Sarcoma"
            4 => "Tumores neuroendocrinos"
            5 => "Tumor carcinoide"
            6 => "Carcinoma de c&#233;lulas escamosas"
            7 => "Revisi&#243;n de la literatura"
          ]
        ]
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        "titulo" => "Abstract"
        "resumen" => "<p id="spar0010" class="elsevierStyleSimplePara elsevierViewall">Most rectal neoplasms are adenocarcinomas&#44; but there is a small percentage of tumors which are of other histological cell lines such as neuroendocrine tumors&#44; sarcomas&#44; lymphomas&#44; and squamous cell carcinomas&#44; which have special characteristics and different treatments&#46; We have reviewed these rare tumors of the rectum from a clinical and surgical point of view&#46;</p>"
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        "titulo" => "Resumen"
        "resumen" => "<p id="spar0005" class="elsevierStyleSimplePara elsevierViewall">La mayor&#237;a de los c&#225;nceres de recto son adenocarcinomas&#44; pero existe un peque&#241;o porcentaje de tumores de otras estirpes histol&#243;gicas&#44; como neoplasias neuroendocrinas&#44; sarcomas&#44; linfomas y carcinomas de c&#233;lulas escamosas&#44; que tienen unas caracter&#237;sticas y tratamientos diferentes&#46; Hemos efectuado una revisi&#243;n de estos raros tumores del recto desde un punto de vista cl&#237;nico y quir&#250;rgico&#46;</p>"
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        "nota" => "<p class="elsevierStyleNotepara" id="npar0005">Please cite this article as&#58; Errasti Alustiza J&#44; Esp&#237;n Basany E&#44; Reina Duarte &#193;&#46; Neoplasias de recto poco frecuentes&#46; Revisi&#243;n de conjunto&#46; Cir Esp&#46; 2014&#59;92&#58;579&#8211;588&#46;</p>"
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          "leyenda" => "<p id="spar0045" class="elsevierStyleSimplePara elsevierViewall">IBD&#58; inflammatory bowel disease&#59; GIST&#58; gastrointestinal stromal tumor&#59; HIV&#58; human immunodeficiency virus&#59; HPV&#58; human papilloma virus&#59; MANEC&#58; mixed adenoneuroendocrine carcinoma&#59; NEC&#58; neuroendocrine carcinoma&#59; NET&#58; neuroendocrine tumor&#59; CRT&#58; chemoradiotherapy&#59; CTx&#58; chemotherapy&#59; RT&#58; radiotherapy&#46;</p>"
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                  <table border="0" frame="\n
                  \t\t\t\t\tvoid\n
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                  \t\t\t\t\ttable-head\n
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                  \t\t\t\t\ttop\n
                  \t\t\t\t" style="border-bottom: 2px solid black">Tumor type&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Cells of origin&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Usual treatment&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-head\n
                  \t\t\t\t  " align="center" valign="\n
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                  \t\t\t\t" style="border-bottom: 2px solid black">Adjuvant treatment&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t">NET&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Kultschitzky cells&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Unknown&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#8211; No&#46; of mitoses&#8211; Ki67&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#60;1<span class="elsevierStyleHsp" style=""></span>cm&#58; local resection&#62;2<span class="elsevierStyleHsp" style=""></span>cm&#58; oncologic resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">90&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">NEC&#44; MANEC&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Oncologic resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">CTx&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">15&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">GIST&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Interstitial cells of Cajal&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Unknown&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; Size&#8211; No&#46; of mitoses&#8211; Tumor rupture&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Resection with free margins&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Imatinib&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">70&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Other sarcomas&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Rectal connective tissue&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; Previous RT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; Differentiation&#8211; Tumor necrosis&#8211; No&#46; of mitoses&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Resection with free margins&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">RT&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">50&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td></tr><tr title="table-row"><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Lymphomas&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Rectal lymphoid tissue&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; HIV&#8211; IBD&#8211; Immunosuppression&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; Histologic type&#8211; Histologic grade&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Oncologic resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">CTx&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">30&#8211;60&nbsp;\t\t\t\t\t\t\n
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                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">Squamous carcinoma&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">Rectal epithelium&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211; HPV&#8211; Chronic rectal inflammatory processes&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
                  \t\t\t\t\ttop\n
                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">CRTOncologic resection&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
                  \t\t\t\t\ttable-entry\n
                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">&#8211;&nbsp;\t\t\t\t\t\t\n
                  \t\t\t\t</td><td class="td" title="\n
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                  \t\t\t\t  " align="left" valign="\n
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                  \t\t\t\t">30&nbsp;\t\t\t\t\t\t\n
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                    0 => array:2 [
                      "titulo" => "Management of less common tumors of the colon&#58; rectum and anus"
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                        0 => array:2 [ …2]
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                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.3816/CCC.2006.n.002"
                      "Revista" => array:6 [
                        "tituloSerie" => "Clin Colorectal Cancer"
                        "fecha" => "2006"
                        "volumen" => "5"
                        "paginaInicial" => "327"
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                        "link" => array:1 [ …1]
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Rare tumors of the colon and rectum&#58; a national review"
                      "autores" => array:1 [
                        0 => array:2 [ …2]
                      ]
                    ]
                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1007/s00384-006-0145-2"
                      "Revista" => array:6 [
                        "tituloSerie" => "Int J Colorectal Dis"
                        "fecha" => "2007"
                        "volumen" => "22"
                        "paginaInicial" => "183"
                        "paginaFinal" => "189"
                        "link" => array:1 [ …1]
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              "etiqueta" => "3"
              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Uncommon colorectal neoplasms"
                      "autores" => array:1 [
                        0 => array:2 [ …2]
                      ]
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                  ]
                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1055/s-0031-1285995"
                      "Revista" => array:6 [
                        "tituloSerie" => "Clin Colon Rectal Surg"
                        "fecha" => "2011"
                        "volumen" => "24"
                        "paginaInicial" => "127"
                        "paginaFinal" => "128"
                        "link" => array:1 [ …1]
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              "referencia" => array:1 [
                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Rare anorectal neoplasms&#58; gastrointestinal stromal tumor&#44; carcinoid and lymphoma"
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                        0 => array:2 [ …2]
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                  "host" => array:1 [
                    0 => array:2 [
                      "doi" => "10.1055/s-0029-1223842"
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              "referencia" => array:1 [
                0 => array:1 [
                  "referenciaCompleta" => "SEER&#46;cancer&#46;gov &#91;website&#93;&#46; Bethesda&#44; Maryland&#58; US National Institutes of Health&#46; &#91;updated 20 Aug 2012&#46; accessed on 10&#46;01&#46;13&#93;&#46; Available from&#58; <a id="intr0010" class="elsevierStyleInterRef" href="http://www.seer.cancer.gov/csr/1975_2009_pops09/results_merged/sect_06_colon_rectum.pdf">http&#58;&#47;&#47;www&#46;seer&#46;cancer&#46;gov&#47;csr&#47;1975&#95;2009&#95;pops09&#47;results&#95;merged&#47;sect&#95;06&#95;colon&#95;rectum&#46;pdf</a>&#46;"
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                0 => array:2 [
                  "contribucion" => array:1 [
                    0 => array:2 [
                      "titulo" => "Gastrointestinal stromal tumours&#58; ESMO clinical practice guidelines for diagnosis&#44; treatment and follow-up"
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                        0 => array:2 [ …2]
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                    0 => array:2 [
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                        "tituloSerie" => "Ann Oncol"
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                        "volumen" => "23"
                        "numero" => "Suppl&#46; 7"
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es en pt

¿Es usted profesional sanitario apto para prescribir o dispensar medicamentos?

Are you a health professional able to prescribe or dispense drugs?

Você é um profissional de saúde habilitado a prescrever ou dispensar medicamentos