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Case study
Childhood cervical lipofibromatosis
Lipofibromatosis cervical en la infancia
Raquel Solaveraa,
Corresponding author
raquel.ssv@gmail.com

Corresponding author.
, Catalina Rieraa, Paul Andradeb, Marta Faubela
a Servicio de Otorrinolaringología, Hospital General de Castellón, Castellón, Spain
b Servicio de Anatomía Patológica, Hospital General de Castellón, Castellón, Spain
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    "textoCompleto" => "<span class="elsevierStyleSections"><span id="sec0005" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0005">Clinical case</span><p id="par0005" class="elsevierStylePara elsevierViewall">The patient was a 4-year-old girl&#44; with no background of interest&#44; who was referred from paediatrics due to a cervical mass which did not respond to antibiotic treatment&#46;</p><p id="par0010" class="elsevierStylePara elsevierViewall">Physical examination revealed an anterior movable cervical tumour of 3&#8239;cm&#44; which was not painful on palpation&#44; extending to the sternal notch&#46;</p><p id="par0015" class="elsevierStylePara elsevierViewall">Cervical ultrasound was performed reporting an ovoid tumour of 4&#46;3&#8239;&#215;&#8239;1&#46;5&#8239;cm located in subcutaneous cell tissue compatible with lipoma and a full magnetic resonance test was performed &#40;<a class="elsevierStyleCrossRef" href="#fig0005">Fig&#46; 1</a>&#41;&#46;</p><elsevierMultimedia ident="fig0005"></elsevierMultimedia><p id="par0020" class="elsevierStylePara elsevierViewall">An ultrasound-guided core needle biopsy was requested&#44; which reported mature adipose tissue with no signs of malignancy&#46;</p><p id="par0025" class="elsevierStylePara elsevierViewall">With a suspected diagnosis of lipoma we decided to continue with conservative treatment&#44; with a bi-annual medical check-up and annual ultrasound scan&#46;</p><p id="par0030" class="elsevierStylePara elsevierViewall">After three years of slow progressive growth we decided to perform surgery with complete excision of the tumour via cervicotomy&#46;</p><p id="par0035" class="elsevierStylePara elsevierViewall">The surgical specimen extracted presented with an encapsulated appearance of 6&#8239;&#215;&#8239;4&#8239;&#215;&#8239;3&#46;5&#8239;cm&#44; alternating between yellowish areas next to whitish fibrous regions in appearance&#46; Microscopic analysis reported adipose tissue lobules separated by fibrous septa compatible with lipoblastoma &#40;<a class="elsevierStyleCrossRef" href="#fig0010">Fig&#46; 2</a>&#41;&#46;</p><elsevierMultimedia ident="fig0010"></elsevierMultimedia><p id="par0040" class="elsevierStylePara elsevierViewall">Immunohistochemical techniques resulted in&#58; negative actin and CD31&#44; Ki67 under 1&#37; and focally positive CD34&#46; A diagnosis of lipofibromatosis was therefore reached&#46;</p></span><span id="sec0010" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0010">Discussion</span><p id="par0045" class="elsevierStylePara elsevierViewall">Lipofibromatosis is a rare benign tumour&#44; located in the subcutaneous cell tissue of extremities&#44; although it may also appear in other regions&#46;<a class="elsevierStyleCrossRef" href="#bib0005"><span class="elsevierStyleSup">1</span></a></p><p id="par0050" class="elsevierStylePara elsevierViewall">It is usually asymptomatic&#44; with patients consulting over aesthetic deformities or symptoms derived from compression of adjacent structures&#46;</p><p id="par0055" class="elsevierStylePara elsevierViewall">With regard to epidemiology&#44; age of appearance on average is at one year&#44; presenting tumours prior to the age of 3 years in most patients&#46; There is no clear predominance of either sex&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p><p id="par0060" class="elsevierStylePara elsevierViewall">Differential diagnosis should be made with entities such as&#58; fibromatosis&#44; calcifying aponeurotic fibroma&#44; fibrous hamartoma of infancy&#44; lipoblastoma&#44; lipofibromatous hamartoma&#44; lipoma&#44; macrocystic lymphatic malformation&#44; myofibroma and rabdomyosarcoma&#46;<a class="elsevierStyleCrossRef" href="#bib0015"><span class="elsevierStyleSup">3</span></a></p><p id="par0065" class="elsevierStylePara elsevierViewall">Ultrasound and nuclear magnetic resonance is used for diagnosis&#44; but definitive diagnosis is histological&#44; presenting as a tumour consisting of adipose tissue separated into lobules by fibroblast or myofibroblasts fascicles with immunohistochemical positive testing for CD99 and CD34 in fused cells and S100 in adipocytes&#46;<a class="elsevierStyleCrossRefs" href="#bib0020"><span class="elsevierStyleSup">4&#8211;6</span></a></p><p id="par0070" class="elsevierStylePara elsevierViewall">The treatment of choice is complete excision&#46; In cases where there is clinical stability and no invasion of deep tissues&#44; a wait-and-see approach may be used&#46;<a class="elsevierStyleCrossRef" href="#bib0010"><span class="elsevierStyleSup">2</span></a></p></span><span id="sec0015" class="elsevierStyleSection elsevierViewall"><span class="elsevierStyleSectionTitle" id="sect0015">Conflict of interests</span><p id="par0075" class="elsevierStylePara elsevierViewall">The authors have no conflict of interests to declare&#46;</p></span></span>"
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ISSN: 21735735
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