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Inicio Endocrinología y Nutrición (English Edition) Primary thyroid lymphoma
Journal Information
Vol. 61. Issue 9.
Pages 497-499 (November 2014)
Vol. 61. Issue 9.
Pages 497-499 (November 2014)
Scientific letter
DOI: 10.1016/j.endoen.2014.04.010
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Primary thyroid lymphoma
Linfoma primario del tiroides
A. Ríos
Corresponding author

Corresponding author.
, J.M. Rodríguez, B. Febrero, P. Parrilla
Servicio de Cirugía General y del Aparato Digestivo I, Hospital Universitario Virgen de la Arrixaca, Murcia, Spain
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Tables (1)
Table 1. Cases of primary thyroid lymphoma.
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Primary thyroid lymphoma (PTL) is an uncommon condition which, to be categorized as such, must only affect the thyroid gland and, eventually, the locoregional lymph nodes. Primary disease in other location should be ruled out.1,2 Diagnosis of PTL is complex, and surgery is often required to make a definitive diagnosis. Therapeutic management has greatly changed over time, and chemotherapy, with or without radiotherapy, is the current treatment of choice.1,2 We report our experience in PTL management since introduction of the new chemotherapy protocols in order to analyze: (1) the need for surgery; (2) recurrences; and (3) changes over time.

Patients treated at our hospital in the past 10 years with histological diagnosis of any of the pathological variants of PTL were selected for the study. Epidemiological, diagnostic, therapeutic, histological, and evolutionary variables were analyzed.

Seven patients, a majority of them women with a mean age of 64 years, met the diagnostic criteria for PTL. PTL occurred in all cases as a rapidly growing neck tumor, associated to compression symptoms in six of them (86%) (Table 1). Ultrasonography showed diffuse thyroid enlargement in 3 patients (43%) and a thyroid nodule occupying a great part of the corresponding half of the thyroid gland in the other 4 patients. Surgery was indicated in all patients, in 4 (57%) due to suspected malignancy and in the other 3 for compression symptoms. Five patients (71%) underwent total thyroidectomy, and the other 2, hemithyroidectomy. There were no postoperative complications. Four patients (57%) had a diffuse B lymphoma, and 3 patients a low grade MALT lymphoma. B lymphomas were associated to Hashimoto thyroiditis. A stage IE tumor was found in 6 patients (86%), and the remaining patient had a stage IIE tumor. All patients were given adjuvant CHOP chemotherapy (cyclophosphamide, adriamycin, vincristine, and prednisone), associated to radiotherapy with 50Gy in 5 patients (71%). Six patients are currently alive and free of disease, and one patient died at 8 years for a cause unrelated to the disease.

Table 1.

Cases of primary thyroid lymphoma.

  Case 1  Case 2  Case 3  Case 4  Case 5  Case 6  Case 7 
Sex  Female  Male  Female  Female  Female  Female  Female 
Age  71 years  45 years  79 years  70 years  54 years  68 years  71 years 
Time since onset  4 months  7 days  3 months  3 months  1 month  3 months  1 month 
Neck enlargement  Yes, sudden  Yes, sudden  Yes, sudden  Yes, sudden  Yes, sudden  Yes, sudden  Yes, sudden 
Main symptom  Dysphonia  Pain  Asymptomatic  Stridor  Dysphonia  Dyspnea  Pain 
Thyroid ultrasonography and adenopathies  LTL noduleNo adenopathies  LTL noduleNo adenopathies  Diffuse enlargementNo adenopathies  Diffuse enlargementAdenopathies  RTL noduleNo adenopathies  Diffuse enlargementNo adenopathies  RTL noduleAdenopathies 
FNA  No  Yes  Yes  No  No  No  Yes 
Cytology  –  Papillary Ca suspected  Proliferation of plasma cells  Inconclusive  –  –  Inconclusive 
Surgery indicated  Goiter with compression syndrome  Suspected malignancy  Suspected malignancy  Suspected malignancy  Goiter with compression syndrome  Goiter with compression syndrome  Suspected malignancy 
Treatment  Total thyroidectomy  Hemithyroidectomy  Total thyroidectomy  Total thyroidectomy  Hemithyroidectomy  Total thyroidectomy  Hemithyroidectomy 
Complications  No  No  No  No  No  No  No 
Pathology  Diffuse B lymphoma  Low grade MALT lymphoma  Low grade MALT lymphoma  High grade MALT lymphoma  Diffuse B lymphoma  Diffuse B lymphoma  High grade diffuse B lymphoma 
Associated thyroiditis  Yes, Hashimoto thyroiditis  No  No  No  Yes, Hashimoto thyroiditis  Yes, Hashimoto thyroiditis  Yes, Hashimoto thyroiditis 
Stage  IE  IE  IE  IE  IE  IE  IIE 
Radiotherapy  50Gy  –  –  50Gy  50Gy  50Gy  50Gy 
Recurrence  No  No  No  No  No  No  No 
Survival  66 months  85 months  70 months  120 months  120 months  35 months  12 months 
Death  No  No  Yes, stroke Free of disease  No  No  No  No 

PTL is an uncommon disease with a greater incidence in women over 60 years of age. Risk of PTL development is 80-fold higher in lymphocytic Hashimoto thyroiditis, but evolution from this condition to lymphoma is uncommon (0.1%).1 In our series, B lymphoma was universally associated to Hashimoto thyroiditis, but in no case to MALT lymphomas.

The most common clinical presentation is a rapidly growing mass that causes symptoms due to compression or tissue infiltration, as seen in our patients. Some authors suggest that patients often experience neck adenopathies, but these did not occur in most of our patients. It should be noted that B symptoms only occur in 10% of thyroid lymphomas.1,2

Diagnosis of certainty usually requires a surgical biopsy, because most examinations have a low sensitivity for diagnosis of lymphoma.2 However, although surgical biopsy appears to be the gold standard for diagnosis, fine needle aspiration (FNA) should be the initial test of choice. Conventional cytological examination will probably be insufficient, and should therefore be supplemented with immunohistochemistry, molecular techniques, flow cytometry, and detection of monoclonal Ig heavy chains by PCR.3 However, this technology may not be available in many hospitals. Thus, Matsuzuka et al.4 stated that 78% of cases may be adequately diagnosed by FNA, and Graff-Baker et al.5 reported an 81% decrease in surgery in their 1973–1987 series, and a 61% decrease in their 1997–2005 series. It should be noted, however, that many patients diagnosed with rapidly growing goiter suspected of malignancy still undergo surgery to make a definitive diagnosis.6 It should be reminded that most PTLs are non-Hodgkin lymphomas, either diffuse large B cell lymphoma or MALT lymphoma, with the follicular, small lymphocytic, and Burkitt subtypes being less common.2,5 All tumors in our series were B cell or MALT lymphomas.

Surgery used to be the standard treatment, but the current treatment of choice is CHOP chemotherapy, and association of rituximab, with or without radiotherapy, is now recommended.7 Some authors prescribe radiotherapy without chemotherapy for low grade lymphomas,8 especially MALT lymphomas.9 Today, the only situation where thyroidectomy could be indicated would be stage IE MALT lymphoma, in which it would lead to a better prognosis as compared to chemotherapy. Treatment is decided based on the Ann Arbor stage. Prognosis depends on disease stage, and 5-year survival rates for stages IE, IIE, and iii-IVE are 80%, 50%, and 35% respectively.1,10 Patients in our series were in poorly advanced stages, and survival to date is 100%.

In conclusion, PTL should be suspected in women with rapid thyroid growth and associated compression symptoms. If tumor is diagnosed at an early stage, prognosis is good with adequate chemotherapy and radiotherapy. Surgery is mainly useful for diagnostic purposes.

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Clinicopathological features of 171 cases of primary thyroid lymphoma: a long-term study involving 24,553 patients with Hashimoto's disease.
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Please cite this article as: Ríos A, Rodríguez JM, Febrero B, Parrilla P. Linfoma primario del tiroides. Endocrinol Nutr. 2014;61:497-499.

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