Primary thyroid gland lymphomas are rare tumors which usually arise in the setting of a chronic autoimmune thyroiditis. These tumors characteristically present with a mass lesion in the thyroid gland.
INCIDENCE 5% of all thryoid neoplasms
2.5-7% of all extranodal lymphomas
AGE RANGE-MEDIAN Middle to older age (4-8th decades)
DISEASE ASSOCIATIONS CHARACTERIZATION Autoimmune thyroiditis Relative risk of developing lymphoma is 40-80x greater than general population
Usually occurs 20-30 years after onset
PATHOGENESIS CHARACTERIZATION MALT lymphomas The majority are MALT lymphomas
RADIOLOGIC STUDIES Radioisotopic I131 scanning reveals a cold nodule or areas of decreased uptake
HISTOLOGICAL TYPES CHARACTERIZATION General
Malignant Lymphoma of the Thyroid Gland A Clinicopathologic Study of 108 Cases
Gregory A. Derringer, M.D.; Lester D. R. Thompson, M.D.; R. Allen Frommelt, Ph.D.; Karen E. Bijwaard, M.S.; Clara S. Heffess, M.D., COL, MC, USA; Susan L. Abbondanzo, M.D.
From the Departments of Hematopathology (G.A.D., S.L.A.), Endocrine and Otorhinolaryngic-Head & Neck Pathology (L.D.R.T., C.S.H.), Cellular Pathology (K.E.B.), and Department of Epidemiology, Repository and Research Services (R.A.F.), Armed Forces Institute of Pathology, Washington, DC, U.S.A.
Am J Surg Pathol 2000;24:623-639 Abstract quote
We report a retrospective clinicopathologic study of 108 primary thyroid gland lymphomas (PTLs), classified using the REAL and proposed WHO classification schemes.
The patients included 79 women and 29 men, with an average age of 64.3 years. All patients presented with a thyroid mass. The PTLs were classified as marginal zone B-cell lymphoma of mucosa-associated lymphoid tissue (MALT) or MZBL (n = 30), diffuse large B-cell lymphoma (DLBCL) with MZBL (n = 36), DLBCL without MZBL (n = 41), and follicle center lymphoma (FCL; n = 1). Excluding the FCL, features of lymphomas of MALT-type were identified in all groups, despite a follicular architecture in 23% of cases. Lymphocytic thyroiditis (LT) was identified in 94%. Ninety-one percent of patients presented with stage IE or IIE disease, whereas 69% had perithyroidal soft tissue infiltration.
All patients were treated with surgical excision followed by adjuvant therapy (76%): chemotherapy (15%), radiation (19%), or a combination of radiation and chemotherapy (42%). Disease-specific survival was 82% at last follow up (mean, 82.8 mos) and 79% at 5 years. Statistically, stages greater than IE, presence of DLBCL, rapid clinical growth, abundant apoptosis, presence of vascular invasion, high mitotic rate, and infiltration of the perithyroidal soft tissue were significantly associated with death with disease. No patients with MZBL or stage IE disease died with disease.
In summary, PTLs typically occur in middle-to older-aged individuals as a thyroid mass, with a predilection for females. Despite their histologic heterogeneity and frequent simulation of other lymphoma subtypes, virtually all PTLs are lymphomas of MALT-type arising in the setting of LT. Mixed DLBCL and MZBL are common.
Overall, PTLs have a favorable outcome with appropriate therapy, but prognosis depends on both clinical stage and histology. MZBL and stage IE tumors have an excellent prognosis, whereas tumors with a large cell component or DLBCL or stage greater than IE have the greatest potential for a poor outcome.
PROGNOSIS AND TREATMENT CHARACTERIZATION Treatment Surgical with adjuvant chemotherapy, radiation, or both
Sternberg S. Diagnostic Surgical Pathology. Third Edition. Lipincott Williams and Wilkins 1999.
Last Updated 10/12/2001
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