- Jonathan W Friedberg, MD
Jonathan W Friedberg, MD
- Professor of Medicine
- James P Wilmot Cancer Center, University of Rochester
- Arnold S Freedman, MD
Arnold S Freedman, MD
- Section Editor — Lymphoproliferative Disorders
- Professor of Medicine
- Harvard Medical School
- R Michael Tuttle, MD
R Michael Tuttle, MD
- Professor of Medicine
- Memorial Sloan Kettering Cancer Center
Primary lymphoma of the thyroid, although rare, should always be considered in the differential diagnosis of patients with thyroid nodules, goiter, and carcinomas, mainly because its prognosis and treatment differ substantially from that of the other disorders. (See "Diagnostic approach to and treatment of thyroid nodules".)
Thyroid lymphomas are nearly always of the non-Hodgkin type, as Hodgkin lymphoma of the thyroid is exceedingly rare . Only about 2 percent of extranodal lymphomas arise within the thyroid gland, and lymphomas represent no more than 2 percent of all malignant thyroid tumors . In a Danish epidemiologic survey, the annual incidence rate was estimated to be 2.1 per million persons, with a four to one female predominance . Most other retrospective series have confirmed this markedly higher frequency of thyroid lymphoma in women [4-7]. The mean and median ages at diagnosis are between 65 and 75 years [3-7].
Preexisting chronic autoimmune (Hashimoto's) thyroiditis is the only known risk factor for primary thyroid lymphoma and is present in about one-half of patients. Among patients with Hashimoto's thyroiditis, the risk of thyroid lymphoma is at least 60 times higher than in patients without thyroiditis [3,8,9]. Worldwide, the frequency of thyroid lymphoma appears to be higher in areas with a higher prevalence of thyroiditis.
Lymphoma may also occur more commonly after iodine supplementation . There appears to be no clear association between exposure to ionizing radiation and lymphoma , although individual cases have been described. Chromosomal abnormalities are rare in thyroid lymphomas.
CLASSIFICATION AND PATHOLOGY
Primary thyroid lymphoma is almost always of B-cell lineage [3,6,7]. Occasional patients with T-cell lymphomas have been reported, often in areas endemic for human T-lymphotrophic virus (HTLV)-I-associated adult T-cell leukemia/lymphoma [12,13]. Sixty to 80 percent of thyroid lymphomas are diffuse large B-cell lymphomas (DLBCLs) [4,5,7,14,15], and considered to be of follicle center cell origin. (See "Classification of the hematopoietic neoplasms".)
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