Introduction
Background
Primary thyroid lymphoma can be defined as a lymphoma that arises from the thyroid gland. This definition excludes lymphomas that invade the thyroid gland because of either metastasis or direct extension from an adjacent lymph node. Primary thyroid lymphomas usually are of the non-Hodgkin type. Primary thyroid Hodgkin disease is extremely rare. Non-Hodgkin lymphomas (NHLs) can be further divided into aggressive and indolent cell types.
The aggressive NHLs comprise a large number of cell types, the most common of which is large-cell lymphoma. NHLs most frequently arise from lymph nodes, but an extranodal site is the primary source in approximately 30% of cases, and the thyroid gland is among the most common of these extranodal sites.
Pathophysiology
Of interest, these disorders are frequently associated with Hashimoto thyroiditis, and the incidence of primary thyroid lymphomas in patients with Hashimoto thyroiditis is markedly increased; some believe that a pathogenetic link exists between this autoimmune disorder and thyroid NHL.1 The proposed theory hypothesizes that chronic antigenic stimulation secondary to the autoimmune disorder leads to chronic proliferation of lymphoid tissue, which eventually undergoes a mutation that results in clonal proliferation, leading to the development of lymphoma. Hypothyroidism has been observed in 30-40% of patients with thyroid lymphoma.
Frequency
United States
Thyroid lymphomas constitute only 3% of all NHLs and approximately 5% of all thyroid neoplasms2,3,4 . Although thyroid NHL is not common, it is highly curable without extensive surgery; for this reason, it should be recognized early and treated correctly.
Mortality/Morbidity
Primary thyroid lymphoma, as discussed in this article, is a highly curable malignancy if diagnosed promptly and managed correctly.
Sex
As expected, because of its association with Hashimoto thyroiditis, thyroid lymphomas are more common in women than in men, with a ratio ranging from 2:1 to as high as 14:1 in some series3,5 .
Age
Similar to other NHLs, thyroid lymphomas usually affect patients with a median age of 60 years6 .
Clinical
History
The most common clinical presentation is that of a rapidly enlarging thyroid mass, frequently in association with neck adenopathy6 (see Image 1). With the exception of anaplastic thyroid carcinoma, thyroid NHL usually grows faster than any other thyroid neoplasm; however, low-grade or indolent NHLs occasionally can arise in the thyroid gland, and their growth rate is slower. Hoarseness, respiratory difficulty, cough, and dysphagia also can occur as presenting symptoms.
Physical
Patients with large-cell thyroid lymphoma usually present with a rapidly growing thyroid nodule; however, those with an indolent histology (eg, those with a mucosa-associated lymphoid tissue [MALT] lymphoma) present with a slow-growing node, which can grow for months or years before it is brought to the physician's attention. Neck adenopathy can also be present in association with the thyroid nodule.
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References
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Glick JH, Kim K, Earle J, et al. An ECOG randomized phase III trial of CHOP vs. CHOP + radiotherapy (XRT) for intermediate grade early stage non-Hodgkin's lymphoma (NHL) [abstract]. Proceedings of the American Society of Clinical Oncology. 1995;14 (A-1221):391.
Laing RW, Hoskin P, Hudson BV, et al. The significance of MALT histology in thyroid lymphoma: a review of patients from the BNLI and Royal Marsden Hospital. Clin Oncol (R Coll Radiol). 1994;6(5):300-4. [Medline].
Miller TP, Dahlberg S, Cassidy JR, et al. Three cycles of CHOP (CHOP-3) plus radiotherapy (RT) is superior to eight cycles of CHOP (CHOP-8) alone for localized intermediate grade non-Hodgkin's lymphoma (NHL). A Southwest Oncology Group study. Proc Ann Meet Am Soc Clin Oncol. 1996;15 (A1257):411.
Ito Y, Yoshida H, Matsuzuka F, et al. Cdc25A and cdc25B expression in malignant lymphoma of the thyroid: Correlation with histological subtypes and cell proliferation. Int J Mol Med. Mar 2004;13(3):431-5. [Medline].
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Further Reading
Keywords
thyroid MALT, thyroid large cell lymphoma, thyroid large-cell lymphoma, primary thyroid lymphomas, non-Hodgkin lymphomas, non-Hodgkin's lymphomas, NHLs, Hashimoto thyroiditis, Hashimoto's thyroiditis, thyroid cancer
Overview: Thyroid Lymphoma