Thyroid Lymphoma

Thyroid Lymphoma

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Primary thyroid lymphoma can be defined as a lymphoma that arises from the thyroid gland. This definition excludes those that invade the thyroid gland as a consequence of either metastasis or direct extension. Primary thyroid lymphomas are practically always non-Hodgkin lymphomas (NHLs). Primary thyroid Hodgkin lymphoma is extremely rare.

NHLs can be divided into aggressive and indolent cell types. Aggressive NHLs comprise a large number of cell types, the most common of which is large-cell lymphoma. They 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.

Thyroid NHL represents approximately 1.2 to 1.7% of all NHLs. [1] It is highly curable, without the need for extensive surgery. Accordingly, early recognition and correct treatment of this condition is vital. The most common cell type is diffuse large-cell lymphoma, either associated or unassociated with mucosa-associated lymphoid tissue (MALT) lymphoma (MALToma).The best treatment results for primary thyroid large-cell lymphoma are with combined-modality therapy; for primary thyroid MALToma, radiation therapy alone is probably adequate. [2, 3] Primary T-cell lymphoma of thyroid is extremely rare, accounting for less than 2% of all primary thyroid lymphomas, and can present both diagnostic and therapeutic challenges. [4]

Thyroid lymphomas are very frequently associated with Hashimoto thyroiditis (HT). Conversely, there is a markedly increased incidence of primary thyroid lymphomas in patients with HT. [5] Such lesions are an aggressive or high-grade variant, whereas a low-grade MALT is an indolent type.

Like other low-grade MALTomas, such as those presenting in the parotid in association with Sjögren syndrome, those arising in the thyroid also occur in association with an autoimmune disorder (in this case, HT). The hypothesis is that chronic antigenic stimulation secondary to the autoimmune disorder leads to chronic proliferation of lymphoid tissue, which eventually undergoes a mutation that leads to the development of lymphoma.

Primary thyroid lymphoma is rare, constituting only 1-2% of all extranodal lymphomas and approximately 2-8% of all thyroid malignancies. [6, 5, 7, 8] As with other non-Hodgkin lymphomas, the median age of presentation in patients with thyroid lymphoma is usually close to 60 years. [8, 7] Most cases occur in women.

The prognosis for patients with thyroid large-cell lymphoma usually is favorable because they typically present with localized disease, which is amenable to treatment with chemotherapy and radiation. The cure rate is typically high (see Treatment). A large population-based study that evaluated prognostic factors found that older age, advanced stage, histologic subtype, and lack of radiation/surgical treatment were adverse factors for survival. [9]

A rare case of pulmonary metastasis has been reported in a 65-year-old woman diagnosed with a primary thyroid MALToma who underwent total thyroidectomy, followed by chemotherapy. After 5 years of follow-up, she was diagnosed with metastatic thyroid MALT lymphoma. [10]

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Mishra P, Banerjee D, Gujral S. T-cell Lymphoma of Thyroid Gland with Lennert Type of Morphology: A Case Report and Review of the Literature. Head Neck Pathol. 2016 Sep. 10 (3):321-6. [Medline].

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Peppa M, Nikolopoulos P, Korkolopoulou P, Lapatsanis D, Dimitriadis G, Hadjidakis D, et al. Primary mucosa-associated lymphoid tissue thyroid lymphoma: a rare thyroid neoplasm of extrathyroid origin. Rare Tumors. 2012 Jan 2. 4(1):e2. [Medline]. [Full Text].

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Satoh K, Morita T, Fumimoto S, Tsuji H, Ichihashi Y, Ochi K, et al. Rare Pulmonary Metastasis From Thyroid Mucosa-Associated Lymphoid Tissue Lymphoma. Ann Thorac Surg. 2015 Aug. 100 (2):700-2. [Medline].

Pappa VI, Hussain HK, Reznek RH, et al. Role of image-guided core-needle biopsy in the management of patients with lymphoma. J Clin Oncol. 1996 Sep. 14(9):2427-30. [Medline].

Ha CS, Shadle KM, Medeiros LJ, et al. Localized non-Hodgkin lymphoma involving the thyroid gland. Cancer. 2001 Feb 15. 91(4):629-35. [Medline].

Watanabe N, Noh JY, Narimatsu H, et al. Clinicopathological features of 171 cases of primary thyroid lymphoma: a long-term study involving 24 553 patients with Hashimoto’s disease. Br J Haematol. 2011 Mar 4. [Medline].

International Non-Hodgkin Lymphoma Prognostic Factors Project. A predictive model for aggressive non-Hodgkin”s lymphoma. The International Non-Hodgkin”s Lymphoma Prognostic Factors Project. N Engl J Med. 1993 Sep 30. 329(14):987-94. [Medline].

Matsuzuka F, Miyauchi A, Katayama S, et al. Clinical aspects of primary thyroid lymphoma: diagnosis and treatment based on our experience of 119 cases. Thyroid. 1993 Summer. 3(2):93-9. [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.

Coiffier B, Thieblemont C, Van Den Neste E, Lepeu G, Plantier I, Castaigne S, et al. Long-term outcome of patients in the LNH-98.5 trial, the first randomized study comparing rituximab-CHOP to standard CHOP chemotherapy in DLBCL patients: a study by the Groupe d’Etudes des Lymphomes de l’Adulte. Blood. 2010 Sep 23. 116(12):2040-5. [Medline]. [Full Text].

Rodriguez J, Cabanillas F, McLaughlin P, et al. A proposal for a simple staging system for intermediate grade lymphoma and immunoblastic lymphoma based on the ”tumor score”. Ann Oncol. 1992 Nov. 3(9):711-7. [Medline].

Fernando Cabanillas, MD Professor of Medicine, University of Puerto Rico School of Medicine; Adjunct Professor of Medicine, MD Anderson Cancer Center, University of Texas Medical School at Houston; Adjunct Professor, Moffitt Cancer Center; Medical Director, Auxilio Mutuo Cancer Center, Puerto Rico

Fernando Cabanillas, MD is a member of the following medical societies: Alpha Omega Alpha, American Association for the Advancement of Science, American Association for Cancer Research, American College of Physicians-American Society of Internal Medicine, American Society of Hematology, New York Academy of Sciences, Texas Medical Association

Disclosure: Nothing to disclose.

Emmanuel C Besa, MD Professor Emeritus, Department of Medicine, Division of Hematologic Malignancies and Hematopoietic Stem Cell Transplantation, Kimmel Cancer Center, Jefferson Medical College of Thomas Jefferson University

Emmanuel C Besa, MD is a member of the following medical societies: American Association for Cancer Education, American Society of Clinical Oncology, American College of Clinical Pharmacology, American Federation for Medical Research, American Society of Hematology, New York Academy of Sciences

Disclosure: Nothing to disclose.

Lodovico Balducci, MD Professor of Oncology and Medicine, University of South Florida College of Medicine; Division Chief, Senior Adult Oncology Program, H Lee Moffitt Cancer Center and Research Institute

Disclosure: Nothing to disclose.

Wendy Hu, MD Consulting Staff, Department of Hematology/Oncology and Bone Marrow Transplantation, Huntington Memorial Medical Center

Wendy Hu, MD is a member of the following medical societies: American Association for the Advancement of Science, American College of Physicians, American Society for Blood and Marrow Transplantation, American Society of Hematology, and Physicians for Social Responsibility

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Thyroid Lymphoma

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