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 increased1,2 ; some believe that a pathogenetic link exists between this autoimmune disorder and thyroid NHL.3 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 neoplasms4,5,6 . 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 series5,7 .
Age
Similar to other NHLs, thyroid lymphomas usually affect patients with a median age of 60 years8 .
Clinical
History
The most common clinical presentation is that of a rapidly enlarging thyroid mass, frequently in association with neck adenopathy, as depicted in the image below.8 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.
More on Thyroid Lymphoma |
Overview: Thyroid Lymphoma |
| Differential Diagnoses & Workup: Thyroid Lymphoma |
| Treatment & Medication: Thyroid Lymphoma |
| Follow-up: Thyroid Lymphoma |
| Multimedia: Thyroid Lymphoma |
| References |
| Further Reading |
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References
Kebapcilar L, Alacacioglu I, Comlekci A, et al. Primary thyroid lymphoma: case series with literature review. J BUON. Apr-Jun 2009;14(2):295-9. [Medline].
Graff-Baker A, Sosa JA, Roman SA. Primary thyroid lymphoma: a review of recent developments in diagnosis and histology-driven treatment. Curr Opin Oncol. Oct 19 2009;[Medline].
Holm LE, Blomgren H, Lowhagen T. Cancer risks in patients with chronic lymphocytic thyroiditis. N Engl J Med. Mar 7 1985;312(10):601-4. [Medline].
Ansell SM, Grant CS, Habermann TM. Primary thyroid lymphoma. Semin Oncol. Jun 1999;26(3):316-23. [Medline].
Austin JR, el-Naggar AK, Goepfert H. Thyroid cancers. II. Medullary, anaplastic, lymphoma, sarcoma, squamous cell. Otolaryngol Clin North Am. Aug 1996;29(4):611-27. [Medline].
Pasieka JL. Anaplastic cancer, lymphoma, and metastases of the thyroid gland. Surg Oncol Clin N Am. Oct 1998;7(4):707-20. [Medline].
Tupchong L, Hughes F, Harmer CL. Primary lymphoma of the thyroid: clinical features, prognostic factors, and results of treatment. Int J Radiat Oncol Biol Phys. Oct 1986;12(10):1813-21. [Medline].
Ha CS, Shadle KM, Medeiros LJ, et al. Localized non-Hodgkin lymphoma involving the thyroid gland. Cancer. Feb 15 2001;91(4):629-35. [Medline].
Velasquez WS, Jagannath S, Tucker SL, et al. Risk classification as the basis for clinical staging of diffuse large- cell lymphoma derived from 10-year survival data. Blood. Aug 1 1989;74(2):551-7. [Medline].
Swan F Jr, Velasquez WS, Tucker S, et al. A new serologic staging system for large-cell lymphomas based on initial beta 2-microglobulin and lactate dehydrogenase levels. J Clin Oncol. Oct 1989;7(10):1518-27. [Medline].
Ito Y, Amino N, Miyauchi A. Thyroid ultrasonography. World J Surg. Oct 13 2009;epub ahead of print. [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. Sep 1996;14(9):2427-30. [Medline].
Joshi A, Chan J, Bruch G, et al. Thyroid lymphoma and airway obstruction - is there a rationale for surgical management?. Int J Clin Pract. Nov 2009;63(11):1647-52. [Medline].
Isaacson PG. Lymphoma of the thyroid gland. Curr Top Pathol. 1997;91:1-14. [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. Sep 30 1993;329(14):987-94. [Medline].
Miller TP, Dahlberg S, Cassady JR, et al. Chemotherapy alone compared with chemotherapy plus radiotherapy for localized intermediate- and high-grade non-Hodgkin''s lymphoma. N Engl J Med. Jul 2 1998;339(1):21-6. [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. Summer 1993;3(2):93-9. [Medline].
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. Nov 1992;3(9):711-7. [Medline].
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].
Macchiarini P, Ostertag H. Uncommon primary mediastinal tumours. Lancet Oncol. Feb 2004;5(2):107-18. [Medline].
Rohana A, Hisham AN. Emergency thyroid surgery: a surgical challenge. Asian J Surg. Apr 2009;32(2):81-4. [Medline].
Further Reading
Related eMedicine Topics
- Hashimoto Thyroiditis
- Lymphomas, Endocrine, Mesenchymal, and Other Rare Tumors of the Mediastinum
- Thyroid Nodule [in the Endocrinology section]
- Thyroid Nodules [in the Radiology section]
- Thyroid, Papillary Carcinoma
Clinical Trials
- 11C-Acetate PET/CT Non-FDG-Avid Tumors
- A Pilot Clinical Trial for Poorly Differentiated Thyroid Cancer - Correlation to Retinoind and Peroxisome-Proliferator-Activated Receptor (PPARy) Expression
- Study of 18F-Fluorodeoxyglucose (FluGlucoScan) in Patients With Cancer or Suspected Cancer
Clinical Guidelines
- American Association of Clinical Endocrinologists and Associazione Medici Endocrinologi medical guidelines for clinical practice for the diagnosis and management of thyroid nodules. American Association of Clinical Endocrinologists - Medical Specialty Society; Associazione Medici Endocrinologi - Medical Specialty Society. 1996 Jan (revised 2006 Feb). 40 pages. NGC:004869
- Long-term follow-up guidelines for survivors of childhood, adolescent, and young adult cancers. Sections 38-91: radiation. Children's Oncology Group - Medical Specialty Society. 2003 Sep (revised 2006 Mar). 74 pages. NGC:005599
- Management guidelines for patients with thyroid nodules and differentiated thyroid cancer. American Thyroid Association - Professional Association. 2006 Feb. 34 pages. NGC:005212
- Thyroid carcinoma. Association of Comprehensive Cancer Centres - Disease Specific Society. 2007 Jun. 146 pages. NGC:006062
Keywords
thyroid lymphoma, 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 tumor, thyroid nodule, thyroid cancer


Overview: Thyroid Lymphoma