Primary thyroid lymphoma

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Overview

Primary thyroid lymphoma is almost always of B cell lineage. Preexisting Hashimoto thyroiditis is a known risk factor for the primary thyroid lymphoma and is present among half of the patients. Thyroid lymphoma is rare with an incidence of 0.1-0.2 per 100,000. Common symptoms of primary thyroid lymphoma include painless enlarging neck mass, hoarseness, Difficulty swallowing, and Difficulty breathing. Classic B symptoms of lymphoma occurs in less than 20% of patients. Physical examination findings consistent with the diagnosis of thyroid lymphoma include abnormal texture of thyroid gland in palpation as well as cervical and/or supraclavicular lymphadenopathy. There is no laoratory findings specific for the diagnosis of primary thyroid lymphoma. Thyroid lymphoma may be difficult to be differentiated from Hashimoto thyroiditis. MRI is useful for defining the extent of extrathyroidal invasion of the primary thyroid lymphoma. The CHOP regimen (cyclophosphamide, doxorubicin, vincristine and prednisone) has been showed high effectiveness for many types of thyroid lymphoma. Radiotherapy is indicated in case of patients with localized, extranodal, marginal zone lymphoma of the thyroid or other indolent histologies (eg, follicular lymphoma, small lymphocytic lymphoma).Surgical intervention might be performed for patients with thyroid lymphoma in addition to chemotherapy and radiation, particularly for MALT lymphomas.

Historical Perspective

  • There is no data available on historical perspective of primary thyroid lymphoma.

Classification

Pathophysiology

Causes

Risk Factors

Screening

  • There is insufficient evidence to recommend routine screening for primary thyroid lymphoma.[9]

Differentiating thyroid lymphoma from Other Diseases

Epidemiology and Demographics

  • Thyroid lymphoma is rare with an incidence of 0.1-0.2 per 100,000.[10]
  • It typically presents between 50-70 years of age.[11]
  • Females are more commonly affected with anaplastic thyroid cancer than males. The females to male ratio are approximately 2-8 to 1.

Natural History, Complications and Prognosis

Diagnosis

Diagnostic Study of Choice

Diagnostic Criteria

Staging

  • Staging of thyroid lymphoma is showed in the table below:[13]
Thyroid lymphoma staging
Stage Characteristics
IE Lymphoma is located within the thyroid
2E Lymphoma is located within the thyroid and regional lymph-nodes
3E Lymphoma is located at both sides of diaphragm
4E Dissemination of lymphoma

History and Symptoms

Physical Examination

  • Common physical examination findings of primary thyroid lymphoma include:[15][16]
    • Thyroid has a firm to hard texture in palpation and is fixed to the surronding tissues.
    • Cervical and/or supraclavicular lymphadenopathy may also be present.

Laboratory Findings

Electrocardiogram

  • There are no ECG findings associated with primary thyroid lymphoma.

X-Ray

  • There are no x-ray findings associated with primary thyroid lymphoma.

Echocardiography or Ultrasound

  • Thyroid lymphoma may be difficult to be differentiated from Hashimoto thyroiditis.[17]
  • Three sonographic patterns are reported and include nodular, diffuse and mixed subtypes.[18]
  • Nodular lymphoma has a very uniform and hypoechoic pattern that may be sufficiently hypoechoic to be pseudo-cystic. The border of the tumor is well defined, and the borderline may be "broccoli-like" or "coastline-like" irregular.
  • Diffuse lymphoma has an indistinguishable border from non-lymphomatous part of thyroid and otherwise is similar to goiter. Internal echoes are also of exceedingly low intensity.
  • The mixed lymphoma showes multiple, patchy hypoechoic lesions, with enhanced posterior echoes in each lesion.

CT

  • CT findings associated with the diagnosis of primary thyroid lymphoma include solitary nodule, multiple nodules, and diffuse enlargement.[19]
  • A characteristic finding ob CT imaging is "donut sign," observed when the lymphoma completely encircles the trachea.

MRI

  • MRI is useful for defining the extent of extrathyroidal invasion of the primary thyroid lymphoma.[17]

Other Imaging Findings

  • There are no other imaging findings associated with primary thyroid lymphoma.

Other Diagnostic Studies

  • There are no other diagnostic studies associated with primary thyroid lymphoma.

Treatment

Medical Therapy

Surgery

Primary Prevention

Secondary Prevention

  • There are no established measures for the secondary prevention of primary thyroid lymphoma.

References

  1. Skarsgard ED, Connors JM, Robins RE (October 1991). "A current analysis of primary lymphoma of the thyroid". Arch Surg. 126 (10): 1199–203, discussion 1203–4. doi:10.1001/archsurg.1991.01410340037006. PMID 1929820.
  2. 2.0 2.1 2.2 2.3 2.4 Pedersen RK, Pedersen NT (January 1996). "Primary non-Hodgkin's lymphoma of the thyroid gland: a population based study". Histopathology. 28 (1): 25–32. doi:10.1046/j.1365-2559.1996.268311.x. PMID 8838117.
  3. Vardell Noble, Victoria; Ermann, Daniel A; Griffin, Emily K; Silberstein, Peter T (2019). "Primary Thyroid Lymphoma: An Analysis of the National Cancer Database". Cureus. doi:10.7759/cureus.4088. ISSN 2168-8184.
  4. Ansell SM, Grant CS, Habermann TM (June 1999). "Primary thyroid lymphoma". Semin. Oncol. 26 (3): 316–23. PMID 10375088.
  5. Aggarwal, Nidhi; Swerdlow, Steven H; Kelly, Lindsey M; Ogilvie, Jennifer B; Nikiforova, Mariana N; Sathanoori, Malini; Nikiforov, Yuri E (2012). "Thyroid carcinoma-associated genetic mutations also occur in thyroid lymphomas". Modern Pathology. 25 (9): 1203–1211. doi:10.1038/modpathol.2012.73. ISSN 0893-3952.
  6. Dundar, Halit Ziya; Sarkut, Pinar; Kirdak, Turkay; Korun, Nusret (2016). "Primary thyroid lymphoma". Turkish Journal of Surgery. 32 (1): 75–77. doi:10.5152/UCD.2015.2935. ISSN 1300-0705.
  7. Holm LE, Blomgren H, Löwhagen T (March 1985). "Cancer risks in patients with chronic lymphocytic thyroiditis". N. Engl. J. Med. 312 (10): 601–4. doi:10.1056/NEJM198503073121001. PMID 3838363.
  8. Hyjek E, Isaacson PG (November 1988). "Primary B cell lymphoma of the thyroid and its relationship to Hashimoto's thyroiditis". Hum. Pathol. 19 (11): 1315–26. doi:10.1016/s0046-8177(88)80287-9. PMID 3141260.
  9. "Exit Disclaimer - US Preventive Services Task Force".
  10. . doi:10.1046/j.1365-2559.1996.268311.x. Check |doi= value (help). Missing or empty |title= (help)
  11. Holm, Lars-Erik; Blomgren, Henric; Löwhagen, Torsten (1985). "Cancer Risks in Patients with Chronic Lymphocytic Thyroiditis". New England Journal of Medicine. 312 (10): 601–604. doi:10.1056/NEJM198503073121001. ISSN 0028-4793.
  12. Hedhili, F.; Kedous, S.; Jbali, S.; Attia, Z.; Dhambri, S.; Touati, S.; Chebbi, A. Goucha; El May, A.; Gritli, S. (2015). "Primary thyroid lymphoma: Case report and review of the literature". Egyptian Journal of Ear, Nose, Throat and Allied Sciences. 16 (2): 171–173. doi:10.1016/j.ejenta.2015.05.002. ISSN 2090-0740.
  13. {{cite book | last = Edge | first = Stephen | title = AJCC cancer staging manual | publisher = Springer | location = New York | year = 2010 | isbn = 978-0-387-88440-0 }
  14. Katna, Rakesh; Shet, Tanuja; Sengar, Manju; Menon, Hari; Laskar, Siddharth; Prabhash, Kumar; D'Cruz, Anil; Nair, Reena (2013). "Clinicopathologic study and outcome analysis of thyroid lymphomas: Experience from a tertiary cancer center". Head & Neck. 35 (2): 165–171. doi:10.1002/hed.22928. ISSN 1043-3074.
  15. Logue JP, Hale RJ, Stewart AL, Duthie MB, Banerjee SS (1992). "Primary malignant lymphoma of the thyroid: a clinicopathological analysis". Int. J. Radiat. Oncol. Biol. Phys. 22 (5): 929–33. doi:10.1016/0360-3016(92)90790-o. PMID 1555984.
  16. Tupchong L, Hughes F, Harmer CL (October 1986). "Primary lymphoma of the thyroid: clinical features, prognostic factors, and results of treatment". Int. J. Radiat. Oncol. Biol. Phys. 12 (10): 1813–21. doi:10.1016/0360-3016(86)90324-x. PMID 3759532.
  17. 17.0 17.1 Takashima S, Nomura N, Noguchi Y, Matsuzuka F, Inoue T (1995). "Primary thyroid lymphoma: evaluation with US, CT, and MRI". J Comput Assist Tomogr. 19 (2): 282–8. PMID 7890856.
  18. Ota H, Ito Y, Matsuzuka F, Kuma S, Fukata S, Morita S, Kobayashi K, Nakamura Y, Kakudo K, Amino N, Miyauchi A (October 2006). "Usefulness of ultrasonography for diagnosis of malignant lymphoma of the thyroid". Thyroid. 16 (10): 983–7. doi:10.1089/thy.2006.16.983. PMID 17042683.
  19. Kim HC, Han MH, Kim KH, Jae HJ, Lee SH, Kim SS, Kim KH, Chang KH (June 2003). "Primary thyroid lymphoma: CT findings". Eur J Radiol. 46 (3): 233–9. doi:10.1016/s0720-048x(02)00134-1. PMID 12758117.
  20. Cha H, Kim JW, Suh CO, Kim JS, Cheong JW, Lee J, Keum KC, Lee CG, Cho J (December 2013). "Patterns of care and treatment outcomes for primary thyroid lymphoma: a single institution study". Radiat Oncol J. 31 (4): 177–84. doi:10.3857/roj.2013.31.4.177. PMC 3912230. PMID 24501704.
  21. Peixoto, Rita; Correia Pinto, João; Soares, Virgínia; Koch, Pedro; Taveira Gomes, António (2017). "Primary thyroid lymphoma: A case report and review of the literature". Annals of Medicine and Surgery. 13: 29–33. doi:10.1016/j.amsu.2016.12.023. ISSN 2049-0801.

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