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Thymic Mucosa-Associated Lymphoid Tissue Lymphoma

MedGen UID:
318257
Concept ID:
C1710402
Neoplastic Process
Synonyms: MALT Lymphoma of the Mediastinum; Mediastinal Mucosa-Associated Lymphoid Tissue Lymphoma; Primary Thymic Extranodal Marginal Zone Lymphoma; Thymic Extranodal Marginal Zone Lymphoma of Mucosa-Associated Lymphoid Tissue

Definition

A low-grade extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue that affects the thymus. It is characterized by the presence of centrocyte-like or monocytoid lymphocytes that surround reactive lymphoid follicles. There is infiltration of the thymic epithelium by lymphocytes and formation of lymphoepithelial lesions. It is associated with the presence of autoimmune disease, most frequently Sjogren syndrome. Patients are usually asymptomatic and the tumor is detected incidentally as a mediastinal mass on chest x-ray. A minority of patients present with dyspnea, hemoptysis, and chest pain. The prognosis is excellent. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVThymic Mucosa-Associated Lymphoid Tissue Lymphoma

Recent clinical studies

Diagnosis

Chen Q, Du Y, Prince S, Zhang P, Li G, Wang L, Wang W
Medicine (Baltimore) 2020 Mar;99(13):e19462. doi: 10.1097/MD.0000000000019462. PMID: 32221068Free PMC Article
Naithani R, Ngan BY, Roifman C, Crump M, Baruchel S, Abla O
J Pediatr Hematol Oncol 2012 Oct;34(7):552-5. doi: 10.1097/MPH.0b013e3182459f0a. PMID: 22469942
Kurabayashi A, Iguchi M, Matsumoto M, Hiroi M, Kume M, Furihata M
Pathol Int 2010 Feb;60(2):125-30. doi: 10.1111/j.1440-1827.2009.02486.x. PMID: 20398198
Shimizu K, Yoshida J, Kakegawa S, Astumi J, Kaira K, Oshima K, Miyanaga T, Kamiyoshihara M, Nagai K, Takeyoshi I
J Thorac Oncol 2010 Jan;5(1):117-21. doi: 10.1097/JTO.0b013e3181c07df8. PMID: 19884857
Kitai R, Sasaki M, Sato K, Kubota T, Takeuchi H, Imamura Y
Gen Thorac Cardiovasc Surg 2009 Oct;57(10):566-9. doi: 10.1007/s11748-009-0439-8. PMID: 19830524

Therapy

Kitai R, Sasaki M, Sato K, Kubota T, Takeuchi H, Imamura Y
Gen Thorac Cardiovasc Surg 2009 Oct;57(10):566-9. doi: 10.1007/s11748-009-0439-8. PMID: 19830524

Prognosis

Chen Q, Du Y, Prince S, Zhang P, Li G, Wang L, Wang W
Medicine (Baltimore) 2020 Mar;99(13):e19462. doi: 10.1097/MD.0000000000019462. PMID: 32221068Free PMC Article
Shimizu K, Yoshida J, Kakegawa S, Astumi J, Kaira K, Oshima K, Miyanaga T, Kamiyoshihara M, Nagai K, Takeyoshi I
J Thorac Oncol 2010 Jan;5(1):117-21. doi: 10.1097/JTO.0b013e3181c07df8. PMID: 19884857

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