MALT Lymphoma of the Thymus



MALT Lymphoma of the Thymus











MALT lymphoma of the thymus shows sheets of small lymphoid cells with clear perinuclear halos cuffing a dilated cystic space. The cystic space is lined by a layer of flattened cuboidal epithelium.






High magnification of MALT lymphoma of the thymus shows monotonous proliferation of lymphocytes with minimal nuclear irregularities. Some of the lymphocytes display clear perinuclear halos.


TERMINOLOGY


Abbreviations



  • Mucosa-associated lymphoid tissue lymphoma (MALT lymphoma)


Synonyms



  • Marginal zone B-cell lymphoma


  • Monocytoid B-cell lymphoma


  • Parafollicular B-cell lymphoma


Definitions



  • Low-grade lymphoma derived from distinct native population of thymic postgerminal center stage parafollicular B cells


ETIOLOGY/PATHOGENESIS


Pathogenesis



  • Believed to represent thymic counterpart of distinctive type of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma)


CLINICAL ISSUES


Site



  • Anterior-superior mediastinum


Presentation



  • Asymptomatic (> 50% of cases)


  • Chest pain and shortness of breath in larger lesions


  • Indolent growth with protracted clinical course (> 80% 5-year survival)


  • Often associated with Sjögren syndrome and polyclonal hypergammaglobulinemia


  • Predilection for middle-aged females


Treatment



  • Surgical excision


  • Postoperative chemotherapy in some cases


Prognosis



  • Generally excellent prognosis with long-term survival


  • Some cases may give rise to development of diffuse large cell lymphoma


MACROSCOPIC FEATURES


General Features



  • Diffusely enlarged thymus with homogeneous rubbery cut surface


  • May display secondary cystic changes


Sections to Be Submitted



  • One section per cm of largest diameter


  • Submit fresh tissue for flow cytometry and cytogenetics

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on MALT Lymphoma of the Thymus

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