MALT Lymphoma of the Thymus
Key Facts
Terminology
Low-grade lymphoma derived from distinct native population of thymic postgerminal center stage parafollicular B cells
Etiology/Pathogenesis
Believed to represent thymic counterpart of distinctive type of extranodal marginal zone lymphoma of mucosa-associated lymphoid tissue (MALToma)
Clinical Issues
Generally excellent prognosis with long-term survival
Some cases may give rise to development of diffuse large cell lymphoma
Indolent growth with protracted clinical course
Microscopic Pathology
Monotonous proliferation of small lymphoid cells in interfollicular distribution
Lymphoid cell population is admixed with reactive, hyperplastic lymphoid follicles
Lymphoid cells infiltrate residual epithelial structures (i.e., Hassall corpuscles), creating lymphoepithelial lesions
Residual thymic epithelium may undergo cystic degeneration resulting in multiple thymic cysts
Small lymphocytes can infiltrate surrounding perithymic fat at edges of lesion
Lymphoid cells display slightly irregular nuclei surrounded by clear cytoplasmic halos
Lymphoid cells may display plasmacytoid features
Rare mitotic figures may be present
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. |
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
Size
5-10 cm in greatest dimensionStay updated, free articles. Join our Telegram channel
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