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Pathology - Extranodal marginal zone lymphoma
Definition: An extranodal mature B-cell neoplasm primarily consisting of tiny neoplastic marginal zone cells.
Epidemiology • Constitutes 7–8% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults with a mean age of 60. Locations of engagement • The gastrointestinal system constitutes 50% of all instances, with the stomach as the predominant site. • Additional locations encompass the lung, salivary gland, skin, thyroid, and breast.
Etiology • Gastric instances are generally linked to Helicobacter pylori. • Additional implicated species comprise Campylobacter jejuni (jejunum) and Borrelia burgdorferi (skin). • Autoimmune illnesses are also linked, such as Hashimoto’s thyroiditis (thyroid) and Sjögren’s syndrome (salivary gland). Pathogenesis • The majority of cases are preceded by a chronic inflammatory condition that results in the accumulation of extranodal lymphoid tissue. • Extended stimulation of lymphoid proliferation ultimately culminates in the transition into a neoplastic process.
Presentation • Symptoms associated with a mass at the affected location.
Histopathology • Affected tissues comprise a diverse assemblage of small neoplastic B-cells that encircle and may infiltrate the underlying reactive lymphoid follicles. • The cellular composition includes marginal zone cells, monocytoid-like cells, small lymphocytes, and dispersed immunoblasts and centroblast-like cells. • In epithelial-lined tissues, the neoplastic lymphoid cells generally infiltrate and obliterate the epithelium, resulting in lymphoepithelial lesions. Immunophenotype: B-cell markers PAX5, CD20, and CD79a are expressed positively. CD5, CD10, CD23, and cyclin D1 are all absent.
Prognosis: Exhibits indolent behavior with extended periods of disease-free remission post-treatment.
Definition: An extranodal mature B-cell neoplasm primarily consisting of tiny neoplastic marginal zone cells.
Epidemiology • Constitutes 7–8% of all non-Hodgkin B-cell lymphomas. • Primarily occurs in adults with a mean age of 60. Locations of engagement • The gastrointestinal system constitutes 50% of all instances, with the stomach as the predominant site. • Additional locations encompass the lung, salivary gland, skin, thyroid, and breast.
Etiology • Gastric instances are generally linked to Helicobacter pylori. • Additional implicated species comprise Campylobacter jejuni (jejunum) and Borrelia burgdorferi (skin). • Autoimmune illnesses are also linked, such as Hashimoto’s thyroiditis (thyroid) and Sjögren’s syndrome (salivary gland). Pathogenesis • The majority of cases are preceded by a chronic inflammatory condition that results in the accumulation of extranodal lymphoid tissue. • Extended stimulation of lymphoid proliferation ultimately culminates in the transition into a neoplastic process.
Presentation • Symptoms associated with a mass at the affected location.
Histopathology • Affected tissues comprise a diverse assemblage of small neoplastic B-cells that encircle and may infiltrate the underlying reactive lymphoid follicles. • The cellular composition includes marginal zone cells, monocytoid-like cells, small lymphocytes, and dispersed immunoblasts and centroblast-like cells. • In epithelial-lined tissues, the neoplastic lymphoid cells generally infiltrate and obliterate the epithelium, resulting in lymphoepithelial lesions. Immunophenotype: B-cell markers PAX5, CD20, and CD79a are expressed positively. CD5, CD10, CD23, and cyclin D1 are all absent.
Prognosis: Exhibits indolent behavior with extended periods of disease-free remission post-treatment.
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