Which of the following human malignancy is highly malignant, rapidly progressive and delay in treatment of even 1-2 days can lead death?
Ans. c (Burkitt's lymphoma). (Ref. Bobbin's 8,h e455)BURKITT'S LYMPHOMA# Very aggressive tumor of mature B cells# Usually arises at extranodal sites# Uniformly associated with translocations involving the c-MYC proto-oncogene# Often associated with latent infection by Epstein-Barr virus (EBV).# Most children and young adults can be cured, the, however, outcome is guarded in adults.# A high mitotic index and apoptotic cell death are typical.# "Starry sky" pattern is characteristic & cells proliferate by C-MYC deregulation.# Chromosome 8, EBV, t (2, 8), t (8, 22) and myc gene translocation are associated with Burkitt's lymphoma# CNS involvement is common# Hodgkin's lymphoma, mycosis fungoides are indolent tumors.SUMMARY OF THE MORE COMMON LYMPHOID NEOPLASMSENTITYSALIENT MORPHOLOGYIMMUNOPHENOTYPECOMMENTSPrecursor B-cell lymphoblastic leukemia/ lymphomaLymphoblasts with irregular nuclear contours, condensed chromatin, small nucleoli, and scant agranular cytoplasmTdT+immature B cells (CD19+, variable expression of other B-cell markers)Usually presents as acute leukemia; less common in adults; prognosis is predicted by karyotypePrecursor T-cell leukemia/ lymphomaIdentical to the precursorB-cell lymphoblastic leukemia/lymphomaTdT+immature T cells(CD2+, CD7+, variable expression of other T-cell markers)Most common in adolescent males; often presents as a mediastinal mass due to thymic involvement; highly associated with mutations in NOTCH1Small lymphocytic lymphoma/chronic lymphocytic leukemiaSmall resting lymphocytes mixed with variable numbers of large activated cells; lymph nodes diffusely effacedCD5+B-cell expressing surface IgOccurs in older adults; usually involves nodes, marrow, and spleen; most patients have peripheral blood involvement; indolentFollicular lymphomaFrequent small ''cleaved" cells mixed with large cells; growth pattern is usually nodular (follicular)CD10+BCL2+mature B cells that express surface IgOccurs in older adults; usually involves nodes, marrow, and spleen; associated with t( 14; 18); indolentMantle cell lymphomaSmall to intermediate-sized irregular lymphocytes growing in a diffuse patternCD5+mature B cells that express cyclin D1 and have surface IgOccurs mainly in older males; usually involves nodes, marrow, and spleen; GI tract also commonly affected; t(11;14) is characteristic; moderately aggressiveExtranodal marginal zone lymphomaVariable cell size and differentiation; 40% show plasmacytic differentiation; B cells home to the epithelium, creating "lymphoepithelial lesions"CDS- CD10- mature B cells with surface IgFrequently occurs at extranodal sites involved by chronic inflammation; very indolent; may be cured by local excisionDiffuse large B-cell lymphomaVariable; most resemble large germinal center B cells; diffuse growth patternMature B cells with variable expression of CD10 and surface IgOccurs in all ages, but most common in older adults; often arise at extranodal sites; aggressiveBurkitt lymphomaIntermediate-sized round lymphoid cells with several nucleoli; diffuse tissue involvement associated with apoptosis produces a "starry-sky" appearanceMature CD10+B cells expressing surface IgEndemic in Africa, sporadic elsewhere; increased frequency in immune suppressed patients; predominantly affects children; often presents with visceral involvement; highly aggressivePlasmacytoma/ plasma cell myelomaPlasma cells in sheets, sometimes with prominent nucleoli or inclusions containing IgTerminally differentiated plasma cells containing cytoplasmic IgMyeloma presents as a disseminated bone disease, often with destructive lytic lesions. Hypercalcemia, renal insufficiency, and bacterial infections are commonMycosis fungoidesIn most cases, small lymphoid cells with markedly convoluted nuclei; cells often infiltrate the epidermis (Pautrier microabscesses)CD4+mature T cellsPresents with localized or more generalized skin involvement; generally indolent. Sezary syndrome, a more aggressive variant, is characterized by diffuse skin erythema and peripheral blood involvementPeripheral T-cell lymphoma, not otherwise specified (NOS)Variable; usually a spectrum of small to large lymphoid cells with irregular nuclear contoursMature T-cell phenotype (CD3+)Probably spans a diverse collection of rare tumors. Often disseminated, generally aggressiveHodgkin lymphoma, nodular sclerosis typeLacunar Reed-Sternberg cell variants in a mixed inflammatory background; broad sclerotic bands of collagen usually also presentCD15+, CD30+Reed- Sternberg cellsMost common in young adults, often arises in the mediastinum or cervical lymph nodesHodgkin lymphoma, mixed cellularity typeFrequent classic Reed- Sternberg cells in a mixed inflammatory backgroundCD15+, CD30+Reed- Sternberg cellsMost common in men, more likely to present at advanced stages than the nodular sclerosis type EBV+in 70% of cases
Hodgkin's lymphoma
Neuroblastoma
Burkitt's lymphoma
Mycosis fungoides