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| Lymphoblastic Lymphoma (Precursor T or B-Lymphoblastic Leukemia/Lymphoma) |
| Etiology • Unknown |
| Pathogenesis • Immunophenotype of immature thymic lymphocytes usually T cell type but occasional B cell neoplasms are seen • T cell receptor gene rearrangement of beta chain in >95% of cases, |
| Epidemiology • Teenage boys (2:1::male:female) with mediastinal mass (>50%) and early involvement of bone marrow and meninges • Most common type of non-Hodgkin's lymphoma in childhood |
| General Gross Description • Large, usually fleshy, occasionally fibrotic masses with areas of hemorrhage |
| General Microscopic Description • Diffuse infiltrate of large cells with little cytoplasm • Nuclei are lobulated with finely granular chromatin and inconspicuous nucleolus • Abundant mitotic rate • Numerous tingible-body macrophages containing lymphocyte debris are seen scattered throughout giving the low power "starry sky" appearance • Mark with terminal deoxynucleotidyl transferase (Tdt), CD1, CD2, CD5 and CD7. |
| Clinical Correlation • Most disease above the diaphragm and patients present secondary to symptoms from the anterior mediastinal mass including effusions, superior vena cava syndrome, and/or airway obstruction • Responsive to aggressive multi-agent chemotherapy |
| References • Cotran RS, et.al. Robbins Pathologic Basis of Disease, 5th edition. Phil.,Saunders, 1994, pp. 641. • Jaffe ES, Surgical Pathology of the Lymph Nodes and related organs, 2nd edition, Phil.,Saunders, 1995, pp. 306-312. |
| Lymphoblastic Lymphoma (Precursor T or B-Lymphoblastic Leukemia/Lymphoma) |
| Synopsis by: Melinda Sanders M.D. (T08000M96023)[331] |
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