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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 Correlations: • 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. W.B. Saunders, 1994, pp. 641. • Jaffe ES, Surgical Pathology of the Lymph Nodes and related organs, second edition, W.B. Saunders, 1995, pp. 306-312. |