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1/5. histiocytic sarcoma involving the central nervous system: clinical, immunohistochemical, and molecular genetic studies of a case with review of the literature.

    histiocytic sarcoma (HS) is a rare disease, and there has been much confusion concerning the diagnostic criteria for this entity. Since immunohistochemical and cytogenetic techniques have become more universally available, many cases initially diagnosed as histiocytic sarcoma have been reclassified as other diseases. We describe a case of HS that presented as a single mass lesion in left occipital lobe. At autopsy the tumor also involved the meninges as a thick exudate. Histologic examination showed numerous large pleomorphic malignant cells with areas of necrosis, numerous neutrophils, and phagocytosis by tumor cells. Immunohistochemically, the tumor cells stained positively with antibodies directed against most histiocytic markers and did not stain with antibodies directed against myeloid markers, dendritic markers, CD30, ALK1, or other lymphoid markers. Molecular cytogenetic analysis showed no rearrangement [i.e. t(2;5) translocation or other variant] by fluorescence in situ hybridization. The T-cell receptor-gamma chain by multiplex polymerase chain reaction showed a polyclonal pattern. No heavy or light chain gene rearrangements were found. To our knowledge, this is the first reported autopsy case of this rare entity primarily involving the brain and meninges.
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2/5. Early molecular detection of central nervous system relapse in a child with systemic anaplastic large cell lymphoma: case report and review of the literature.

    We report a case of anaplastic large cell lymphoma (ALCL) with central nervous system relapse in an 11-year-old boy. The relapse was suspected on morphologic examination of the cytospin preparations of the cerebrospinal fluid (CSF) with a WBC of 10 cells/microl. CSF relapse was confirmed using immunohistochemistry (IHC), fluorescence in situ hybridization (FISH), and reverse transcriptase-polymerase chain reaction (RT-PCR) for abnormal ALK expression or gene structure. The patient developed large intracranial metastases, despite systemic, and intrathecal chemotherapy. This case demonstrates the feasibility of detecting ALCL in paucicellular CSF specimens and suggests that even low CSF involvement can herald massive parenchymal disease.
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3/5. Primary central nervous system T-cell lymphoma with a predominant CD8 immunophenotype.

    BACKGROUND. Primary T-cell lymphomas of the central nervous system are uncommon neoplasms. methods. A case of a primary central nervous system T-cell lymphoma in a 66-year-old female who died 8 months after surgery is described. The biopsy specimen was evaluated by routine histology, immunohistochemistry, flow cytometry, and Southern blotting/dna hybridization. RESULTS. The neoplasm was composed of pleomorphic medium and large cells. Virtually all of the neoplastic cells reacted with antibodies to CD3, CD5, and CD8. Multiple rearranged bands were detected with the T-cell receptor beta-chain gene probe. CONCLUSION. To the authors' knowledge, this is the first description of a primary central nervous system T-cell lymphoma composed of a predominant population of CD8-expressing T cells, and the first case confirmed by Southern blotting/dna hybridization.
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4/5. Epstein-Barr virus-associated primary central nervous system lymphoma in a child with the acquired immunodeficiency syndrome. A case report and review of the literature.

    A 34-month-old black boy who had contracted acquired immunodeficiency syndrome from his mother presented with fever, vomiting, and cough. He was cachectic, hypertonic, and developmentally delayed. A brain computed tomography scan revealed masses in the left frontal horn, subependymal, and periventricular regions; secondary edema; and hydrocephalus. The differential diagnosis was cerebral lymphoma versus toxoplasmosis. The patient had disseminated mycobacterium avium-intracellulare infection, lymphoid interstitial pneumonitis, as well as pseudomonas and klebsiella pneumonia. He died of respiratory insufficiency 53 days after admission. The autopsy confirmed a primary cerebral B-cell lymphoma, large cell type, which was positive for Epstein-Barr virus, latent phase, by in situ hybridization. Primary central nervous system lymphomas are rare in children, in contrast to adults. To our knowledge, only five well-documented cases of primary cerebral lymphomas in infants and children with acquired immunodeficiency syndrome have been reported previously. The current study shows that these childhood lymphomas are associated with and presumably caused by Epstein-Barr virus and thus have a pathogenesis similar to that of primary central nervous system lymphomas in adults.
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5/5. Primary central nervous system lymphoma in a child with acute B-cell lymphoblastic leukaemia: consecutive Epstein-Barr virus-related malignancies.

    Primary central nervous system lymphoma (PCNSL), observed among immunocompromised AIDS patients, has not been reported during chemotherapy for acute lymphoblastic leukaemia (ALL). We report a case of PCNSL occurring in a child receiving intensive multiagent chemotherapy for B-cell ALL. in situ hybridization studies demonstrated Epstein-Barr virus genome in both tumours, suggesting a possible link between the two diseases. The clinical response of the PCNSL to conservative therapy highlights the importance of accurately diagnosing such EBV-related disorders, especially in patients where immune compromise can be reversed.
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