Cases reported "Nervous System Neoplasms"

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1/3. Neoplastic angioendotheliomatosis. Report of two autopsy cases with special reference to the origin of atypical cells.

    Two autopsy cases of neoplastic angioendotheliomatosis (NAE) were presented. Both patients were elderly woman, characterized by an ascending progression of transverse myelopathy and abnormal brain shadows similar to metastatic tumor in computer tomographic examination in Case 1, and by typical clinical features of cerebral infarction in Case 2. Postmortem examinations of both cases revealed an exclusive intravascular distribution of large atypical cells in generalized organs, especially in the central nervous system, and associated multiple cerebral infarcts and, in Case 1, widespread demyelination of the spinal cord. There were no distinct lesions suggestive of a primary focus. In both cases only a few atypical cells were immunohistochemically positive for factor viii-related antigen, likely due to non-specific absorption of serum factor viii into the cells. On the other hand, almost all of the atypical cells were immunoreactive for LN-1, LN-2, and leukocyte common antigen, suggestive of lymphocytic (B cell) origin.
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2/3. diagnosis of CNS lymphoma using immunofluorescent phenotyping of CSF mononuclear cells.

    We describe the use of a panel of monoclonal antibodies, directed against leukocyte surface antigens to characterize CSF mononuclear cells with regard to malignancy when cytopathology was inconclusive. Cytocentrifuged preparations from three patients in which traditional modalities had not yielded a diagnosis were studied, utilizing a panel of antibodies for B and T cell antigens. All three patients were found to have B cell lymphoma of the CNS. Rapid institution of the appropriate therapy resulted in marked improvement of CNS symptoms in each case. Our results indicate that in patients with CNS disease and CSF pleocytosis of undefined nature, this technique may provide rapid and precise diagnostic information.
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3/3. Primary melanocytic schwannoma of the stomach. Evidence of dual melanocytic and schwannian differentiation in an extra-axial site in a patient without neurofibromatosis.

    Pigmented neoplasms of the peripheral nervous system are uncommon. Such lesions, while well documented in the para-axial region, are infrequently encountered in more peripheral extra-axial sites. The authors present a case of a melanocytic schwannoma arising in the gastric antrum of a 51-year-old woman. Ultrastructural features attesting to the nerve sheath origin of the neoplasm included the presence of a well-developed basal lamina, complex infoldings of the plasma membrane, and intercellular bundles of long spaced collagen ("Luse bodies"). Premelanosomes and melanosomes in all stages of development were identified within the cytoplasm of many of the neoplastic cells, providing morphologic support for a close relationship between schwann cells and melanocytes. Immunoperoxidase studies with S-100 protein disclosed intense cytoplasmic staining throughout the tumor. The S-100 protein antigen has been documented in both nerve sheath elements and melanocytes; this shared antigenicity lends further support to the close relationship between melanocytic and schwannian elements suggested by ultrastructural observations. Additional immunoperoxidase studies employing antibody to glial fibrillary acidic protein revealed focal cytoplasmic staining, an uncommon phenomenon in peripheral nerve sheath neoplasms. A review of the literature failed to provide any previous well documented cases of melanocytic schwannoma of the stomach. The patient was treated with en bloc resection of the tumor and gastric antrum, and was free of evidence of recurrent or metastatic disease 22 months postoperatively. Theories regarding the histogenesis of pigmented nerve sheath neoplasms and prognostic implications are discussed, based on experience with this and previously reported pigmented nerve sheath tumors.
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