INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS
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sels ( angiocentric pattern); it elicits little tendency to necrosis The nuclei are oval or bean-shaped with scant cytoplasm, and mitotic gures are numerous B-cell markers applied to xed tissue de ne the lymphoblastic cell population as monoclonal and identify the tumor cell type The stainability of reticulum and microglial cells, the latter by Hortega s silver carbonate method, also serves to distinguish this tumor microscopically There is no tumor tissue outside the brain It should also be mentioned that several of our cases of meningeal and cranial nerve lymphoma with similar histologic characteristics to primary CNS lymphoma were complications of lymphatic leukemia Primary lymphoma involving the cerebral hemispheres pursues a clinical course somewhat similar to that of the glioblastoma but with a vastly different response to treatment The interval between the rst symptom and operation has been approximately 3 months Behavioral and personality changes, confusion, dizziness, and focal cerebral signs predominate over headache and other signs of increased intracranial pressure as presenting manifestations Seizures may occur but are less common in our experience than they are as the introductory feature of gliomas Most cases occur in adult life, but some have been observed in children, in whom the tumor may simulate the cerebellar symptomatology of medulloblastoma Rare cases that remit spontaneously appear in the literature from time to time The nding on CT and MRI of one or several dense, homogeneous, and enhancing periventricular masses is characteristic (Fig 31-9) However, nodular, ring-like enhancement also occurs, and any part of the brain may be involved The radiologic appearance in AIDS patients is less predictable and may be dif cult to distinguish from that of toxoplasmosis or another process with which lymphoma may coexist Characteristic is the radiographic disappearance of the lesions or complete but transient resolution of
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contrast enhancement in response to corticosteroids Lymphocytic and mononuclear pleocytosis of CSF is more frequent than with gliomas and metastatic tumors The immunohistochemical demonstration in CSF of monoclonal lymphocytes or an elevated beta microglobulin points to leptomeningeal spread of the tumor (Li et al), but frequently the diagnosis is not possible from CSF cytologic examination alone As already indicated, patients with AIDS and less common immunode ciency states, such as the Wiskott-Aldrich syndrome and ataxia-telangiectasia, and those receiving immunosuppressive drugs for long periods, such as patients undergoing renal transplantation, are particularly liable to develop this type of primary cerebral lymphoma Many of the tumors in immunosuppressed patients contain the Epstein-Barr virus (EBV) genome, suggesting a pathogenetic role for the virus (Bashir and colleagues); however, the EBV genome has been found in the lymphoma cells in both immunocompetent and immunosuppressed patients (Hochberg and Miller) Sometimes this tumor appears as a complication of an obscure medical condition such as salivary and lacrimal gland enlargement (Mikulicz syndrome) Stereotactic needle biopsy is the preferred method of establishing the histologic diagnosis in sporadic cases But in AIDS patients, the differential diagnosis of a solitary brain nodule that is suspected to be lymphoma is aided by the response, or lack thereof, to treatment for toxoplasmosis, the main alternative diagnosis to brain lymphoma (see page 646) Reduction in the size of the lesion(s) with antimicrobial drugs makes biopsy unnecessary Treatment Because the tumors are deep and often multicentric, surgical resection is ineffective except in rare instances Treatment with cranial irradiation and corticosteroids often produces a partial or, rarely, complete response, as remarked above, but the tumor recurs in more than 90 percent of patients Until recently the median survival of patients treated in this way has been 10 to 18 months,
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Figure 31-9 Cerebral lymphoma MRIs with gadolinium infusion Left: T1-weighted MRI of a nodular cerebral lymphoma after gadolinium infusion Right: Another typical appearance of a smaller ring-enhancing periventricular mass with subtle in ltration of the subependymal regions
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