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cently it has been suggested that hydroxyurea has the capacity to shrink unresectable and recurrent meningiomas (Schrell et al); this mode of treatment is still being evaluated Primary Cerebral Lymphoma As mentioned earlier, this tumor has assumed increasing signi cance in the last two decades because of its frequency in AIDS and other immunosuppressed states However, it is also occurring with increased frequency in immunocompetent persons a nding without evident explanation (although theories abound) There is a preponderance of men, with the peak incidence in the fth through seventh decades, or in the third and fourth decades in patients with AIDS For many years, the cell of origin of this tumor was thought to be the reticulum cell and the tumor was regarded as a reticulum cell sarcoma The meningeal histiocyte and microgliacytes are the equivalent cells in the brain to the reticulum cell of the germinal centers of lymph nodes Later, the intracerebral lymphocytes and lymphoblasts, also prominent components of the tumor, led to its reclassi cation as a lymphoma (large-cell histiocytic type) It is appreciated, on the basis of immunocytochemical studies, that the tumor cells are B lymphocytes There is a ne reticulum reaction between the reticulum cells derived from broblasts and microglia or histiocytes As matters now stand, most pathologists believe that the B lymphocyte or lymphoblast is the tumor cell, whereas the ne reticulum and microgliacytes are secondary interstitial reactions We tend to believe that both cell types are present and have not entirely abandoned the notion of an origin in a reticulum cell In contrast, T-cell lymphomas of the nervous system are rare but do occur in both immunocompetent patients and in immunosuppressed patients with AIDS Since the brain is devoid of lymphatic tissue, it is uncertain how this tumor arises; one theory holds that it represents a systemic lymphoma with a particular proclivity to metastasize to the nervous system This seems unlikely to the authors; systemic lymphomas of the usual kind rarely metastasize, as discussed further on, under Involvement of the Nervous System in Systemic Lymphoma These tumors may arise in any part of the cerebrum, cerebellum, or brainstem, with 60 percent being in the cerebral hemispheres; they may be solitary or multifocal A periventricular localization is common Vitreous, uveal, and retinal (ocular) involvement occurs in 10 to 20 percent of cases; here vitreous biopsy may be diagnostic, but it is not often performed (Two-thirds of patients with ocular lymphoma will have cerebral involvement within a year) The pia-arachnoid may be in ltrated, and a purely meningeal form of B-cell lymphoma that involves peripheral and cranial nerves is also known Most such cases of what has been termed neurolymphomatosis present with painful, predominantly motor polyradiculopathies One such patient of ours presented with a accid paraparesis and back and sciatic pain; MRI showed tumor in ltrating the cauda equina nerve roots and contiguous meninges Lai and colleagues have presented evidence that, in advanced cases that were autopsied, microscopic deposits of tumor were found in many regions of the brain and not solely in areas indicated by nodular enhancement on MRI Whether this indicates a widespread or multifocal origin of brain lymphoma is not clear The primary large-cell lymphoma forms a pinkish gray, soft, ill-de ned, in ltrative mass in the brain, dif cult at times to distinguish from a malignant glioma Perivascular and meningeal spread results in shedding of cells into the CSF, accounting perhaps for the multifocal appearance of the tumor in almost half of the cases The tumor is highly cellular and grows around and into blood ves-.

Figure 31-8 Gadolinium-enhanced MRI of meningioma Large subfrontal mass with central calci cation and surrounding vasogenic edema Homogeneous enhancement is characteristic of the tumor

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on CT and MRI or by tumor blush on angiography Meningiomas characteristically take the form of smoothly contoured masses, sometimes lobulated, with one edge abutting the inner surface of the skull or the falcial or tentorial dura On the CT scan they are isointense or slightly hyperintense unless enhanced by contrast infusion; calci cation at the outer surface or heterogeneously throughout the mass is common Except for the mass effect they cause, they may be dif cult to see on MRI scans performed without gadolinium The amount of edema surrounding the tumor is highly variable The CSF protein is usually elevated Treatment Surgical excision should afford permanent cure in all symptomatic and accessible surface tumors Recurrence is likely if removal is incomplete, as is often the case, but for some the growth rate is so slow that there may be a latency of many years A few show malignant qualities ie, a high mitotic index, nuclear atypia, marked nuclear and cellular pleomorphism, and invasiveness of brain Their regrowth is then rapid if they are not completely excised Tumors that lie beneath the hypothalamus, along the medial part of the sphenoid bone and parasellar region, or anterior to the brainstem are the most dif cult to remove surgically By invading adjacent bone, they may become impossible to remove totally Carefully planned radiation therapy including gamma-knife or proton beam treatment is bene cial in cases that are inoperable and when the tumor is incompletely removed or shows malignant characteristics (Kornblith et al) Smaller tumors at the base of the skull can be obliterated or greatly reduced in size by focused radiation, probably with less risk than surgery would pose (Chang and Alder) Conventional chemotherapy is probably ineffective; hormonal therapy with the antiprogestin agents has been used with variable results but is still under study (McCutcheon) More re-.

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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