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combined effects of occlusion of small vessels and concentric in ltration of the adjacent tissue by neoplastic cells In half of the cases, meningeal vessels are involved and the neoplastic cells have incited an in ammatory response that can be detected in the spinal uid, although malignant cells are not found in the uid In a few cases, the peripheral nerves, or more particularly the roots, have also been involved by the neoplasm, and we have seen two cases with a accid paraplegia on this basis Although the lymphoid origin of the anaplastic cells is now clear, not all of them are T cells, as was at one time believed; an equal number have the features of B cells Moreover, in some cases as in primary CNS lymphoma, portions of the genome of the Epstein-Barr virus (EBV) have been isolated from the malignant B cells within the pleocellular in ltrates For these reasons it has been proposed that the disorder represents an EBV-induced proliferation of B cells with a prominent in ammatory T-cell reaction (Guinee et al) Because of the inconsistent location and size of the nervous system lesions, there is no uniform syndrome, but the disease should be suspected in patients with a subacute encephalopathy and indications of focal brain and spinal cord or nerve root lesions Headache is a prominent early component in some cases One of our patients had intermittent seizures 3 months before confusion and progressive encephalopathy The variety of clinical presentations is emphasized in the reviews of 8 cases by Beristain and Azzarelli and the article by Glass and associates All had focal cerebral signs, 7 had dementia, 5 had seizures, and 2 had myelopathy Some of our own cases, as mentioned above, have also had a accid paraplegia due to in ltration of the cauda roots; this peripheral involvement has been commented on by other authors Only a few patients will have nodular or multiple in ltrative pulmonary lesions, skin lesions, or adenopathy; almost all of our cases were restricted to the brain and spinal cord, but other reports suggest systemic disease in a high proportion MRI shows multiple nodular or variegate abnormalities in T2weighted images throughout the white matter of the brain; most lesions are enhanced by gadolinium In one of the cases we studied there were numerous hemorrhagic lesions De nitive diagnosis is possible only through a biopsy of radiographically involved lung or nervous tissue that includes numerous intrinsic blood vessels A helpful diagnostic feature is the presence of antibodies to nuclear cytoplasmic antigens (c-ANCA), which are present in some cases, as they are in a number of other vasculitic and granulomatous processes A few of our own patients have also had adrenal or renal enlargement; presumably due to in ltration of the vessels of these organs by the neoplasm The spinal uid has a variable lymphocytic pleocytosis and protein elevation, but malignant cells are not found The sedimentation rate and serum LDH are said to be elevated in most patients, but this has not been so in our experience Like demyelinating and lymphomatous lesions, these abnormalities may recede temporarily in response to treatment with corticosteroids, and some clinical improvement occurs The course tends to be indolent and relapsing over months or years, although one of our patients died within weeks despite treatment In a few cases, whole-brain irradiation has been successful in prolonging survival, but the outlook in most instances is poor An uncertain number of these patients have AIDS (none in our group) The illness must be distinguished from multiple sclerosis, brain lymphoma, gliomatosis cerebri, and a process that simulates it closely, sarcoidosis (which produces brain and lung lesions) as well as from the cerebral vasculitides and Behcet disease,.

Figure 611 A D Heavy neck and jowls is best treated with aggressive liposuction in addition to a full rhytidectomy

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but intravascular lymphoma is generally more rapidly progressive than most of these conditions Sarcomas of the Brain These are malignant tumors composed of cells derived from connective tissue elements ( broblasts, rhabdomyocytes, lipocytes, osteoblasts, smooth muscle cells) They take their names from their histogenetic derivation namely, brosarcoma, rhabdomyosarcoma, osteogenic sarcoma, chondrosarcoma and sometimes from the tissue of which the cells are a part, such as adventitial sarcomas and hemangiopericytoma All these tumors are rare They constitute from 1 to 3 percent of intracranial tumors, depending on how wide a range of neoplasms one chooses to include in this group (see below) Occasionally one or more cerebral deposits of these types of tumors will occur as a metastasis from a sarcoma in another organ Others are primary in the cranial cavity and exhibit as one of their unique properties a tendency to metastasize to nonneural tissues a decidedly rare occurrence with primary glial tumors It is a disturbing fact that a few sarcomas have developed 5 to 10 years after irradiation or, in one instance among 3000 patients of which we are aware, after proton beam irradiation of the brain Fibrosarcomas have occurred after radiation of pituitary adenomas and osteogenic sarcoma after other types of radiation, all localized to bone or meninges Our experience with hemangiopericytoma has been limited to two intracranial lesions that simulated meningiomas and two others that arose in the high cervical spinal cord and caused subacute quadriparesis initially misdiagnosed as a polyneuropathy A number of other cerebral tumors, described in the literature as sarcomas, are probably tumors of other types The rapidly growing, highly malignant monstrocellular sarcoma of Zulch or gi ant-cell brosarcoma of Kernohan and Uihlein, so named for their multinucleated giant cells, have been reinterpreted by Rubinstein as a form of giant-cell glioblastoma or mixed glioblastoma and brosarcoma The hemangiopericytoma of the leptomeninges, also classi ed by Kernohan and Uihlein as a form of cerebral sarcoma, is considered by Rubinstein to be a variant of the angioblastic meningioma of Bailey and Cushing.

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