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INTRACRANIAL NEOPLASMS AND PARANEOPLASTIC DISORDERS
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The age of onset is usually between 15 and 50 years Blacks, whites, and Asians are equally affected Dominant inheritance of von Hippel-Lindau disease is well known Seizinger and coworkers, in cases associated with renal cell carcinoma and pheochromocytoma, have localized a defect in a tumor suppressor gene (termed VHL) on chromosome 3p (see Chap 38) The diagnosis can be deduced from the appearance on CT or MRI of a cerebellar cyst containing an enhancing nodular lesion on its wall Often the associated retinal hemangioma will be disclosed by the same imaging procedure The angiographic picture is also characteristic: a tightly packed cluster of small vessels forming a mass 10 to 20 cm in diameter (Fig 31-13) Craniotomy with opening of the cerebellar cyst and excision of the mural hemangioblastomatous nodule is usually curative, but there is a high rate of recurrence if the entire tumor, including the nodule, is not completely removed In the series of Boughey and colleagues, the lesion was successfully excised in 80 percent of patients Fifteen percent of patients, who had only partial resection of an isolated cerebellar lesion, developed recurrent tumors More recently, several groups have used endovascular embolization of the vascular nodule prior to surgery, but it is not clear if this reduces the incidence of recurrence Treatment with small doses of focused radiation is also being undertaken but must be considered experimental until more results are available Hemangioblastomas of the spinal cord are frequently associated with a syringomyelic lesion (greater than 70 percent of cases); such lesions may be multiple and are located mainly in the posterior columns A retinal hemangioblastoma may be the initial nding and leads to blindness if not treated by laser The children of a parent with a hemangioblastoma of the cerebellum should be examined regularly for an ocular lesion and renal cell carcinoma New lesions continue to be formed over a period of years while the patient is under observation
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Pineal Tumors There has been much uncertainty as to the proper classi cation of pineal tumors Originally they were all thought to be composed of pineal cells, hence they were classi ed as true pinealomas, a term suggested by Krabbe Globus and Silbert believed that these originated from embryonic pineal cells But later Russell pointed out that some tumors in the pineal region are really atypical teratomas resembling the seminoma of the testicle Four types of pineal tumor are now recognized germinoma (atypical teratoma), pinealoma (pineocytoma and pineoblastoma), true teratoma with cellular derivatives of all three germ layers, and a glioma originating in astroglial cells of the pineal body The germinoma is a rm, discrete mass that usually reaches 3 to 4 cm in greatest diameter It compresses the superior colliculi and sometimes the superior surface of the cerebellum and narrows the aqueduct of Sylvius Often it extends anteriorly into the third ventricle and may then compress the hypothalamus A germinoma may also arise in the oor of the third ventricle; this has been referred to as an ectopic pinealoma or suprasellar germinoma Microscopically, these tumors are composed of large, spherical epithelial cells separated by a network of reticular connective tissue and containing many lymphocytes Of the four groups of pineal tumors, approximately 50 percent are germinomas True teratomas and gliomas are relatively infrequent Children, adolescents, and young adults males more than females are affected Only rarely does one see a patient with a pineal tumor that has developed after the 30th year of life The pineocytoma and pineoblastoma reproduce the normal structure of the pineal gland These tumors enlarge the gland, are locally invasive, and may extend into the third ventricle and seed along the neuraxis Their growth characteristics resemble those of the germinoma Cytologically, the pineocytoma is a moderately cellular tumor with none of the histologic attributes of anaplasia The tumor cells tend to form circular arrangements, so-called
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Figure 31-13 Hemangioblastoma MRI in the axial plane (left) shows the vascular tumor in the left cerebellar hemisphere Selective left vertebral angiogram (right) de nes a hypervascular nodule with dilated draining veins
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