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We report two patients with spinal epithelioid hemangioendothelioma (EH), an uncommon vascular, potentially malignant tumour. Neurological signs, diagnostic images, surgical techniques and complications, histology and the role of adjuvant therapy are discussed. Primary manifestation of EH of the vertebral column is rare. Thorough preoperative clinical and radiological workup, radical surgical excision, and close postoperative follow-up are recommended.

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The nevus sebaceus syndrome (NSS) is a neurocutaneous disorder characterized by unilateral hyperplasia of skin appendages and skeletal hemihypertrophy, hemimegalencephaly, or hemiatrophy along with disabling seizures. Despite the proneness of the dermal stigmata to eventually undergo neoplastic transformation, the malformative lesions of the central nervous system rarely evolve into frank tumors. We present the case of a 10-year-old girl with left-sided sebaceus nevi, ipsilateral enlargement of the skull, and a desmoplastic neuroepithelial tumor (DNET) in the right fronto-parietal area of the brain. The tumor was removed by surgery. Histologically, it corresponded to a mitotically active small-cell anaplastic astrocytoma with genuine desmoplasia. Investigative methods included immunohistochemical positivity for glial fibrillary acidic protein, lack of expression of neuronal markers, and ultrastructural documentation of sheaths of basal lamina and collagen around tumor cells. A survey of the literature of brain tumors associated with NSS revealed two cases of histologically verified pilocytic astrocytomas, and one each of a choroid plexus papilloma, a mixed glioma, and a meningioma, as well as a subependymal giant cell astrocytoma--the latter possibly in an overlap syndrome of NSS and tuberous sclerosis. We hypothesize that the tumor described herein, one involving both atypical differentiation and enhanced growth potential, is paradigmatic of neuropathological events to be expected in the NSS.

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Cholesterol-granuloma is a pseudotumoral mass that is believed to enlarge by a self-perpetuating sequence of repeated hemorrhages and reparative tissue reaction. Albeit an almost ubiquitous phenomenon throughout the body, cholesterol-granuloma has recently been appreciated as a distinctive lesion mimicking or associated with craniopharyngiomas. Upon review of a surgical series of 15 purported craniopharyngiomas, the authors identified 3 such occurrences. All were characterized by a predominance of slit-like cholesterol clefts with multi-nucleated giant cells embedded in a fibrotic stroma permeated with lipid laden macrophages, lymphocytes, as well as organizing hemorrhage. Non-craniopharyngioma specific cuboidal epithelium was present in one case. The mean age of patients--all males--with cholesterol-granuloma was 26 years, and all but one had an intrasellar tumor component. Clinical symptoms referrable to hypopituitarism predominated. At variance with the above, patients with adamantinomatous or papillary craniopharyngiomas were 23.5 and 46 years old, respectively, and presented with neurological deficits or ones due to hypothalamic involvement by their tumors. With marginal central nervous tissue present in 53 percent of the specimens, 75 percent of adamantinomatous craniopharyngiomas, but only 12 percent of cholesterol-granulomas showed invasive growth. At present cholesterol-granulomas are conceived as a clinicopathologically distinctive lesion of uncertain origin. They most probably represent a clinically relevant entity in the ontogenesis of adamantinomatous craniopharyngiomas with predisposing factors yet to be elucidated.

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The dysembryoplastic neuroepithelial tumor is a benign central nervous system neoplasm of children and young adults manifesting almost exclusively in complex partial seizures. The authors report the case of an 8-year-old boy presenting with characteristic clinical and radiologic features who subsequently underwent surgery. Light microscopic, immunohistochemical and ultrastructural traits of the tumor are described demonstrating pluripotential differentiation of tumor cells and architectural features suggesting a dysontogenic lesion. A brief literature review on the biology and histologic diagnosis of the dysembryoplastic neuroepithelial tumor is provided. Although this uncommon tumor accounts for only a minority of intracranial neoplasms, its pathogenetic role has to be considered in the differential diagnosis of temporal lobe epilepsies. This is the first report on this recently described, rare neoplastic condition in Hungary.

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The authors present the policy they have worked out for hydrocephalus patients with special reference to the pressure measurement and test methods and to rCBF, SPECT and transcranial Doppler sonography (TDC) studies. For diagnosis, the protocol proposed by Gjerris and Borgesen was followed in 75 cases: besides other methods (CT, radionuclide cisternography, MRI) the intracranial pressure waves routinely recorded and analyzed by means of ventricular catheters for 24 h. The patients were roughly divided into groups in terms of diagnosis, baseline pressure, compliance, results of infusion tests and of surgery. In 13 patients the investigations were supplemented by rCBF SPECT and in 42 patients by TCD studies before and after CSF shunting or withdrawal to analyze the acute effects on cerebral circulation. Clinical follow-up shows that need for shunting was indicated fairly well by the common results of baseline ICP, compliance and infusion loading. The rCBF SPECT studies revealed a significant increase of the cerebral perfusion at the basal ganglia after shunting while, on the basis of CBF velocity changes three types of vasoregulatory response could be defined with TCD. In our hands, monitoring of the pressure and craniospinal capacity has proved to be a valuable aid in decisions on surgery; however, for a more precise (and beneficial) appreciation of whether surgery is indicated the vasoregulatory responses should also be taken into account in future.

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