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PubMedDate not supplied

Cerebellar primitive neuroectodermal tumor with multipotent differentiation in a family with von Hippel-Lindau disease. Case report.

A family with von Hippel-Lindau disease (vHLD) is presented. Three family members suffered from typical cerebellar hemangioblastomas. Another family member presented with a cerebellar neoplasm of different histology. Detailed histological analysis revealed a primitive neuroectodermal tumor (PNET) with neuronal, glial, myoid and ependymal differentiation. This is apparently the first description of the association of vHLD and a cerebellar PNET with multipotent differentiation.

Adolescent↗
PubMed1978

Cerebellar hemangioblastoma: computed tomographic, angiographic and clinical correlation in seven cases.

Seven patients with histologically confirmed cerebellar hemangioblastomas are reviewed in order to establish computed tomographic (CT), angiographic and clinical characteristics and correlations. Noncontrast CT scans demonstrated most cystic lesions but did not always depict nodules on the cyst wall and small solid tumors. Contrast-enhanced CT scans demonstrated the solid and cystic types of tumors but in some cases angiography better delineated them and the presence of nodules. The CT appearance reflected the pathologic characteristics, which included vascular channels, cyst formation and nodules. Solid tumors enhanced homogeneously and could not be definitively distinguished from other cerebellar neoplasms by CT alone. One atypical undifferentiated tumor appeared as a solid mass with a necrotic center. The CT appearance of associated retinal angiomatosis is also described. Hydrocephalus, gait or appendicular ataxia was the initial clinical presentation in these patients.

Adolescent↗
PubMed1992

Large-cell medulloblastomas. A distinct variant with highly aggressive behavior.

We present four cases of infantile cerebellar neoplasms composed of cells with large vesicular nuclei with prominent nucleoli. All four cases were strongly immunoreactive for synaptophysin, and one case showed immunoreactivity for neurofilaments. Filter hybridization for N-myc and c-myc oncogenes showed a 27-fold c-myc amplification in one case. The cytogenetic analysis in this case showed Double-Minutes and isochromosome 17q. An intracerebral xenograft in nude mice obtained from one such tumor showed a similar morphology to that of the original tumor as well as strong immunoreactivity for synaptophysin and neurofilaments. All the neoplasms were characterized by highly aggressive behavior leading to early cerebrospinal fluid dissemination despite radiotherapy and chemotherapy. We conclude that large-cell medulloblastoma represents a distinct and more aggressive variant of medulloblastoma that requires more aggressive therapy.

Blotting, Southern↗
PubMed2003

Cerebellar liponeurocytoma with an unusually aggressive clinical course: case report.

OBJECTIVE AND IMPORTANCE: Liponeurocytomas are rare cerebellar neoplasms in adults, with benign histological features and a favorable clinical prognosis. Current clinical opinion is based on a total of less than 20 published cases and suggests that gross total resection and long-term follow-up monitoring, with possible additional surgery and radiotherapy for treatment of recurrent tumors, represent the best treatment approach for this relatively benign tumor type. CLINICAL PRESENTATION: A 51-year-old Caucasian woman presented with worsening unsteady gait and headaches, suggesting increased intracranial pressure. INTERVENTION: The patient underwent subtotal resection of a cerebellar liponeurocytoma, followed by fractionated radiotherapy (total dose of 54 Gy). She experienced a local recurrence of the tumor 12 months later and underwent additional surgery for removal of the cerebellar mass. A second recurrent tumor was diagnosed on magnetic resonance imaging scans 3 months later and was surgically resected. The tumor histological findings were consistently devoid of atypical features, apart from leptomeningeal invasion noted in the first surgical specimen. CONCLUSION: This unusual case demonstrated an atypical clinical course of a highly aggressive and radiation-resistant tumor, despite the consistent absence of aggressive histological features. Cerebellar liponeurocytomas may not be as benign as the current literature and typical low-grade cytological and histological features suggest.

Cerebellar Neoplasms↗
PubMed1978

Fine structure of a cerebellar neuroblastoma.

A cerebellar neoplasm of an 18-month-old boy was examined with both the light and electron microscopes. The diagnosis of neuroblastoma was made on the basis of the presence of numerous synaptic vesicles in the great majority of cell processes and the occasional complete synapses within the tumor tissue.

Cerebellar Neoplasms↗
PubMed1998

Tumor devascularization by intratumoral ethanol injection during surgery. Technical note.

Preoperative reduction in tumor vascularity has been accomplished previously by selective catheterization of tumor vessels and delivery of occlusive materials. The results of percutaneous infusion of vertebral hemangiomas and other vascular lesions led the authors to speculate that rapid devascularization of tumors by direct injection of ethanol (ETOH) could be used to reduce bleeding and facilitate resection during surgery. Thus, the use of intratumoral injection of ETOH and its effects on tumor hemostasis and resectability were examined. Four patients received direct injection of ETOH into either a spinal epidural (two renal cell carcinomas and one rhabdomyosarcoma) or a large cerebellar neoplasm (hemangioblastoma). Intraoperative perfusion of the tumors with ETOH produced immediate blanching and devascularization and enhanced visualization and resection. Incremental tumor devascularization is achieved by careful injection of small amounts of ETOH directly into the lesion, producing immediate and complete regional tumor devascularization. Use of this technique reduces intratumoral bleeding and enhances the ease and effectiveness of resection.

Blood Loss, Surgical↗
PubMed2000

Cerebellar neuroblastoma in an infant.

A cerebellar neoplasm in an 8-month-old boy is reported. While this tumour was composed of small cells and had regions resembling desmoplastic medulloblastoma, it showed ultrastructural neuronal characteristics including bundles of microtubules in the cell processes, numerous synaptic vesicles, and occasional abortive or complete synapses. These characteristic features warranted the diagnosis of a neuroblastoma of the cerebellum. The nature of this rare intraparenchymal tumour in infants is also briefly discussed.

Cerebellar Neoplasms↗
PubMedDate not supplied

[Supratentorial primitive neuroectodermal tumor: a single center experience and comparison with the literature].

Supratentorial primitive neuroectodermal tumors (stPNETs) are malignant tumors. We saw within three years six children with stPNETs. In four of the six children radical resection could be achieved. All had craniospinal irradiation and chemotherapy according to the HIT-91 protocol. The two children with incomplete resection died due to tumor progression after 7 and 10 months. Two of the 4 children with complete tumor resection had local relapses 8 months after diagnosis and died after 14 and 18 months. One child had a diffuse meningeal relapse 12 months after diagnosis. Despite (high-dose) systemic chemotherapy and intraventricular mafosfamide, he died 21 months after diagnosis due to tumor although remission could be achieved. Only one child is still in remission 86 months after diagnosis.

Brain Neoplasms↗
PubMed2006

Lhermitte-Duclos disease and Cowden's syndrome. Report of two cases.

OBJECTIVE AND IMPORTANCE: Lhermitte-Duclos disease, or dysplastic cerebellar gangliocytoma is a rare entity characterized by a hamartomatous lesion in the posterior fossa. Cowden's syndrome, or hamartoma-neoplasia syndrome is a rare underdiagnosed autosomal dominant genodermatosis with high incidence of malignant tumors. Several recent reports suggest that Lhermitte-Duclos disease may be a component of Cowden's syndrome. CLINICAL PRESENTATION: We report two cases of Lhermitte-Duclos and Cowden disease occurring in adult patients. A 40-year-old woman had symptoms of raised intracranial pressure and macrocephaly. She displayed the stigmata of fibrocystic breast disease, thyroid goitre. Clinical examination showed mucocutaneous lesions. Her mother, brother and uncle had manifestations of Cowden's disease. An asymptomatic 38-year-old male had bilateral optic nerve drusen related to a cerebellar neoplasm. He exhibited manifestations of Cowden's syndrome and his familial history confirmed this hypothesis. INTERVENTION: The first patient was operated on for Lhermitte-Duclos disease. A conservative strategy was performed for the second patient and the clinical and imaging follow-ups were uneventful over 5 years. CONCLUSION: We stress the possibility that Lhermitte-Duclos and Cowden disease might be a peculiar form of phakomatosis. A conservative strategy can be chosen without neurological signs because of slow tumor growth. However, these patients should be carefully examined and followed up because of the risk of future malignancy.

Adult↗
PubMedDate not supplied

Apparent diffusion coefficients for differentiation of cerebellar tumors in children.

BACKGROUND AND PURPOSE: Diffusion-weighted imaging (DWI) and apparent diffusion coefficient (ADC) maps provide information at MR imaging that may reflect cell attenuation and integrity. We hypothesized that cerebellar tumors in children can be differentiated by their ADC values. METHODS: Brain MR imaging studies that included ADC maps were retrospectively reviewed in 32 patients with histologically proved cerebellar neoplasm. There were 17 juvenile pilocytic astrocytomas (JPA), 8 medulloblastomas, 5 ependymomas, and 2 rhabdoid (atypical teratoid/rhabdoid tumor [AT/RT]) tumors. Absolute ADC values of contrast-enhancing solid tumor regions and ADC ratios (ADC of solid tumor to ADC of normal-appearing white matter) were compared with the histologic diagnosis. ADC values and ratios of JPAs, medulloblastomas, and ependymomas were compared by using a 2-tailed t test and one-way analysis of variance (ANOVA). RESULTS: ADC values were significantly higher in pilocytic astrocytomas (1.65 +/- 0.27) (mean +/- SD) than in ependymomas (1.10 +/- 0.11) (P = .0003) and medulloblastomas (0.66 +/- 0.15) (P < .0001). Ependymomas demonstrated significantly higher ADC values than medulloblastomas (P = .0005). The observed differences were statistically significant on ANOVA (P < .001). ADC ratios were also significantly different among these 3 tumor types. AT/RT ADC values were similar to medulloblastoma. The range of ADC values and ratios within JPAs and ependymomas did not overlap with that of medulloblastomas. CONCLUSION: Assessment of ADC values of enhancing solid tumor is a simple and reliable technique for preoperative differentiation of cerebellar tumors in pediatric patients. Our cutoff values of >1.4 x 10(3) mm(2)/s for JPA and <0.9 x 10(3) mm(2)/s for medulloblastoma were 100% specific.

Adolescent↗
PubMed1983

[The neuroblastoma of the cerebellum].

A report is given demonstrating the maturation of a neuroblastoma of the cerebellum and its transformation into a ganglioglioma. In a male infant statomotor retardation was diagnosed at the age of 6 months. 9 months later, he developed symptoms indicating a cerebellar neoplasm. Craniotomy showed a vermian tumor which was classified histologically as a neuroblastoma. 51/2 years after the first operation, a control CT scan revealed a recurrence which showed light and electron microscopically the structure of a ganglioglioma. The child is now 8 years old and develops relatively well. On the basis of this observation and of the limited data published in the literature, it is suggested that, with the aid of light microscopy, it is possible to distinguish neuroblastomas from common medulloblastomas what is of practical importance because neuroblastomas seem to have a better prognosis. However, differential diagnostic criteria need further precision. Problems of the nosological placement of neuroblastomas and their histogenetic relationship to medulloblastomas are discussed. The statement of the WHO Classification of Tumors of the CNS that neuroblastomas correspond to grade IV cannot be accepted generally.

Cerebellar Neoplasms↗
PubMed1977

Primary position upbeat nystagmus. A clinicopathologic study.

Eye movements were studied with electro-oculography in a patient with primary position, large amplitude, upbeat nystagmus. The upbeat nystagmus increased in amplitude on upward gaze, decreased on downward gaze, and was not altered by loss of fixation. The patient could not produce smooth pursuit movements upward or to the left, but had normal saccadic and vestibular induced eye movements in all directions. At necropsy, a low grade glioma was found involving primarily the medulla and caudal pons. The inferior olives and prepositus hypoglossal nuclei were diffusely infiltrated with tumor. These results suggest (1) primary position upbeat nystagmus is due to a defect in the upward smooth pursuit system, (2) the lower brain stem at the level of the inferior olives and nucleus prepositus hypoglossi is important in the mediation of vertical pursuit, and (3) primary position upbeat nystagmus can result from damage to several nuclei and interconnecting pathways in the caudal brain stem and midline cerebellum involved in control of vertical smooth pursuit.

Ataxia↗