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At least 19 recordsLinked to original sources

Medullocytoma (lipidized medulloblastoma). A cerebellar neoplasm of adults with favorable prognosis.

This study describes three cases of neuroectodermal cerebellar neoplasms occurring in adults, characterized by a monomorphic population of round cells with scanty cytoplasm and focal areas of lipid accumulation. Astrocytic and neuronal differentiation was confirmed in these cells by glial fibrillary acidic protein and synaptophysin immunoreactivity. Electron microscopy performed in two cases showed neuritic processes, synapses, and dense-core granules. Patients included two men and one woman, and the age at diagnosis was 36, 37, and 57 years, respectively. Two patients refused any postoperative treatment. One of these had two surgically removed recurrences after 10 and 11 years and died postoperatively from intracranial hemorrhage. The second had two recurrences after 10 and 15 years and is alive and in good health at the last follow-up. The third patient received postoperative radiotherapy and is alive and well after 2 years. Review of the literature revealed seven cases of cerebellar neoplasms with histological features similar to those observed in our series. These lesions have been considered a variant of medulloblastomas. The age of patients ranged from 42 to 77 years (mean age, 51 years); four were women, 3 men. Follow-up information available in two cases indicates a 5-year survival with surgery alone. These data indicate that these cerebellar neuroectodermal neoplasms have morphologically unique features and indolent biologic behavior that distinguish them from the highly aggressive medulloblastoma; the term medullocytoma for this form is suggested.

Adipocytes↗

[A specific organelle in the vascular endothelial cells of cerebellar neoplasms (author's transl)].

A "rod-shaped tubulated body" (tubular body) was first described by Weibel and Palade in the vascular endothelial cells of various organs in both man and animals. This is now considered to be an organelle specific to the endothelial cell, but its function is still unknown. Both in experimental and human pathology this organelle has been observed more often in either seemingly young or abnormal endothelial cells of the blood vessels in tissue regeneration, inflammation, brain tumors among others. This report deals with ultrastructural study of two surgical cases of cerebellar neoplasm, in which the vascular endothelium was examined for a tubular body. The first case was a 12-year-old boy with cerebellar hemangioblastoma, and the second a 36-year-old female who had a history of renal cell carcinoma removed approximately 5 years previously. Histological diagnosis of the cerebellar tumor in the latter case was indetermined, because a part of the tumor consisted of clear cells suggestive of clear cell carcinoma and another part of well developed endothelial cells and vascular channels apparently indicative of hemangioblastoma. The findings of the ultrastructural study were rather compatible with that of renal cell carcinoma metastatic to the cerebellum although inconclusive. The tubular body observed in the endothelial cells of those tumor vessels consisted of a membrane-limited round, oval or elongated shaped intracytoplasmic body which contained tubules of 170 to 200 A outer diameter with approximately 50 to 60 A thickness. The tubules were arranged mostly in a parallel fashion along their long axis. In the first type of tubular body they were embedded in a relatively pale matrix, and in the second their arrangement appeared to be more compact. The third tubular body, so far undescribed in human endothelial cells except in our previous communication, showed an irregularly and markedly enlarged matrix, surrounded by a limiting membrane which was occasionally observed connected with either a coated vesicle or cytoplasmic membrane. Abundant tubules were intermingled without showing a particular arrangement. Morphological and functional significance of the third type tubular body is unknown, but it might represent a pathological change of a tubular body in cerebellar neoplasms. These findings might give us some clues in understanding a tumor angiogenesis.

Adenocarcinoma↗

Cerebellar neoplasm of mixed mesenchymal and neuroepithelial origin. Case report.

A 3 1/2-year-old boy had a neoplasm of mixed mesenchymal and neuroepithelial origin in the cerebellar vermis. The tumor was composed of rhabdomyosarcoma and medulloblastoma. There was additional differentiation within the neuroepithelium, including neoplastic neurons, neuroblasts, astroglia, oligodendroglia, ependymal cells as well as foci of glioblastomatous transformation. It is suggested that this combined tumor arose from the neural crest or combined neural crest and neural tube. The rhabdomyosarcoma could have arisen from the mesenchymal portion of the neural crest, and the medulloblastoma from the neuroectodermal part of the neural crest or from the neural tube itself. The two tumor types formed a neoplasm of mixed mesenchymal and neuroepithelial origin.

Cerebellar Neoplasms↗

[Developmental Gerstmann syndrome associated with cerebellar neoplasm. Report of a case and review of the literature].

A Developmental Gerstmann syndrome in a 7 years-old-boy with hyperactivity, short attention span, acalculia, agraphia, right-left confusion, finger agnosia and constructional apraxia is reported. An initial trial with methylphenidate was done with good improvement regarding hyperactivity, attention span and constructional apraxia. In the clinical course developed a cerebellar syndrome and intracranial hypertension. Surgical exploration of the cerebellum discovered a medulloblastoma. The author believes that this is the first described association of Developmental Gerstmann syndrome and cerebellar neoplasia. The clinical findings, the etiology and the topography of the Gerstmann syndrome are discussed.

Cerebellar Neoplasms↗

HPV in situ hybridization with catalyzed signal amplification and polymerase chain reaction in establishing cerebellar metastasis of a cervical carcinoma.

We report an unusual case of cerebellar metastasis from a cervical adenosquamous carcinoma in which molecular techniques assisted in establishing the correct diagnosis. The patient was a 43-year-old woman with surgically unresectable cervical carcinoma diagnosed 2 years before presenting with neurological symptoms. A magnetic resonance imaging scan showed a large, enhancing cerebellar lesion with significant brain stem compression. The excised cerebellar tumor resembled a small cell carcinoma and was initially not thought to be a metastasis from the cervical adenosquamous carcinoma. In situ hybridization with catalyzed signal amplification and polymerase chain reactions with primers specific for human papilloma virus (HPV) types 16 and 18 were used to determine the relationship between the cervical and the cerebellar neoplasms. A positive signal was present in the nuclei of both neoplasms by in situ hybridization using HPV16/18 DNA probes. Polymerase chain reaction revealed the presence of HPV-18 DNA sequences in the cervical and cerebellar neoplasms confirming that the cerebellar neoplasm was a metastasis from the cervical primary.

Adult↗