Cases reported "Neurocytoma"

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1/5. Cerebellar liponeurocytoma. Case report with considerations on prognosis and management.

    BACKGROUND: Cerebellar liponeurocytoma has only recently been recognized as a distinct clinicopathological entity. Although the few cases described in literature support the relatively benign nature of this lesion, optimum treatment strategy and long term behaviour still have to be defined. CLINICAL PRESENTATION: This 61-year-old man came under our observation with unspecific signs of intracranial hypertension and a symptomathology revealing a probable posterior fossa lesion. Imaging studies showed a cerebellar mass lesion with prevalent adipose content. INTERVENTION: Gross total tumour removal was performed. The pathological examination revealed a lesion composed of medulloblastoma-like cells with prominent areas of lipidisation. A review of literature has been made in an attempt to investigate on recurrence patterns, importance of the extent of removal and the usefulness of radiotherapy. CONCLUSION: The few cases reported in literature so far seem to confirm the relatively benign nature of cerebellar liponeurocytoma. No radiotherapy was given in our case and the reasons for this decision are reported.
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2/5. Cerebellar liponeurocytoma: a case report and review of the literature.

    Cerebellar liponeurocytoma has recently been recognised by the 2000 world health Organisation classification of tumours of the central nervous system as a distinct clinicopathologic entity. To our knowledge, 18 cases have been reported so far, under different names, such as "lipomatous medulloblastoma, lipidized medulloblastoma, neurolipocytoma, medullocytoma and lipomatous glioneurocytoma". The new classification included cerebellar liponeurocytoma in the category of glioneuronal tumours grade I or II because of its favourable clinical behaviour. The origin and nature of the lipomatous component have been matter of debate and make this tumour entity puzzling. We describe a new case of liponeurocytoma removed from the left cerebellar hemisphere of a 38-year-old-woman. The patient showed unspecific signs of intracranial hypertension and symptoms suggesting a posterior fossa lesion.
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3/5. Extraventricular neoplasms with neurocytoma features. A clinicopathological study of 11 cases.

    The clinicopathological features of a series of neuronal and mixed neuronal and astrocytic neoplasms of the CNS are described. patients were aged 5 to 63 years. Six cases were composed predominantly of small round cells with clear cytoplasm resembling central neurocytoma but lacked the characteristic intraventricular location of that tumor. The remaining five cases had similar neurocytomatous features associated with a benign astrocytic component. Ganglion cells and hyalinization vessels were observed in both groups. The growth fraction evaluated with monoclonal antibody Ki67Mib1 was low, ranging from 1 to 1.5%. Immunohistochemical detection of synaptophysin played a crucial role in identifying the neuronal nature of these neoplasms and was instrumental in distinguishing them from oligodendrogliomas, with which they are readily confused. The neuronal nature of the oligodendroglial-like cells was confirmed ultrastructurally in one case. The present cases, together with others reported previously, suggest that neoplasms of the CNS with "neurocytic" components are more frequent than generally assumed and expand the morphologic spectrum of neuronal and mixed neuronal-glial tumors. Except for one patient who died postoperatively, all patients were alive at follow-up ranging from 6 to 80 months.
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4/5. neurocytoma/rhabdomyoma (myoneurocytoma) of the cerebellum.

    An unusual case of cerebellar neurocytoma with rhabdomyomatous differentiation in a 6-year-old boy is reported. Immunocytochemical and ultrastructural features of the tumour were studied. Abortive synapse formation, the presence of clear vesicles and synaptophysin immunoreactivity of the tumour cells indicated its intermediate neuronal differentiation, while the presence of myoblasts and myotubes and immunolabelling by desmin confirmed the rhabdomyomatous differentiation. In addition, the mesenchymal cells variably expressed neurofilament protein and glial fibrillary acidic protein, suggesting inductive interaction between the neuroectodermal and ectomesenchymal elements and persistence of the pleuripotential nature of the cells along the rhombic area of the brain stem.
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5/5. Central neurocytoma presenting with intratumoral hemorrhage 15 years after initial treatment by partial removal and irradiation.

    A 50-year-old male presented with a central neurocytoma with intratumoral homorrhage. He had undergone partial removal of the tumor and postoperative radiation therapy 15 years previously. He was followed as an outpatient after the initial treatment, and there was no evidence of regrowth of the residual tumor. Removal of the hematoma and biopsy of the tumor were performed. Abnormally developed blood capillaries in the tumor may have undergone disturbances of circulation caused by irradiation which resulted in bleeding. patients with partially resected central neurocytomas which have low proliferative potential may not require radiation therapy, due to the benign nature and the risk of delayed complications of irradiation including intratumoral hemorrhage.
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