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Is polar spongioblastoma a tumor entity?

The distribution of cells in a parallel fashion with palisades of nuclei is common in neuroepithelial tumors. The authors have selected 16 such tumors from their series for study, as examples of different neuroepithelial oncotypes containing palisades of nuclei: three ependymomas, three hemispheric pilocytic astrocytomas, three oligodendrogliomas, three medulloblastomas, three cerebellar astrocytomas, and one central neuroblastoma. In two additional tumors, affecting a 12-year-old girl and a 51-year-old woman, this feature was present in the entire surgical specimen and the diagnosis was consistent with a polar spongioblastoma. This diagnosis applies in the literature to rate tumors of childhood and adolescence, both malignant and with embryonal features. In one specimen, a clear ependymomatous feature was found in a remote area of the tumor and in the other there were ultrastructural characteristics of neuroblastoma. Nuclear palisades can be found as local architectural features in many neuroepithelial tumors. The rare tumors diagnosed as polar spongioblastoma, according to published criteria, correspond to ependymomas and neuroblastomas. Polar spongioblastoma does not exist as a tumor entity.

Astrocytoma↗

An immunohistochemical study of the primitive and maturing elements of human cerebral medulloepitheliomas.

Four examples of human cerebral medulloepithelioma were studied immunohistochemically with a panel of antibodies and antisera to neuronal and glial proteins. The tumors, in addition to primitive medullary epithelium, contained areas of neuroblastic, ganglionic, astrocytic, ependymoblastic and ependymal differentiation, and in one tumor, areas resembling polar spongioblastoma. Tumor cells throughout the primitive medullary epithelium displayed focal immunoreactivity for vimentin, glial fibrillary acidic (GFA) protein and for the neuron-associated class III beta-tubulin isotype. Neuroblasts showed immunoreactivity for the class III beta-tubulin isotype, microtubule-associated protein 2 and neuron-specific enolase. Immunoreactivity for neurofilament epitopes and synaptophysin was detected in areas of ganglionic differentiation and coincided with the demonstration of neurofibrils in Bielschowsky's silver impregnations. Vimentin was the only marker detected in ependymoblastic and ependymal rosettes or in areas of polar spongioblastoma, as well as in mesenchymal cells. The results indicate that, even in very primitive neoplastic neuroepithelium, immunocytochemical evidence of early commitment of some of the cells to a neuronal or glial lineage can be demonstrated. The neuron-associated class III beta-tubulin isotype appears to be one of the earliest markers indicative of neuronal differentiation in normal and neoplastic primitive neuroepithelium.

Adult↗

Brain tumor as a second malignant neoplasm following neuroblastoma stage IV S.

A rare brain tumor (spongioblastoma polare) occurring 7 years after treatment of neuroblastoma stage IV S is reported. The literature concerning the occurrence of a second cancer in children exposed to mutagenic therapy for their initial tumor is reviewed, and genetic and environmental factors are discussed. Diminishing aggressiveness of the treatment in childhood cancer with good prognosis should be considered. Continuous follow-up of children cured of cancer is warranted.

Abdominal Neoplasms↗

December 1996--cervicomedullary tumor.

A cervicomedullary tumor in a 22-month-old female with an eight-month history of symptoms showed the histological features of a pilocytic astrocytoma but had many areas of palisading cells identical to those described in polar spongioblastomas. The tumor recurred 18 months later and showed predominately pilocytic features. The case is used to discuss polar spongioblastomas and tumors which can histologically simulate them.

Astrocytoma↗

[Glioblastosis].

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Brain Neoplasms↗

Evolution and outcome in malignant astroglial neoplasms of the cerebellum.

Malignant astroglial neoplasms of the cerebellum are rare and the clinical behavior of these tumors is unpredictable. The authors describe the histological characteristics and clinical outcome in 10 patients; eight had malignant cerebellar astrocytomas, and one each had an astroblastoma and a true polar spongioblastoma. Malignant glial tumors involving the cerebellum usually behave very aggressively. Cerebellar astroblastomas and polar spongioblastomas are too rare to predict their natural history. Some hypotheses are proposed concerning the evolution and interrelationship of malignant glial tumors of the cerebellum.

Adolescent↗

Intracranial tumors of children.

A report is made on 323 intracranial tumors of children reported to the Cancer Registry in Finland in 1958-1967. The most common sites were the cerebellum, cerebral hemispheres and brain stem. 54% of the tumors were in the posterior fossa. The most common histological types were medulloblastoma, cerebellar astrocytoma and ependymoma. The incidence on the basis of this series was 2.4/10(5)/y. The general outlook for intracranial tumors in childhood was not very good. After a follow-up time of from 5 to 16 years, 31% of the patients were alive. The best prognosis was in cerebellar astrocytomas and cerebral spongioblastomas. In some tumors of the thalamus and third ventricle remissions for over 10 years were achieved by shunt and radiotherapy alone.

Adolescent↗