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At least 19 recordsLinked to original sources
PubMed1983

Neuronal and astrocytic differentiation in human neuroepithelial neoplasms. An immunohistochemical study.

Neuroepithelial neoplasms of childhood were examined immunohistochemically using antibodies against a neurofilament polypeptide and glial fibrillary acidic protein. Ninety-one cases, including 11 controls, were examined. Positively reacting cells, indicating neuronal and glial differentiation, were found in 59 of the 80 tumors. The study supports a neuroepithelial origin for medulloblastomas, central neuroblastomas, and primitive neuroectodermal tumors of childhood. The results also indicate that only a small number of the tumor cells differentiate along either neuronal or glial cell lines.

Astrocytoma↗
PubMed1983

An ultrastructural study of intranuclear rodlets in a malignant extracranial neuroepithelial neoplasm.

This report describes for the first time the presence of intranuclear rodlets in a malignant extracranial neuroepithelial neoplasm. This malignant neoplasm appeared poorly differentiated by light microscopy and presented initially as a diagnostic problem. Electron microscopy revealed neoplastic cells joined by macula adherens-type junctions and containing numerous interdigitating cell processes diagnostic of a neuroepithelial neoplasm. A quantitative analysis of 500 cells revealed the presence of intranuclear rodlets in 5% of the neoplastic cells. The rodlets were composed of individual filaments that measured 7-9 nm in width. An extensive review of the literature revealed the high frequency of reported rodlets in normal neurons, normal paraneurons, neoplasms of the nervous system, and paraneuromas. The significance of this interesting structure to the physiology of the cell is discussed.

Aged↗
PubMed1980

Melanotic mucin-producing neuroepithelial neoplasm of mesencephalon with consideration of similar tumours in different locations.

A six month old boy had a mesencephalic melanotic neoplasm of the neuro-epithelial derivatives which produced mucin. Location in the mesencephalon and production of the mucin have not been described previously for this kind of intracranial tumour. The various cellular elements within the growth are in accord with diverse differentiations of the neuroepithelium of the neural tube, the neuroectodermal part of the neural crest, or both. Derivatives of the neural tube and crest can be melanotic, and some neuroepithelial derivatives (ependyma, oligodendrocyte, and choroidal epithelium) produce mucin. Hence, melanotic neuroepithelial neoplasms with mucinous product can occur. The present tumour is similar to lesions variously designated as melanotic medulloblastoma, retinal anlage tumour, and progonoma. Diverse differentiation of the neuroepithelium of the neural tube, or of the neuroectodermal part of the neural crest, or both would create these variously named tumours. The frequency of melanotic neuroepithelial neoplasms in infancy, and the presence of papillae strongly suggest congental derivation.

Adult↗
PubMed1993

Surgery, hyperfractionated craniospinal irradiation, and adjuvant chemotherapy in the management of supratentorial embryonal neuroepithelial neoplasms in children.

Supratentorial embryonal neuroepithelial tumors are undifferentiated neoplasms. We have used this term in preference to the controversial classification primitive neuroectodermal tumors (PNET). These lesions in children are malignant neoplasms which are usually fatal within 2 years of diagnosis in spite of therapy with surgery, radiotherapy, and chemotherapy. We have adopted an aggressive approach to the treatment of these tumors with surgical resection, hyperfractionated craniospinal irradiation of 30.6-43.9 Gy followed by a tumor boost to a total dose of 50-63.7 Gy, and adjuvant chemotherapy with cyclophosphamide, vincristine, and cis-platinum. We have treated five children, aged 4-18 years, with this approach. In contrast to the results reported in the literature, four children are alive without evidence of tumor from 4.3 to 8.0 years following diagnosis. One has suffered a tumor relapse at 2.3 years following diagnosis but remains alive. The basis of our therapeutic strategy for childhood supratentorial embryonal neuroepithelial tumors and the implications of our clinical results are discussed.

Adolescent↗
PubMed1989

[Radiation and combined treatment of anaplastic neuroepithelial neoplasms].

The data on 645 patients with anaplastic neuroepithelial tumors subjected to radiotherapy, irradiation with radiosensitizers (metronidazole) and chemoradiotherapy are presented. Total-differential brain irradiation was applied: 10-40 Gy on the whole brain and 10-20 Gy on the tumor bed. Fractionation depended on tumor radiosensitivity. The median survival time for anaplastic astrocytoma was 50 +/- 5.6 mos., oligodendroglioblastoma - 42 +/- 7.4 mos., ependymoblastoma - 61 +/- 8.2 mos. and glioblastoma - 22 +/- 1.5 mos. High-dose dynamic fractionation proved to be most effective in cases of glioblastoma. The outcome of glioblastoma was modified neither by radiosensitizers nor by cytotoxic agents.

Antineoplastic Agents↗
PubMed1998

Anti-Hu immunolabeling as an index of neuronal differentiation in human brain tumors: a study of 112 central neuroepithelial neoplasms.

Anti-Hu is a polyclonal immunoglobulin G associated with a syndrome of paraneoplastic sensory neuropathy/encephalomyelitis that principally afflicts patients with small cell lung carcinoma. Anti-Hu antibodies, which identify a family of RNA-binding proteins that are normally neuron restricted and that appear to be integral to neuronal differentiation and maintenance, selectively label the nuclei (and, less strongly, the cytoplasm) of neurons throughout the human neuraxis. Small cell carcinomas of the lung and many neuroblastomas are also labeled. We screened 112 tumors of central neuroepithelial lineage for immunohistochemical evidence of Hu expression with anti-Hu immunoglobulin G that was purified from patient sera and with a recombinant Fab fragment (Fab GLN 495) selected from a patient-derived combinatorial antibody phage display library using a recombinant Hu protein (HuD). Both antibodies uniformly labeled, in addition to native neurons, the nuclei of central neurocytomas (6 of 6) and the neuronal components of "classic" (12 of 12) and desmoplastic infantile (2 of 2) gangliogliomas. Of 33 embryonal tumors, 29 were anti-Hu reactive, including 87% of medulloblastomas (26 of 30). Glial neoplasms (n = 59) were anti-Hu negative save for one "oligodendroglioma" (of 17 oligodendroglial/oligoastrocytic tumors) that may have been an extraventricular neurocytoma. Anti-Hu immunoglobulin G/Fab GLN 495 identifies neoplasms of differentiated neuronal type and embryonal tumors with neuronogenic potential.

Antibodies, Neoplasm↗
PubMed1982

Pigmented olfactory neuroblastoma: a new example of melanotic neuroepithelial neoplasm.

The light and electron microscopic features of a pigmented olfactory neuroblastoma are described. The neuroblastic nature of the tumor was confirmed by the demonstration of axons, some of which originated from the neoplastic cells, and by the presence of dense-core vesicles in the perikarya and processes of the tumor cells. The tumor contained a large amount of pigment, most of which had the histochemical reactions of melanin, but some of which had that of lipofuscin. Electron microscopy failed to demonstrate the presence of melanosomes or premelanosomes: the pigment showed some of the features of neuromelanin, although the fine structural appearances were not typical. The pigment was interpreted in this case as presumably representing a modified catecholamine degradation product, perhaps related to lipofuscin. The presence of melanotic pigment in an example of olfactory neuroblastoma, unreported until now, augments the list of neurogenic neoplasms in which an association with melanin formation has been described. The significance of this association in tumors originating from the nervous system or its coverings needs further investigation, as the nature of pigment appears to be heterogeneous. These tumors may contain either melanosomal pigment or pigment identified as neuromelanin (or a related substance), seemingly regardless of the derivation of the pigment-bearing cells.

Adult↗
PubMed2000

Discrepancies in diagnoses of neuroepithelial neoplasms: the San Francisco Bay Area Adult Glioma Study.

BACKGROUND: Valid and reliable diagnoses of disease are key both to meaningful epidemiologic and clinical investigations and to decision-making about appropriate treatment. One previous study highlighted the lack of precision in diagnosing primary brain tumors in a neuropathology referral practice. The current study explores diagnostic discrepancies in a population-based adult glioma series by hospital of origin, specialty training of the original diagnosing pathologist, and clinical significance. METHODS: To confirm patients' eligibility for the San Francisco Adult Glioma Study, the authors obtained participants' pathology specimens and conducted a uniform secondary neuropathology review. Eligible patients were all adults age 20 years or older newly diagnosed with glioma between August 1, 1991, and March 31, 1994, who resided in 1 of 6 San Francisco Bay Area counties. RESULTS: Overall, the original and secondary diagnoses were the same (concordant) for 352 (77%) of the 457 cases available for study. Twenty-six percent of the cases from community hospitals were discordant, compared with 12% of the cases from academic hospitals P= 0.004. Of the 105 discordant diagnoses, 17 (16%) were determined to be clinically significant, defined as a difference that could significantly alter patient management and/or prognosis. Sixteen of these 17 cases originated at community hospitals, and only 1 originated at a hospital with a neuropathologist. Based on the distribution of review diagnoses, subjects presenting at nonacademic hospitals were more likely than those presenting at academic hospitals to have glioblastoma (61% vs. 52%; P = 0.07). CONCLUSIONS: The percentage of cases with discrepant original and review diagnoses was higher among those originally diagnosed at community hospitals without a neuropathologist than among those originally diagnosed at an academic hospital with a neuropathologist. Clinically significant discrepancies were much more likely to have originated at a community hospital without a neuropathologist. These data highlight the importance of review of brain tumors by a neuropathologist prior to decision-making regarding treatment. A separate implication of this study is that glioma cases selected exclusively from academic or nonacademic institutions in a particular geographic area are unlikely to be representative of all cases occurring in that area.

Adult↗
PubMed1997

[Dysembryoplastic neuroepithelial tumor. Case report].

Dysembryoplastic neuroepithelial tumor (DNT) is a relatively new neuroepithelial neoplasm that was first described by Daumas-Duport et al. in 1988. It was incorporated in the revised World Health Organization classification of brain tumors in 1993. The authors present the first case of DNT reported in the Latin-American literature. A 16-year-old woman had been found at age of five years to have complex partial seizures accompanied by secondary generalization. She was treated with various anti-epileptic drugs but always with incomplete control of seizures activity. Neurological examination was normal. Magnetic resonance imaging demonstrated a lesion in the left temporal lobe. She underwent a craniotomy with complete resection of the tumor. Histological study showed atypical neuroglial proliferation characteristic of DNT. Monoclonal antibody immunostaining for glial fibrillary acidic protein and S-100 protein confirmed the glial component, and neuronal specific enolase and synaptophysin emphasized the neuronal component.

Adolescent↗
PubMed1992

Retinoblastoma, microphthalmia, coloboma, and neuroepithelioma of the pineal body.

A one-month-old infant boy was examined early in life because his mother had bilateral retinoblastoma and his father had bilateral microphthalmia. The ophthalmologist found his right eye was normal size with a coloboma of the iris, choroid, and retina. The left eye was microphthalmic with a coloboma of the uveal tract and retina. A vascularized fluffy white mass in the posterior pole was diagnosed clinically as a retinoblastoma. The tumor regressed with radiation. When the patient was four years of age, a large tumor was found in the region of the pineal recess, causing hydrocephalus and seizures. A biopsy showed an undifferentiated malignant neuroepithelial neoplasm. The patient died within three months of diffuse central nervous system tumor. The unusual findings of a retinoblastoma in a microphthalmic eye with bilateral colobomas and a neuroepithelial neoplasm of the pineal gland are discussed.

Abnormalities, Multiple↗
PubMed1985

Immunolocalization of laminin in neoplasms of the central and peripheral nervous systems.

Laminin is a basement membrane glycoprotein that is expressed in vitro by immature and neoplastic astrocytes. The expression of laminin in vivo was examined immunohistochemically in normal adult brain and 90 neoplasms of the central and peripheral nervous systems. In normal adult brain, laminin was detected in the vasculature, arachnoid, pial-glial membrane, and choroid plexus. The vasculature in all 90 tumors demonstrated intense laminin immunoreactivity. Deposits of laminin were observed at the glioma-mesenchymal junction in several neoplasms, but never between or within neuroepithelial cells. The glial basement membrane often remained intact although surrounded on both sides by invasive glioma or medulloblastoma. However, there was always fragmentation and disruption of the glial membrane in adjacent fields. Laminin expression by tumor cells was observed in 10/10 schwannomas, 9/10 fibroblastic meningiomas, 3/19 nonfibroblastic meningiomas, and 3/6 mixed glioma-sarcomas. Laminin expression in the normal nervous system and in neuroepithelial neoplasms corresponds to regions of recognized basal lamina formation, including the junction between glial and mesenchymal elements. Although invasive gliomas are able to break down the pial-glial basement membrane and gain access to the perivascular or subarachnoid space, this membrane often remains intact late in the invasive process and may represent a partial barrier to tumor invasion. Laminin may be a useful marker for schwannomas, fibroblastic meningiomas, and vascular neoplasms of the nervous system.

Basement Membrane↗
PubMed1988

[Ossified choroid plexus papilloma--case report].

While the calcification has been documented radiologically in 4 - 25 percent of the cases with choroid plexus papilloma, the ossification of choroid plexus papilloma has been reported only in 3 cases on literature. In this paper, we present a case of large ossified choroid plexus papilloma in the right lateral ventricle. A 35-year-old man was admitted with left hemiparesis and headache. Skull X-rays showed an abnormal calcified mass (25 mm x 23 mm x 14 mm) in the right temporal region. CT scan revealed an abnormal high density area protruding into the right lateral ventricle, and a low density area at the right corona radiata. Operation was carried out with a right temporal craniotomy. The mass was yellowish-elastic hard and protruded into the right inferior horn of the lateral ventricle. The histological examination of surgical specimen showed an ossified choroid plexus papilloma with some bone marrow formation and numerous calcification. Calcification of choroid plexus papilloma is found frequently, but ossification is a very rare neuropathological abnormality. Bone and/or cartilage formation in neuroepithelial neoplasms is an unusual event histopathologically. Several mechanisms have been proposed, such as (1) metaplasia of connective tissue, (2) transformation of neuroepithelial cells to mesenchymal tissue, (3) teratomatous nature, (4) heteroplasia, (5) mixed mesenchymal-neuroepithelial nature, and (6) ossification as an end stage of mucoid degeneration. In our case, it was considered that the bone formation resulted from metaplasia of connective tissue of choroid plexus papilloma.

Adult↗
PubMed1979

Cerebral medulloepithelioma. Report of a case with multiple divergent neuroepithelial differentiation.

A cerebral medulloepithelioma, a rare embryonal multipotential central neuroepithelial neoplasm of childhood, is described, bringing to 16 the total number of cases reported in the literature. It is the first example recorded to date which displayed the entire range of differentiation from embryonal to mature cells, of both glial and neuronal lineage (i.e., primitive medullary epithelium, spongioblasts, astrocytes, oligodendroglia, ependymal cells, neuroblasts, and mature ganglion cells). The significance of this tumor as a paradigm of multipotential divergent neuroepithelial differentiation is discussed.

Adolescent↗
PubMed1981

Soft tissue sarcomas of childhood: the differential diagnostic dilemma of the small blue cell.

In its histologic features, embryonal rhabdomyosarcoma (RMS), the prototype of malignant soft tissue tumors in childhood, summarizes the problems associated with the diagnosis of this entire group of neoplasms. Many of the tumors that do not fulfill the criteria for RMS have been designated "sarcomas of uncertain histogenesis." The introduction of the concept of a soft tissue equivalent of Ewing's sarcoma may have eased the semantic anxiety without improving our conceptual understanding. It is thought that the embryonal RMS, Ewing's sarcoma, and other are derived from a primitive mesenchymal cell. Another separate category of "small blue cell tumors" are those which presumably originate from the primitive neuroepithelium. Some of the diagnostic terms applied to this category are "neuroepithelioma," "medulloepithelioma," and "peripheral neuroblastoma." Because most of these tumors are hormonally inactive and electron microscopy is not performed, the diagnosis is infrequently considered or proved. The recently described small cell tumor of thoracopulmonary origin is likely a malignant neuroepithelial neoplasm. Hematopoietic tumors, such as non-Hodgkin's malignant lymphomas, granulocytic sarcoma, and malignant histiocytosis, may appear in the soft tissues as the initial manifestation of these system diseases. A final group of malignant soft tissue tumors are the fibrohistiocytic ones with a biphasic pattern of small round cells and spindle cells. It now has become increasingly difficult for the pathologist to satisfy his clinical colleagues with the diagnosis of "undifferentiated malignant tumor" in a child.

Bone Neoplasms↗
PubMed1985

Composite cerebral neuroblastoma and astrocytoma. A mixed central neuroepithelial tumor.

This report describes an exceptional example of cerebral neuroblastoma in association with neoplastic glial cells, which were frequently arranged in clusters around the supporting blood vessels. The neuroblastic nature of the tumor was confirmed by the presence of numerous dense-core vesicles in the cell processes. The glial nature of the associated cells was confirmed by their positivity for glial fibrillary acid protein by immunoperoxidase staining and by the presence of abundant cytoplasmic filaments of intermediate size on electron microscopy. Of particular interest was the presence of dense-core vesicles in some of the glial cells, and that of a basal lamina underlying the perivascular glial cells. The tumor was interpreted as a composite cerebral neuroblastoma and astrocytoma. No undifferentiated neuroepithelial stem cells could be identified. The cytogenesis of this mixed neuroepithelial neoplasm is uncertain.

Astrocytoma↗