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Results for “Neuroectodermal Tumors, Primitive, Peripheral”

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At least 937 records · Page 52Linked to original sources
PubMedDate not supplied

Neuroendocrine tumors of the nasal cavity: an ultrastructural and morphometric study of 24 cases.

A series of 24 small cell nasal tumors with neuroendocrine differentiation was studied by electron microscopy, and the numbers of secretory granules and cell junctions were assessed. To investigate the relationship between the extent of dendrite formation and the behavior of the tumors, the size of the tumor cells and the area occupied by dendritic processes were determined for each tumor by morphometric analysis performed on low-magnification electron micrographs. A positive correlation was demonstrated between the dendritic area index and the survival of the patients (P = 0.017). Neither the number of secretory granules nor the frequency of cell junctions was prognostically significant.

Adolescent↗
PubMedDate not supplied

Appraisal of the comparative utility of immunohistochemistry and electron microscopy in the diagnosis of childhood round cell tumors.

To provide an objective assessment of the comparative utility of fluorescence- and peroxidase-based immunohistochemistry and electron microscopy, an observer blinded study was conducted under realistic study conditions utilizing a large sampling of poorly differentiated pediatric round cell tumors. Working independently, using a single ancillary technique of particular expertise, each of three investigators attempted to render a specific diagnosis with regard to 50 diagnostically challenging tumors. The results were compared against the subsequent "file diagnosis" established by consensus with all relevant information made available. A grading scheme was applied wherein points were awarded based on the accuracy and confidence of diagnosis. A comparative efficiency rating, expressed as a percentage, was formulated by dividing the number of points awarded each technique by the total number of points theoretically available. Electron microscopy proved superior overall, with an efficiency rating of 89%. Immunoperoxidase and immunofluorescence studies yielded efficiency ratings of 71 and 61%, respectively. Used in combination, the techniques achieved an efficiency rating of 95%. Application of these ancillary techniques resulted in a revision of the provisional diagnosis in 11 of 50 cases, and left only two cases without a firm specific diagnosis.

Adolescent↗
PubMed1993

Esthesioneuroblastoma. A report of seven cases.

The biologic behavior of esthesioneuroblastoma in seven patients, treated from 1978 to 1989, is reviewed. The patients were initially treated with surgical resection (2 cases), radiation alone (1 case) or a combination of radiation and surgery (4 cases). The radiation dose ranged from 30 to 62 Gy. Operations were performed via a transmaxillary approach (2 cases), lateral rhinotomy approach (3 cases) and craniofacial approach (1 case). Four of the seven patients experienced local recurrence, occurring after disease-free intervals as long as 6 years. The other three patients died of distant metastasis within one year after initial treatment. The effectiveness of radiation therapy varied, and in some patients a dose of 60 Gy was not enough to control microscopic disease. One patient developed bone marrow metastases which was fatal due to the ensuing pancytopenia. One patient developed a brain metastasis. Hyams' histopathologic staging of the tumor appeared to be related to prognosis.

Adolescent↗
PubMed1986

Olfactory neuroblastoma (esthesioneuroblastoma) in a 2-year-old boy.

A 2-year-old boy presented with sudden blindness and epistaxis resulting from olfactory neuroblastoma with intracranial extension. This disease is uncommon in children, with only 27 cases reported in patients less than 17 years of age. This article reviews the clinical and histological features of this tumor and discusses treatment. Therapy has generally consisted of surgery and radiation. Our child and 2 others treated with radiation and combination chemotherapy (including cyclophosphamide and anthracyclines) have done well with follow-ups of up to 9 1/2 years. Chemotherapy is an important form of adjuvant therapy in this disease.

Antineoplastic Combined Chemotherapy Protocols↗
PubMed1992

Unilateral benign medulloepithelioma associated with bilateral retinal detachment.

A two-month-old girl presented with a unilateral benign cystic medulloepithelioma from the ciliary body associated with bilateral retinal non-attachment. The authors discuss the histopathologic findings and the pathogenesis of the congenital retinal non-attachment: are these incidental occurrences or do they arise from a common pathogenetic mechanism?

Ciliary Body↗
PubMed1995

Three-dimensional MRI reconstructions of musculoskeletal tumors. A preliminary evaluation of 2 cases.

We have developed a new method for three-dimensional (3D) reconstruction of malignant bone and soft-tissue tumors from MRI. Data from transverse images of T2-weighted images were transferred into a personal computer. The contours of tumors, major vessels, major nerves and bones were outlined, whereupon the 3D data were processed into a 3D display, using a solid model. The determination of tumor volume both before and after preoperative adjuvant therapy was quantified. The 3D images allowed a better understanding of the relationship between the tumor and the major vessels and nerves, thereby aiding in preoperative planning. The images also provided a quantitative, reproducible measurement of the effect of adjuvant therapy.

Adolescent↗
PubMed1980

Intracranial esthesioneuroblastoma associated with unilateral visual loss. Case report.

Esthesioneuroblastoma is a rare tumor that arises from the olfactory mucosa and presents usually as a mass in the nasal cavity. Neurological complications occur in about 20% of these cases. Nine cases have been recorded so far in which the neoplasm manifested initially as an intracranial mass. These cases are reviewed and another patient, presenting with progressive unilateral visual loss, is reported.

Adolescent↗
PubMed1985

Embryonal central neuroepithelial tumors and their differentiating potential. A cytogenetic view of a complex neuro-oncological problem.

The embryonal central nervous system (CNS) neoplasms are reviewed with special reference to their differentiating potential and in the light of current neuro-oncogenetic concepts partly derived from the experimental induction of neural tumors. The conceptual (and, occasionally, practical) distinction between adult-type and embryonal CNS tumors raises a complex problem, because neoplastic transformation essentially involves replicating stem cells in tissues of renewal and because in the human brain such cells are found mostly in the course of CNS development. A cytogenetic scheme is therefore needed to serve as a frame of reference for a classification of embryonal CNS tumors that will account for the different histological entities documented so far and for the range and the restrictions of their differentiating capabilities. Most embryonal CNS tumors can be fitted into such a scheme. The cerebral medulloepithelioma, the cerebral and cerebellar neuroblastomas, the primitive polar spongioblastoma, and the ependymoblastoma show characteristic morphological features and a correspondingly distinctive cellular differentiating potential. The differentiating capabilities of the cerebellar medulloblastoma, the pineoblastoma, and the retinoblastoma are also distinctive, and are apparently determined by the cytogenesis of the area of the CNS in which the tumors originate. The indiscriminate application of a simplistic concept that would include all the so-called "primitive neuroectodermal tumors" into a single neuroepithelial tumor entity is unlikely to bring further understanding to the problem.

Age Factors↗
PubMed2000

Dysembryoplastic neuroepithelial tumor of the cerebellum and brainstem. Case report.

The authors present a case in which dysembryoplastic neuroepithelial tumors (DNETs) occurred in the cerebellum and brainstem of a 44-year-old woman. A magnetic resonance image of the brain revealed multiple cystic lesions in the right cerebellar hemisphere, vermis, tonsil, and brainstem. Partial removal of the tumors was performed. There were gray multinodular gelatinous lesions on the cerebellar hemisphere. Histologically, the tumors exhibited areas of multiple microcystic nodules in the cerebellar white matter, which were composed of oligodendroglia-like cells (OLCs), astrocytes, and neurons. There were multiple, variable nodules in the lesions, lined by OLCs. The adjacent cerebellar cortex displayed dysplastic features. Reduction of granule neurons and dislocation of Purkinje cells into the molecular layer were observed. The pathological profile of this patient agrees with that described by Daumas-Duport, et al., as a "dysembryoplastic neuroepithelial tumor."

Adult↗
PubMedDate not supplied

Esthesioneuroblastoma.

A retrospective review of the clinical records of 31 patients with esthesioneuroblastoma is presented. A bimodal age distribution was noted. The tumor is extremely rare among blacks. Most patients presented with locally advanced disease. However, regional and distant metastases at the time of initial diagnosis are uncommon. Local recurrence at the primary site was very common, and this reflects either the conservative initial surgical treatment employed or the multicentric nature of the tumor. Cervical lymph node metastasis is present in less than 10 percent of patients at the time of diagnosis. The survival rates were better in patients with early stages of disease. From this study, we conclude that the current management of these tumors should consist of a combination of radiation therapy and surgery. Radical resection followed by postoperative radiation therapy appears to be the treatment combination of choice. The five-year survival rate in our series was 52 percent.

Adolescent↗