The olfactory neuroepithelial tumors; a review of the literature and report of six additional cases.
Explore the source record for details and available documents.
Humans↗
SEARCH · Search PubMed
Results for “Neuroectodermal Tumors, Primitive, Peripheral”
Search indexed PubMed citations on genomics, clinical trials, systematic reviews and public health. Explore titles, authors and supplied subject terms, then open the PubMed record.
Quote a phrase for an exact phrase match. Source license links do not imply unrestricted reuse.
Congenital retinoblastoma and sarcoma botryoides of the vagina: report of a case.
Explore the source record for details and available documents.
Eye Neoplasms↗
Embryonic ependymoma arising in a cerebral hemisphere.
Explore the source record for details and available documents.
Brain Neoplasms↗
Rhabdomyosarcomatous differentiation in malignant intraocular medulloepitheliomas.
Explore the source record for details and available documents.
Adult↗
Dopamine-B-hydroxylase and catecholamines in an olfactory esthesioneuroma.
In a case of olfactory esthesioneuroma, of which an unfixed specimen and blood sample were available, we demonstrated immunohistochemically and biochemically the presence of dopamine-B-hydroxylase and catecholamines in low concentration. This result indicates that this nasal tumor is probably of sympathetic origin, in whole or in part, and provides a new aid in diagnosis. Furthermore, biochemical assay of dopamine, V.M.A., and H.V.A. excretion in urine could perhaps bring information of diagnostic and prognostic value before and after treatment.
Adult↗
Olfactory neuroblastoma. A clinical analysis of 17 cases.
Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal acvity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.
Adolescent↗
Ultrastructure of the esthesioneuroblastoma.
A tumor in the anatomic location and with the light microscopic appearance of an esthesioneuroblastoma was examined with the electron microscope. Secretory-type granules, cytoplasmic fibrils, and microtubules similar to those described in neuroblastomas were found in many of the tumor cells, which supports an origin from the sympathetic autonomic nervous system. Similar organelles were found in three other nasal tumors considered to be esthesioneuroblastomas, but their detail was obscured because they were examined after being initially processed in paraffin.
Aged↗
Olfactory neuroblastomas: a clinicopathologic study.
Clinical and pathologic findings in seven patients with olfactory neuroblastomas are reviewed. Thes neoplasms occurred over a wide age range and, regardless of microscopic pattern, had an unpredictable clinical course. Total extirpation followed by radiation provided optimal therapeutic benefit. The difficulty of pathologic diagnosis of olfactory neuroblastomas is emphasized by the number of neoplasms excluded from this series after critical review of clinical and microscopic findings.
Adenocarcinoma↗
Olfactory neuroblastoma: an ultrastructural study.
The ultrastructural features of two olfactory neuroblastomas are reported. By means of light microscopy, one was well differentiated while the other was a poorly differentiated, small cell neoplasm. The latter case required ultrastructural examination to establish the diagnosis. Electron microscopy of human tumors for diagnostic purposes may be particularly helpful in deciphering small, round cell tumors. Catecholamines were not biochemically detected in a portion of recurrent tumor from Case 2. The significance of this is equivocal in view of the limited previous biochemical studies of this neoplasm. However, by both light and electron microscopy, the morphology of olfactory and sympathetic neuroblastomas are strikingly similar.
Adolescent↗
A new histochemical approach to olfactory esthesioneuroma. A nasal tumor of neural crest origin.
A close collaboration between E.N.T. surgeons and pathologists permitted us to obtain 6 cases of olfactory esthesioneuroma in which fresh tumor tissue was available. Histochemical and biochemical studies were made in comparison with neuroblastoma. Different reactions and assays performed revealed a similarity with sympathetic tumors. Moreover, the presence of catecholamines in esthesioneuroma provides a great help in diagnostic as does electron microscope examination.
Catecholamines↗
Olfactory neuroblastoma (esthesioneuroblastoma): a light and ultrastructural study of two cases.
Olfactory neuroblastoma is a malignant neoplasm with a varied biological behavior. Its clinical course is unpredictable and there is no correlation between its microscopic features and biological behavior. The present study deals with light and ultrastructural characteristics of two cases of olfactory neuroblastoma of the nasal cavity. In one patient, the definitive diagnosis was established on the basis of ultrastructural features of the lesion. The most consistent fine structural findings were the presence of intracytoplasmic densecored neurosecretory granules, "true" and "pseudo-" rosettes, and the neuritic processes emanating from the tumor cells. On the basis of their biochemical, histochemical, and ultrastructural characteristics, olfactory neuroblastomas are similar to neuroblastomas arising from the adrenals or sympathetic nervous system. These findings, therefore, support the hypothesis that olfactory neuroblastomas are most likely of neural crest origin and thus belong to a group of neoplasms collectively known as "apudomas" or neurocristomas. The literature review strongly favors combined surgery and postradiation as the most effective treatment of olfactory neuroblastoma.
Aged↗
Esthesioneuroblastoma.
Esthesioneuroblastoma is an uncommon tumor of neural crest origin arising in the nasal cavity. Since 1966, 97 cases have been reported in the world literature. In this report an analysis is presented of the following aspects of these 97 patients: age and sex distribution, disease staging, treatment results, interval to recurrence, and survival. Staging was according to a system proposed by Kadish et al. There is a bimodal age distribution with peaks in age groups 11-20 years and 51-60 years. Prognosis is favorable in early stage disease (A and B) with 3-year crude survivals of 88.9% and 83.3%, respectively. Stage C patients have a poor prognosis, with 52.9% surviving 3 years. Treatment consisted of radiotherapy, surgery, or a combination of radiotherapy and surgery. In stage A, prognosis is favorable in all three treatment groups with one 1 of 24 patients dying of recurrent disease. In stage B, recurrence rates and 3-year survivals are also similar in the three treatment groups. Thus, single modality treatment is as effective as combined treatment for early stage disease. Local recurrence is the predominant site of failure occurring in 68% of recurrences. The overall crude survival for the 97 patients was 95.4%, while the determinate survival (corrected for intercurrent disease) was 70.8%.
Adolescent↗
Olfactory esthesioneuroblastoma--variables predictive of tumor control and recurrence.
This report surveys the world literature concerning prognostic variables for patients with esthesioneuroblastoma, an uncommon tumor arising from the olfactory epithelium. Specific sites of local and regional tumor extension, recurrence of disease after treatment, distant metastasis, and patient age were all found to be related to patient prognosis. There is a higher cure rate for patients treated after 1966, indicating an improvement in treatment methods. Also, the results indicate that in more recent years patients are being diagnosed and treated with less advanced disease. A statistical procedure, discriminant analysis, was employed, which permits the oncologist to predict patient outcome accurately. The discriminant function equation was 87% correct in identifying patients with tumor control (for a minimum period of 36 months) or recurrence and was 84% correct in classifying patients free of disease for at least 60 months or with recurrence. The statistical procedures employed in this report can be extended to other tumors and survival criteria.
Adolescent↗
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↗
Neuroblastomas and neuroendocrine carcinomas of the nasal cavity: a proposed new classification.
The histologic characteristics of 29 nasal tumors previously diagnosed as neuroblastomas, unclassified carcinomas, or unclassified malignant neoplasms were reviewed. Electron microscopy was performed in 17. Nine tumors were neuroblastomas; six of these were classical neuroblastomas while the other three exhibited olfactory differentiation in addition to the classical neuroblastoma component. Areas of ganglioneuroblastoma were found in the metastasis of one of the three olfactory neuroblastomas. Twenty tumors were classified as neuroendocrine carcinoma because all showed a neuroendocrine pattern with remarkably uniform cells growing from benign glandular epithelium; membrane bound granules were present in the cytoplasm of cells of the ten cases in this group examined by electron microscopy. The mean age of the patients with neuroblastomas was 20 years; survival in this group was 75% at five and seven years, respectively, and 67% at ten years. Recurrences, metastasis, and death occurred within 3 years of diagnosis. There was a low percentage (25%) of multiple recurrences. The metastases were located in cervical lymph nodes, brain and spine. The mean age of the patients with neuroendocrine carcinoma was 50 years. Survival was 100% at five years, 88% at seven years, and 77% at ten years. Recurrences and metastasis in 70% of the cases occurred later than the third year. Multiple recurrences were present in 54% of the cases. The metastases affected lymph nodes, brain and spine in all cases except in one in which lungs and femur were involved. In the latter case adenocarcinoma was also present in addition to the neuroendocrine carcinoma. Three patients died, all more than five years from the time of diagnosis. No correlation was found between staging and prognosis in either group, except for Stage I disease.
Cell Differentiation↗
Response of esthesioneuroblastoma to chemotherapy. Report of five cases and review of the literature.
Five patients with advanced stage or metastatic esthesioneuroblastoma treated with chemotherapy are reported, and another eight cases found in the literature are reviewed. In this collective experience with chemotherapy in this disease, the authors found that 8 of 13 patients (62%) had an objective response to chemotherapy. The agents which may be active in this disease and the role of chemotherapy, particularly adjuvant therapy for patients presenting with advanced disease, are discussed.
Adolescent↗
The association of embryonal tumors originating in the kidney and in the brain. A report of seven cases.
This report documents, in seven infants younger than 2 years of age, a previously unrecognized association of a renal embryonal neoplasm (malignant rhabdoid tumor in six patients and a Wilms' tumor in one) with an embryonal primary tumor originating in the central nervous system. The neuroepithelial tumors included three cerebellar medulloblastomas, one pineoblastoma, one primitive neuroepithelial tumor (probably cerebral neuroblastoma), one malignant subependymal giant cell astrocytoma, and one cerebellar medulloepithelioma with divergent glial and neuronal differentiation. There is no evidence that this association is based on the selective neoplastic transformation of embryonal cells of similar histogenetic or cytogenetic origin. The relationship between these dissimilar, embryologically unrelated tumors remains enigmatic.
Astrocytoma↗
Treatment of advanced esthesioneuroblastoma with high-dose chemotherapy and autologous bone marrow transplantation. A case report.
A 46-year-old woman presented with an advanced unresectable esthesioneuroblastoma which failed to respond to radiation therapy and one course of chemotherapy. She underwent treatment with high-dose chemotherapy (cyclophosphamide, doxorubicin, and vinblastine) followed by autologous bone marrow transplantation. The major toxicity from the regimen was severe oropharyngeal mucositis. A complete remission was achieved and the patient is free of disease and asymptomatic 3.5 years after treatment.
Antineoplastic Combined Chemotherapy Protocols↗