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Query: UMLS:C0027819 (neuroblastoma)
27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Thirty-nine patients with esthesioneuroblastoma are reviewed. The presentation of the tumor, symptomatology, investigation, and treatment are discussed. A recommended treatment regimen is outlined. Histologic typing is valueless in predicting tumor behavior. An illustrative and difficult case of recurrent base of skull esthesioneuroblastoma is presented. The resection performed is described, and the problem of extradural oropharyngeal communication is discussed. The solution was to use a temporalis and galeal frontalis flap. Reconstruction was with an external and intraoral prosthesis. Optimal treatment in a fresh lesion is radical surgery with or without radiation therapy. Esthesioneuroblastoma is a rare and often misdiagnosed malignant tumor of the olfactory epithelium. Originally described by Bergen et al. in 1924 as "esthesioneuroepithelioma olfactif," it was introduced into the North American literature by Schall and Lineback in 1951. Since then, fewer than 200 cases have been collected. The various terms used to describe it--olfactory esthesioneuroblastoma, esthesioneurocytoma, and olfactory neuroblastoma--all denote origin from the neural crest. The sensory nerves of smell are short bundles of fibers that originate in the olfactory bulb and pass through the cribriform plate to the olfactory area of the nasal mucosa. This mucosa is located in the most superior part of both nasal fossae. Thus the usual primary sites of occurrence include the superior nasal cavity or nasal septum, and turbinates, the ethmoid, or the cribriform plate, although an extranasal site of origin has been suggested. Symptoms are usually progressive and range from nasal obstruction or epistaxis to diplopia, ocular pain, and headaches in the more advanced disease state.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Esthesioneuroblastoma: treatment of skull-base recurrence. 402 92

Esthesioneuroblastoma or olfactory neuroblastoma is a rare tumor of the nasal cavities and paranasal sinuses. In the majority of cases the cerebral involvement is secondary. Only ten cases have been reported in which the tumor was primarily intracranial. An eleventh case is reported and literature reviewed.
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PMID:Primary intracranial esthesioneuroblastoma. Case report. 406 32

"Olfactory neuroblastoma" covers several types of esthesioneurogenic tumors such as esthesioneuroepithelioma, esthesioneuroblastoma and others. They are thought to be of olfactory mucosal origin and present with typical light microscopical "neurogenic features" e.g. rosettes and/or axon production. Some cases have been analyzed ultrastructurally and contained membrane bound granula like others tumors of the APUD system, originating from the neural crest. Furthermore neuronal differentiation of various degrees has been described. The human case of this contribution did not contain rosettes, but axons could be demonstrated in considerable number by silver impregnation. Electron microscopy could demonstrate the presence of dendritic processes with microtubuli and filaments as well as abundance of secretory granules of 1800 A. The comparison with the up to now published findings shows that the production of dense core granules is the most constant ultrastructural feature in these tumors; however, additional ultrastructural features in typical human cases as here presented, earlier experimental results and few human descriptions show, that DCV production is not essential for olfactory neuroblastoma. This might shed new light on the histogenesis of these tumors.
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PMID:Histogenesis of olfactory neuroblastoma. I. Electron microscopy of typical human case. 408 Jun 36

We present the first case report of an olfactory neuroblastoma (esthesioneuroblastoma) diagnosed by cytologic examination. The patient was a 40-year-old male who had a 13-year history of "adenocarcinoma" of the nasal cavity until the correct diagnosis of olfactory neuroblastoma was made cytologically from pleural fluid shortly before his death. The cells had the typical features of rosette formation, scanty elongated cytoplasm, clustering of cells and nuclear compression resulting in an "onion-skin" appearance. Surgical specimens, several biopsies and fine needle aspiration of a metastatic deposit in a lymph node all showed, retrospectively, features of esthesioneuroblastoma. Electron microscopy showed membrane-bound dense-core secretory granules. Autopsy findings revealed multiple metastases but no tumor at the original site; that tumor had been treated with high-dose radiation therapy as well as systemic chemotherapy. Olfactory neuroblastoma is a rare tumor, but it is important to recognize because it has a better prognosis than the more commonly encountered malignancies of the nose.
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PMID:Cytologic diagnosis of olfactory neuroblastoma. Report of a case with multiple diagnostic parameters. 657 48

A case of esthesioneuroblastoma, the pathological diagnosis of which almost always causes great difficulties, was investigated ultrastructurally, biochemically, and immunohistologically, using antibodies against the five known types of intermediate filaments [keratin, vimentin, desmin, glial fibrillary acidic protein (GFAP) and neurofilaments]. The tumour cells did not react with antibodies against any of the five intermediate filament proteins. Ultrastructural investigations showed dense cored secretory granules in the cytoplasm and cell processes. Thus, immunohistology offers by "exclusion" a differential diagnosis to avoid often misdiagnosed tumours (undifferentiated carcinomas, embryonal rhabdomyosarcomas, and malignant lymphomas), since carcinomas react with antikeratin, embryonal rhabdomyosarcomas with antibodies to desmin and malignant lymphomas show immunofluorescence with antibodies to vimentin. The biological behaviour (age distribution, tendency to metastasize), the normal values of biochemical parameters, homovanillic acid and vanilmandelic acid (HVA, VMA), and the absence of neurofilaments distinguish this type of tumour from the peripheral sympathetic neuroblastoma.
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PMID:Esthesioneuroblastoma: ultrastructural, immunohistological and biochemical investigation of one case. 671 29

Three cases of neuroblastoma with intracranial metastases were reported. The first case was a 13-year-old girl with sacral neuroblastoma who had metastases to the left parietal bone and dura, and had bilateral intraorbital metastases. The second case was a 13-year-old girl with mediastinal neuroblastoma who had bilateral intraorbital metastases. The third case was a 49-year-old woman with esthesioneuroblastoma which had invaded to the dura at the right middle fossa and the right orbit. CT findings of the brain of these three cases were investigated, and summarized as follows: 1) The intracranial invasions of the metastatic tumors were seen to be slightly high density (EMI-number 25-27) in plain scans, and homogeneously enhanced as a well-defined high density mass (EMI-number 35-37). 2) The tumors invading the skull were observed as a mass lesion of the bone on osteolytic lesion. 3) The intraorbital metastatic tumors were seated on the orbital wall invading the rectal muscles, but they remained extraconal. It was emphasized that CT could give valuable information for the diagnosis of skull and intracranial metastasis of neuroblastoma.
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PMID:[CT findings of skull and intracranial metastatic neuroblastoma--report of 3 cases (author's transl)]. 711 May 17

A nasal neuroblastoma (esthesioneuroblastoma) extending into the brain in a heifer produced mouth breathing and proptosis. The mass filled much of the left nasal cavity, palatine sinus and maxillary sinus, with turbinate atrophy and deviation of the septum. Caudally the neoplasm extended into the nasopharynx and olfactory bulb. It was a cellular neoplasm composed of small, undifferentiated piriform cells showing infrequent pseudorosettes and immature axonal processes. Mitosis was common is some areas.
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PMID:Olfactory neuroblastoma in a heifer. 725 93

Two cases of esthesioneuroblastoma occurring in African Nigerians are reported, with a review of the literature. Though the tumour has highly suggestive clinical features, histologic diagnosis may be difficult. The name 'esthesioneuroblastoma' is considered to be preferable to 'olfactory neuroblastoma' in order to avoid consideration of such a tumour as a secondary from the adrenal gland. Early diagnosis and treatment by surgery gives the best chance of cure.
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PMID:Esthesioneuroblastoma: report of two cases in Nigerians. 743 Aug 14

We report the light microscopic and immunohistochemical features of vascular proliferations associated with 26 extracranial neural and neuroendocrine neoplasms including esthesioneuroblastoma, neuroblastoma/ganglioneuroblastoma, the primitive neural component of immature teratoma, mediastinal teratoma, primitive neuroectodermal tumor, intra-abdominal desmoplastic small cell tumor, Merkel cell carcinoma of the skin, and thyroid medullary carcinoma. These vascular proliferations were similar to those associated with high-grade glial neoplasms and were characterized by tufts of vessels with a glomeruloid configuration or by long cords of vessels. Immunohistochemical evaluation documented the presence of endothelial cells, perithelial cells, and basement membrane components within the foci of proliferating vessels. We propose that these vascular proliferations represent a characteristic feature of the neuroendocrine/neural neoplastic phenotype and that they possibly arise as the result of angiogenic factors produced by the neoplastic cells. The presence of these distinctive vascular lesions in the stroma of a poorly differentiated neoplasm should alert the pathologist to the possibility of the neoplasm being of a neural or neuroendocrine nature.
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PMID:Florid vascular proliferation associated with neural and neuroendocrine neoplasms. A diagnostic clue and potential pitfall. 877 95

Sinonasal teratocarcinosarcoma (SNTCS) is a very unusual and aggressive neoplasm characterized by the combination of malignant teratoma and carcinosarcoma features, of which less than forty cases have been reported in the literature. We report on a 75-year-old man with SNTCS that involved the left ethmoid, maxillary and sphenoidal sinuses. The tumor showed a complex histological pattern with mature and immature glands, benign squamous and malignant poorly differentiated epithelia, as well as neuroblastoma-like tissue and sarcoma component with rhabdomyoblastic differentiation. This peculiar blend of tissue types makes the diagnosis of this entity a difficult challenge, especially in small biopsies or in tumors only partially removed. This tumor must be differentiated from several types of carcinomas, esthesioneuroblastoma, craniopharyngioma, malignant mixed tumor of salivary gland type and germ cell tumors. The present case represents, to our knowledge, the third SNTCS described in the european literature.
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PMID:Sinonasal teratocarcinosarcoma: an unusual neoplasm. 756 86


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