Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A total of seven patients with head and neck tumors underwent a surgery of the anterior skull base in combination with an extracranial approach. Two patients had squamous cell carcinoma of the frontal sinus; one, undifferentiated carcinoma of the ethmoid sinus; two, squamous cell carcinoma of the ethmoid sinus; one, olfactory neuroblastoma; and one, recurrent squamous cell carcinoma of the nasopharynx. Two patients, one with frontal sinus carcinoma and one with undifferentiated carcinoma, developed recurrence. The former was treated with a salvage operation and the latter, with radiotherapy and chemotherapy. Six patients are alive disease free (4, 5, 6, 8, 27 and 73 months) whereas the other is alive with disease (8 months). One patient developed partial necrosis of the reconstructed bony skull base and another developed enophthalmus and temporary diplopia postoperatively. No serious complications, however, were noted in any cases.
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PMID:[Surgery of the anterior skull base for head and neck tumors]. 204 Sep 11

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

The sinonasal undifferentiated carcinoma (SNUC) is an aggressive and rare neoplasm arising in the nasal cavity and the paranasal sinuses. To date, over 50 cases of histologically proven SNUCs have been reported since its original description in 1986. Presenting symptoms include facial pain, nasal obstruction, diplopia, epistaxis, proptosis, and periorbital swelling. The histologic features of this neoplasm include cohesive cells arranged in nests, ribbons, and trabeculae. The cells exhibit hyperchromatic nuclei and a high nuclear to cytoplasmic ratio. A brisk mitotic rate, tumor necrosis, and vascular invasion are prominent features. Confirming the diagnosis of SNUC at the light microscopic level can be challenging, since the microscopic differential diagnosis includes olfactory neuroblastoma, rhabdomyosarcoma, undifferentiated nasopharyngeal carcinoma (lymphoepithelioma), malignant lymphoma, malignant melanoma, and neuroendocrine (small cell undifferentiated; oat cell) carcinoma. Sinonasal undifferentiated carcinoma can be differentiated from these other neoplasms by correlating clinical, light microscopic, histochemical, immunohistochemical, and ultrastructural characteristics. Aggressive, multimodal therapy can provide the best opportunity for local control of this neoplastic process, but the optimal treatment has yet to be determined.
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PMID:Sinonasal undifferentiated carcinoma: a distinctive clinicopathologic entity. 1056 93

Despite circumstantial evidence that opsoclonus-myoclonus (OM) is often immune mediated, no specific autoantigen has been identified. Using sera of 21 patients with several types of OM (idiopathic, associated to small cell lung cancer, and associated to neuroblastoma), we probed a brainstem cDNA library to isolate target neuronal antigens. Thirty-seven clones coding for 25 proteins were isolated, with two groups of autoantigens emerging: (1) proteins of the postsynaptic density, among them the adenomatous polyposis coli, and 2) proteins with expression or function restricted to neurons, including RNA or DNA-binding proteins and zinc-finger proteins. Usually, each patient's serum recognized a different autoantigen, except for adenomatous polyposis coli that was recognized by sera of two patients with idiopathic OM and two control patients with nystagmus, diplopia, and paraneoplastic brainstem dysfunction. Overall, in the indicated types of OM, (1) we found frequent and heterogeneous immunity to neuronal autoantigens without a single specific antibody marker of OM, (2) the occasional detection of antibodies to known onconeuronal antigens (ie, Hu proteins) probably is related to cancer-induced immunity rather than to OM, and (3) the postsynaptic density is a frequent source of novel autoantigens, with several proteins of this complex targeted by antibodies of OM patients.
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PMID:Autoantigen diversity in the opsoclonus-myoclonus syndrome. 1260 2

Esthesioneuroblastoma (ENB) also known as olfactory neuroblastoma is an uncommon malignant neoplasm arising in the roof of nasal cavity. It is now understood to originate from the olfactory epithelium. Case reports published worldwide have been very few. Common presenting symptoms of Esthesioneuroblastoma include nasal obstruction, epistaxis, facial pain, diplopia, proptosis, and anosmia. Apart from being locally aggressive, it metastasizes widely by both hematogenous and lymphatic routes.
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PMID:Esthesioneuroblastoma a case report. 2275 35

We report a case of an olfactory neuroblastoma revealed by atypical presentation with only ophthalmic symptoms. A 73-year-old woman presented with exophthalmos, left chemosis, decreased visual acuity, and diplopia. CT scan revealed a heterogeneous mass with isolated maxillary sinus, orbital floor, and lower eyelid invasion. This orbital lesion has no major ethmoidal extension (a single anterior ethmoidal cell appears retrospectively invaded). The biopsy sample of the hardened tumefaction revealed after histological examination an olfactory neuroblastoma (or esthesioneuroblastoma). The patient was treated by surgery (resection of the lesion and neck dissection) followed by postoperative irradiation.Olfactory neuroblastoma has no specific symptoms, but habitually presents as an ethmoidal lesion possibly accompanied with ophthalmic manifestations (in 20% to 30% of cases) due to orbital extension. This type of unusual presentation requires our attention, and we discuss the actual knowledge about the pathogenesis, clinical presentation, and treatment of this rare entity.
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PMID:Olfactory neuroblastoma presenting with exclusive orbital manifestations. 2352 73