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 patient with a four-year history of unexplained hyponatremia was seen with recurrent nasal discharge and was found to have a typical olfactory neuroblastoma. The clinical laboratory diagnostic studies suggested that the patient's sodium deficiency was secondary to the syndrome of inappropriate antidiuretic hormone. Subsequent resection of the neoplasm led to resolution of the hyponatremia, suggesting that a (tumor-associated) humoral factor, such as vasopressin or a vasopressinlike substance, was responsible for the electrolyte disturbance. A search of the literature disclosed one previous case of vasopressin-secreting nasal neuroblastoma.
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PMID:Hyponatremia secondary to olfactory neuroblastoma. 687 Jun 50

Microsurgical techniques have considerably improved the results of surgical treatment for esthesioneuroblastoma (olfactory neuroblastoma). Nevertheless, these rare tumours of the frontal skull base are still associated with high rates of tumour recurrence and mortality, thus remaining a challenge even for experienced surgeons. A novel therapeutic approach that combines endoscopic sinus surgery and radiosurgery (gamma knife) is presented here. Six patients (3 males, 3 females) aged between 27 and 75 years (median 38 years) were treated between August 1993 and July 1999. Following paranasal and nasal endoscopic sinus surgery, marginal irradiation doses ranging from 16 to 34 Gy were applied radiosurgically involving up to 7 isocentres. At present, the median follow-up period is 57 months (range: 9 - 79 months). Without mortality, tumour control was achieved in all patients. One patient, who had to undergo additional craniotomy because of extensive neoplastic infiltration, developed postoperative liquorrhea. In another case the clinical course was complicated by a bilateral frontal sinusitis. All patients complained of nasal discharge and crusts. However, a preoperative Karnovsky Index ranging from 80 to 100 % remained stable in four patients whereas an improvement was observed in two patients. Based on the favourable results observed so far, the combination of endoscopic sinus surgery and radiosurgery can be considered as promising new option for the treatment of esthesioneuroblastoma that merits further investigation.
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PMID:Olfactory neuroblastoma (esthesioneuroblastoma): report of six cases treated by a novel combination of endoscopic surgery and radiosurgery. 1148 89

Neuroendocrine carcinoma (NEC) is rare. We report a case of probable sinonasal NEC in a 73-year-old man who had presented with a history of right nasal obstruction, nasal discharge, and recurrent epistaxis. On examination, a red, friable, gelatinous, polypoid mass with a tendency to bleed was seen in the right nasal cavity. Computed tomography revealed that the lesion was confined to the right nasal cavity; coincidental or reactive opacification was seen in the adjacent sinuses. The final histologic evaluation of the excised biopsy specimens yielded a diagnosis of an invasive, poorly differentiated NEC, probably a large-cell variant, with the differential diagnosis lying at a point somewhere between poorly differentiated large-cell NEC and high-grade olfactory neuroblastoma. The patient underwent a right lateral rhinotomy and medial maxillectomy followed by adjuvant radiotherapy. At 20 months of follow-up, he exhibited no sign of recurrence.
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PMID:Sinonasal neuroendocrine carcinoma: a case report. 1857 85

Primary sinonasal tract and nasopharyngeal adenoid cystic carcinomas (STACC) are uncommon tumors that are frequently misclassified, resulting in inappropriate clinical management. Eighty-six cases of STACC included 45 females and 41 males, aged 12-91 years (mean 54.4 years). Patients presented most frequently with obstructive symptoms (n = 54), followed by epistaxis (n = 23), auditory symptoms (n = 12), nerve symptoms (n = 11), nasal discharge (n = 11), and/or visual symptoms (n = 10), present for a mean of 18.2 months. The tumors involved the nasal cavity alone (n = 25), nasopharynx alone (n = 13), maxillary sinus alone (n = 4), or a combination of the nasal cavity and paranasal sinuses (n = 44), with a mean size of 3.7 cm. Patients presented equally between low and high stage disease: stage I and II (n = 42) or stage III and IV (n = 44) disease. Histologically, the tumors were invasive (bone: n = 66; neural: n = 47; lymphovascular: n = 33), composed of a variety of growth patterns, including cribriform (n = 33), tubular (n = 16), and solid (n = 9), although frequently a combination of these patterns was seen within a single tumor. Pleomorphism was mild with an intermediate N:C ratio in cells containing hyperchromatic nuclei. Reduplicated basement membrane and glycosaminoglycan material was commonly seen. Necrosis (n = 16) and atypical mitotic figures (n = 11) were infrequently present. Pleomorphic adenoma was present in 9 cases; de-differentiation was seen in two patients. Immunohistochemical studies showed positive reactions for pan-cytokeratin, CK7, CK5/6, CAM5.2, and EMA, with myoepithelial reactivity with SMA, p63, calponin, S100 protein and SMMHC. CD117, CEA, GFAP and p16 were variably present. CK20 and HR HPV were negative. STACC needs to be considered in the differential diagnosis of most sinonasal malignancies, particularly poorly differentiated carcinoma, olfactory neuroblastoma and pleomorphic adenoma. Surgery (n = 82), often accompanied by radiation therapy (n = 36), was generally employed. A majority of patients developed a recurrence (n = 52) 2-144 months after initial presentation. Overall mean follow-up was 19.4 years (range 0.4-37.5 years): 46 patients died with disease (mean 6.4 years); 5 were alive with disease (mean 5.4 years), and 35 patients were either alive or had died of unrelated causes (mean 16.3 years). ACC of the SNT is uncommon. Recurrences are common. The following parameters, when present, suggest an increased incidence of either recurrence or dying with disease: mixed site of involvement, high stage disease (stage IV), skull base involvement, tumor recurrence, a solid histology, perineural invasion, bone invasion, and lymphovascular invasion.
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PMID:Sinonasal tract and nasopharyngeal adenoid cystic carcinoma: a clinicopathologic and immunophenotypic study of 86 cases. 2403 41