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)

Two cases of olfactory neuroblastoma (ONB) representing two morphological variants of the tumor are described. Case 1 exhibited a neuroblastoma-like histological pattern corresponding to the usually reported type of ONB--the esthesioneuroblastoma, whereas in case 2 a very rare variant of ONB-the esthesioneuroepithelioma was recognized. The histological and immunohistochemical differences between the cases are discussed with regard to still controversial opinions concerning the subclassification of ONB and the histogenesis and clinical prognosis of these tumors.
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PMID:Olfactory neuroblastoma (esthesioneuroblastoma) and esthesioneuroepithelioma: histologic and immunohistochemical study. 975 18

Olfactory neuroblastoma is an uncommon tumor. It is usually diagnosed at advanced stages. Most of the patients have an indolent clinical course with slow progression, late recurrence and relative infrequency of distant metastasis. Because of the rarity and lack of a randomized clinical trial of patients with olfactory neuroblastoma, there is no standard treatment for the disease. The survival period has increased as treatments have been improved since 1980. Conventional treatments mainly consist of surgery and/or radiotherapy. Chemotherapy was only administered to a few patients; however, it achieved good response. More clinical experience is needed to improve the treatment strategy in order to reduce the possibility of disease recurrence, and also for rescue therapy. We present 2 patients with Kadish C tumors treated using chemotherapy followed by radiotherapy. Both patients demonstrated rapid and excellent response to induction chemotherapy, and have been free of disease for more than 5 years and 2 years after treatment, respectively.
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PMID:Kadish stage C olfactory neuroblastoma successfully treated by chemoradiotherapy: report of two cases. 1007 39

The computed tomography (CT) and magnetic resonance imaging (MRI) studies of 22 patients with a histologically proven olfactory neuroblastoma were retrospectively reviewed. The tumours displayed a variety of imaging characteristics and aggressiveness. The expansile tendency of olfactory neuroblastoma is characterised by bowing of the sinus walls. The destructive aspect is manifested as tumour replacing the turbinates, septum, and sinus walls with extension into contiguous areas. The density/signal and enhancement characteristics are non-specific. Olfactory neuroblastoma should be suspected in all ages following identification of a mass in the superior nasal cavity demonstrating both expansile and destructive growth patterns. The otorhinolaryngologist and the radiologist should be aware of this tumour entity, as early diagnosis appropriately guides therapy and predicts survival.
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PMID:Computed tomography and magnetic resonance imaging features of olfactory neuroblastoma: an analysis of 22 cases. 1054 31

Olfactory neuroblastoma is a rare tumor originating in the upper nasal cavity. It rarely extends intracranially. We report a clinical case of olfactory neuroblastoma with intracranial extension and distant metastasis. A 35-year-old man complained of nasal stuffiness and bleeding, headache and vomiting. Neurological examination showed anosmia and papilledema. MRI showed a huge mass that occupied the right nasal and paranasal cavities, and extended into the right frontal base. The tumor was removed totally and was histologically diagnosed as olfactory neuroblastoma. About two months after surgery, however, MRI demonstrated a rapid recurrence of the tumor in the nasal and paranasal cavities and the frontal lobe. Metastatic lesions were also seen in the right cervical lymph nodes. Chemotherapy was administered using cisplatin and etoposide. The tumor in the frontal lobe shrunk markedly but the other lesion persisted. Whole brain irradiation and local irradiation of the cervical lymph nodes were performed. The tumors became smaller but did not disappear. MRI demonstrated spinal dissemination. Irradiation of the whole spinal cord was performed. The metastatic lesions disappeared. The patient was discharged without neurological deficits, but died of pneumonia 15 months after surgery. Olfactory neuroblastoma is a slow-growing tumor and is highly radiosensitive, but it rarely extends or develops multiple distant metastases and seldom shows a short survival time, as in our case. A review of the literature documented responses in patients treated with a cisplatin-based drug combination. We recommend systemic control using cisplatin-based chemotherapy in addition to irradiation to prevent local recurrence in cases of advanced or metastatic olfactory neuroblastoma.
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PMID:[A case of olfactory neuroblastoma with intracranial extension and distant metastasis]. 1106 69

Olfactory neuroblastoma (Esthesioneuroblastoma) is a rare malignant tumour arising from olfactory epithelium. It has a predilection for cervical lymph node metastasis and also has potential for distant metastasis to unusual sites like scalp, face, aorta, spleen, liver, adrenal gland and ovary. We report here a rare case of olfactory neuroblastoma in an adolescent girl with metastatic deposits in the breast. A poor prognosis due to rapidly progressive disseminated disease was observed. The relevant literature regarding metastatic olfactory neuroblastoma and metastasis in the breast from non-mammary malignant neoplasms is reviewed.
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PMID:Olfactory neuroblastoma metastatic to the breast. 1107 62

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

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare malignant neoplasm originating from neuroepithelial cells. Treatment for this tumor has traditionally required a craniofacial resection through an external approach. The safety and efficacy of transnasal endoscopic resection is uknown. A series of five patients with Kadish stage A or B lesions of the anterior skull base were resected and immediately reconstructed endoscopically over a nine-year period. There were 2 males and 3 females with a mean age of 64 years. Three patients were treated primarily and two were treated secondarily for recurrences after failing a traditional external craniofacial resection and postoperative radiotherapy. All primary lesions received adjuvant radiotherapy. The mean follow-up time was 31 months. All patients were discharged within 4 days or less. Operative complications included temporary postoperative infraorbital anesthesia (1 patient), unplanned cerebrospinal fluid leak (1 patient), and can orbital hematoma (1 patient). None of these complications resulted in any permanent sequelae. Two patients had prolonged nasal crusting for up to one year due to a variety of factors; large cavities, radiation therapy, and exposed lyophilized dural graft. However, all skull base defects healed without any short- or long-term sequelae. There have been no local recurrences. Two patients (1 primary and 1 secondary) developed regional metastasis to the orbit or cervical lymph nodes managed with primary transorbital excision or modified neck dissection, respectively. All but one patient remain free of disease by clinical, endoscopic, and radiographic (CT or MRI) surveillance. The remaining patient recurred distally as an undifferentiated carcinoma in his temporal bone and lungs 12 months after the initial resection. In experienced hands select cases of esthesioneuroblastoma can be safely excised and reconstructed endoscopically with comparable degrees of tissue removal as with external approaches. Short-term oncologic results in this mall series of patients appear to be comparable to traditional methods. Longer follow-up on a larger series of patients is warranted.
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PMID:Endoscopic resection of esthesioneuroblastoma. 1155 61

We describe a case of olfactory neuroblastoma diagnosed by 99mTc-ECD SPECT. Although MRI and CT are very important for delineating these tumors, they are, by no means, specific for neuroblastomas. 131I-MIBG scintigraphy, the standard method for imaging tumors of neural crest origin, also failed to detect a histologically proven esthesioneuroblastoma.
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PMID:Olfactory neuroblastoma visualized by Technetium-99m-ECD SPECT. 1172 58

Although neuroblastoma is the most common of extracranial solid tumors of childhood and infancy, we report the first case of an isolated neuroblastoma of a paranasal sinus. A 15-year-old girl with a right maxillary sinus mass was asymptomatic except for persistent epiphora. Computed tomography and magnetic resonance imaging scans showed that the mass extended into the nasal cavity, encroached on the lamina papyracea, and obstructed the nasofrontal duct. An extensive workup revealed no evidence of systemic disease. The patient underwent right craniofacial resection. Immunohistochemistry and electronmicroscopic findings were consistent with conventional neuroblastoma. Fluorescence in situ hybridization analysis was performed with probes selected to demonstrate genetic alterations associated with neuroblastoma. Studies revealed deletion of chromosome arm 1p, gain of chromosome 17, and normal N-myc gene copy number. In summary, the tumor exhibited morphologic features and genetic alterations more consistent with those of neuroblastoma than with those of esthesioneuroblastoma.
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PMID:Primary neuroblastoma of the maxillary sinus. 1195 7

Esthesioneuroblastoma (olfactory neuroblastoma) is a rare tumor of the olfactory epithelium. Approximately 1,000 cases have been described in the literature since the original description in 1924. It occurs in older individuals and is rare in children. The authors describe the clinicopathologic presentation in a series of five children treated with neoadjuvant/adjuvant chemotherapy and review the English literature for previously described patients younger than 18 years to assess clinical presentation, mode of treatment, and outcome in this age group.
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PMID:Esthesioneuroblastoma in children. 1221 98


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