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1/6. Olfactory neuroblastoma metastatic to the breast.

    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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2/6. Esthesioneuroblastoma: case report.

    Esthesioneuroblatoma (ENB) is a rare tumor arising from the olfactory epithelium of the nasal vault which frequently invades the cranial base, cranial vault and orbit. ENB has a bimodal age distribution between 11 and 20 years and between 51 and 60 years. ENB accounts for approximately 1 to 5% of intranasal cancers and no consensus has been reached regarding treatment of this tumor. We report on a 66 year old female patient with a Kadish stage C tumor with frontal lobe invasion submitted a total craniofacial resection with a combined head neck and neurosurgeon team. The purpose of this study is to analyze the natural history, treatment and prognosis of this tumor, based on the literature review.
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3/6. Misdiagnosis of olfactory neuroblastoma.

    OBJECT: Olfactory neuroblastoma (ON) is a rare neoplasm arising from the olfactory epithelium and found in the upper nasal cavity. The authors studied the frequency with which ON is misdiagnosed with other tumors of the paranasal sinuses such as neuroendocrine carcinoma (NEC), pituitary adenoma, melanoma, lymphoma, and sinonasal undifferentiated carcinoma (SNUC). Based on the belief that misdiagnosis commonly occurs, they emphasized the importance of establishing the correct diagnosis, because the treatment regimens and prognosis of these tumor types are often significantly different. methods: Twelve consecutive patients in whom ON was diagnosed were referred to the Department of neurosurgery at the M. D. Anderson Cancer Center between January 1998 and March 2000. Demographic data were collected, physical findings and mode of treatments were documented, and neuroimaging studies were assessed. Pathologists at the authors' institute reviewed the histological specimens. Only in two of 12 patients was the diagnosis of ON confirmed. Lesions in 10 patients were misdiagnosed; there were two cases of melanoma, three cases of NEC, three cases of pituitary adenoma, and two cases of SNUC. Eight of 10 patients in whom lesions were misdiagnosed required significant alteration in the initially proposed treatment plan. CONCLUSIONS: Neurosurgeons should be acutely aware of the variety of neoplasms that occur in the paranasal region. The correct diagnosis should be ensured before initiating treatment to provide the optimum therapy and spare the patients from needless and potentially toxic treatment.
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4/6. Leptomeningeal infiltration in esthesioneuroblastoma: report of two cases with poor prognosis.

    Esthesioneuroblastoma is a rare neoplasm of neuroectodermal origin, arising from the olfactory epithelium in the roof of the nasal cavity. Extension to the brain can occur less frequently than local recurrence, but leptomeningeal metastases without brain involvement have rarely been mentioned. We report 2 cases with poor prognosis of leptomeningeal infiltration during the evolution of esthesioneuroblastoma.
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5/6. somatostatin receptor imaging of olfactory neuroblastoma.

    Neural-crest tumours, including neuroblastomas, express somatostatin receptors. This can be shown by radionuclide labelling of octreotide, a somatostatin analogue. Studies on imaging with this substance have dealt with childhood neuroblastomas. Olfactory neuroblastoma (aesthesioneuroblastoma) is a rare tumour in which somatostatin receptor content has not been analysed, nor have radionuclide methods for diagnostic purposes been described. We report a case of olfactory neuroblastoma, in which scanning with 111In-labelled octreotide was performed. A strong uptake was seen at the base of the skull. This was confirmed as a recurrent tumour by magnetic resonance (MR) imaging. Uptake was also observed in the neck and chest, indicating extensive spread of the disease. somatostatin receptor expression has been shown to correlate with prognosis in childhood neuroblastoma. The accuracy of labelled octreotide in the diagnosis of olfactory neuroblastoma indicates that it might be useful in radionuclide therapy of patients with advanced disease, when no other treatment modalities are available.
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6/6. Olfactory neuroblastoma (esthesioneuroblastoma) and esthesioneuroepithelioma: histologic and immunohistochemical study.

    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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