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1/16. Blastomatous tumor with teratoid features of nasal cavity: report of a case and review of the literature.

    A case of blastomatous tumor with teratoid features is presented. The polypoid mass was observed in the left nasal cavity of a 72-year-old man. Histologically, the lesion was composed of neuroepithelial cells with blastomatous appearance, cystic squamous nests filled with keratin materials, many mucous glands, complex tubular and glandular structures with edematous fibroblastic stroma. Sinonasal neoplasms including teratoid components and immature neuroepithelium are exceedingly rare. We suggest that the term 'immature teratoma' is more suitable than blastoma or blastomatous tumor when there is no carcinomatous or sarcomatous component besides the immature neuroepithelium and teratoid elements.
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2/16. Olfactory neuroepithelioma arising from the olfactory placode.

    The patient was a 54-year-old man, who had lost his sense of smell 6 years previously and had started to become forgetful about 6 months prior to presenting at hospital. MRI admission showed a large multicystic tumor with Gd-DTPA enhancement extending from the anterior cranial fossa through the sphenoid sinus and into the nasal cavity. Histopathological examination revealed extensive proliferation of small round cells that were divided by connective tissue septae. The tumor cells occasionally formed tubular structures, although no basement membranes were present. On immunostaining, round tumor cells were positive for neuron-specific enolase, synaptophysin, and chromogranin a, while cells forming tubules were positive for AE 1 and CAM 5.2. Almost all of the tumor cells were positive for Ber-EP4, and some of the epithelioid cells surrounding the tubular structures were also positive for luteinizing hormone-releasing hormone (LH-RH). Electron microscopy demonstrated sporadic intercellular junctions, many microtubules in the tumor cell processes, and clear- and dense-cored vesicles in the cytoplasm. Based on the results, this case appears to be the first documented neuroepithelioma with Ber-EP4- and LH-RH-positive cells arising from the olfactory placode.
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3/16. Fine needle aspiration cytology of metastatic olfactory neuroblastoma: a case report.

    BACKGROUND: Olfactory neuroblastoma (ONB) is an uncommon tumor, presenting as a polypoid mass arising from the upper nasal cavity. This tumor has been seldom diagnosed by direct fine needle aspiration (FNA). CASE: Metastatic ONB was diagnosed by FNA. The patient was a 40-year-old female with a polypoid mass in the nasal cavity and ipsilateral cervical lymphadenopathy. The punch biopsy of the nasal tumor revealed a smudged small round cell neoplasm with neuroendocrine differentiation, consistent with ONB. In FNA smears from the cervical lymph node, there were well-preserved, small, monotonous cells with hyperchromatic nuclei, fibrillary cytoplasm and indistinct cell borders. Also noteworthy were occasional pseudorosettes as well as rare true rosettes. By immunocytochemistry, tumor cells were positive for cytokeratin, chromogranin and synaptophysin. CONCLUSION: ONB, like adrenal neuroblastoma, shows distinctive cytologic features, including a rosette or pseudorosette and fibrillary network. FNA can accurately demonstrate these characteristic findings, and in some cases it may be a better diagnostic modality than incisional biopsy.
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4/16. Endoscopic removal of esthesioneuroblastoma.

    Esthesioneuroblastoma is a rare tumor of neural crest origin that arises in the nasal cavity. There is still no consensus on the optimal treatment for this neoplasm, and the literature contains very few accounts of endoscopic excision in these cases. We described a case report of 12-year-old girl with esthesioneuroblastoma that was confined to the nasal cavity and paranasal sinuses, with no orbital or intracranial extension. The tumor was removed via intranasal endoscopic approach and radiotherapy was administered postoperatively. The patient is currently being followed, and there has been no recurrence in 24 months after surgery.
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5/16. Isolated esthesioneuroblastoma of sphenoid sinus.

    Esthesioneuroblastoma is a rare neuroendocrine tumor that arises from the olfactory epithelium and accounts for approximately 3% of all intranasal tumors. The tumor involvement in the nasal cavity with extension to paranasal sinuses, orbit, and anterior cranial fossa, is reported in the literature. In this report, we present an interesting case of isolated sphenoid sinus esthesioneuroblastoma, which is the first case to be reported in the literature, and discuss the pathology, clinical manifestations, and various treatment options for this tumor.
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6/16. Primary sphenoid esthesioneuroblastoma studied with MR.

    Esthesioneuroblastoma (ENB) is an uncommon tumor type of neural crest origin, which, in the majority of cases, arises in the nasal cavity. We present a rare primary presentation of ENB in the sphenoidal area studied with computed tomography (CT) and magnetic resonance (MR). To our knowledge, this is the first case in which MR findings are available.
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7/16. Primary sphenoid sinus esthesioneuroblastoma.

    Esthesioneuroblastoma is a rare malignant tumor of undifferentiated neuroectodermal origin. Because these tumors are derived from olfactory epithelium, most cases arise in the superior nasal cavity at the level of the cribriform niche. The development of an esthesioneuroblastoma outside of the region in which olfactory epithelium exists is exceedingly rare. We report a rare case of a primary sphenoid sinus esthesioneuroblastoma and discuss the pathogenesis, clinical presentation, and treatment of this rare entity.
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8/16. Olfactory neuroblastoma: a case report and review of the literature.

    Malignant tumors of the nasal cavity are rare. We report the case of an elderly woman who consulted us with a 4-year history of progressive nasal obstruction, occasional epistaxis, facial pain, and watering of the eyes. A diagnosis of olfactory neuroblastoma was established by histopathology and confirmed by immunohistochemistry. On staging, the mass was classified as a Kadish stage B tumor. The mass was excised via a lateral rhinotomy approach, and the tumor was peeled away completely from the cribriform plate with endoscopes. The patient underwent postoperative radiation, and she was free of recurrence at follow-up 15 months later.
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9/16. 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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10/16. Intracavitary chemotherapy (Gliadel) for recurrent esthesioneuroblastoma: case report and review of the literature.

    Esthesioneuroblastoma is an uncommon malignancy of the nasal vault with a treatment regimen consisting of surgical resection followed by radiotherapy for primary lesions and addition of chemotherapy for patients with advanced, recurrent or metastatic lesions. We report a case of a 39-year-old female with a history of esthesioneuroblastoma, previously treated with resection, radiation and chemotherapy, presenting with a recurrent disease that was successfully treated with re-resection and placement of Gliadel) wafers in the surgical resection cavity. The novel option of controlled-release and local delivery of a chemotherapeutic agent for treatment of recurrent esthesioneuroblastoma should be recognized and considered.
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