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1/66. Kadish stage C olfactory neuroblastoma successfully treated by chemoradiotherapy: report of two cases.

    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. ( info)

2/66. 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. ( info)

3/66. Sinonasal teratocarcinosarcoma ("mixed olfactory neuroblastoma-craniopharyngioma") presenting with syndrome of inappropriate secretion of antidiuretic hormone.

    Sinonasal teratocarcinosarcoma (SNTC) is a rare, aggressive, histologically heterogeneous neoplasm of the paranasal sinuses and nasopharnyx of adults that is composed of variably benign or malignant neuroepithelial, epithelial, and mesenchymal elements. Occasional cases show intracranial extension and may be operated on by neurosurgeons and encountered by neuropathologists who may not be familiar with the entity. STNCs have not previously been associated with functional hypersecretory status. We report a 59-year-old male who presented with headache and syndrome of inappropriate secretion of antidiuretic hormone (SIADH) and was subsequently found to have a bulky tumor of the frontal and ethmoid sinuses with focal dural invasion. The tumor was predominantly composed of olfactory neuroblastoma areas (90% of tumor) admixed with unusually well-developed craniopharyngioma-like mature squamous epithelium and ghost cells ( 10% of tumor). Scattered neuroblastoma tumor cells showed strong immunoreactivity with antibodies to arginine vasopressin, supporting ectopic hormone secretion by the tumor. While the coexistence of neuroectodermal and oral ectodermal-like differentiation in SNTCs is characteristic, in our case it was developed to an extreme functional and morphologic degree and was unassociated with other mesenchymal or epithelial elements often found in these complex tumors. SNTCs with limited differentiation have prompted controversy in classification. ( info)

4/66. 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. ( info)

5/66. Primary sphenoid and petrous apex esthesioneuroblastoma: case report.

    A 62-year-old woman presented with raised intracranial pressure and features of a right cerebellopontine angle tumour with extension into the right middle cranial fossa. The patient died before a surgical excision could be performed. The autopsy revealed a primary esthesioneuroblastoma of the sphenoid sinus eroding the petrous bone and extending into the middle cranial fossa with metastatic tumour in the liver, and paratracheal and hilar lymph nodes. Although rare, esthesioneuroblastoma must be considered in the differential diagnosis of petrous-sphenoid lesions. ( info)

6/66. 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. ( info)

7/66. Olfactory neuroblastoma (esthesioneuroblastoma): report of six cases treated by a novel combination of endoscopic surgery and radiosurgery.

    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. ( info)

8/66. Endoscopic resection of esthesioneuroblastoma.

    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. ( info)

9/66. Olfactory neuroblastoma as a second malignant neoplasm in a patient previously treated for childhood acute leukemia.

    Various kinds of second malignant neoplasms after sucessful treatment for childhood acute leukemia have been reported. The authors describe an unusual case of an olfactory neuroblastoma in a patient previously treated for childhood acute leukemia including autologous bone marrow transplantation. The prophylactic cranial irradiation and the total body irradiation during autologous bone marrow transplantation may have induced the development of their patient's olfactory neuroblastoma. Although a second primary olfactory olfactory neuroblastoma is rare is rare, it should be added to the list of second malignant neoplasms in the sinonasal region. ( info)

10/66. Olfactory neuroblastoma visualized by technetium-99m-ECD SPECT.

    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. ( info)
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