[The nose, paranasal sinuses, and nasopharynx].
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A variety of primary and secondary tumors can involve the optic nerve head. Examples of primary optic disc tumors include capillary, cavernous and racemose hemangiomas, astrocytomas, and melanocytomas. Secondary optic disc tumors include metastatic carcinoma, leukemia, and those which invade the nerve head from adjacent structures (choroidal melanoma, retinoblastoma, and meningioma). These tumors may produce a variety of clinical features and are discussed in this review. Included also are histopathologic correlations and guidelines for management of the individual entities.
Neuroblastoma in adults is uncommon. Previous reports have suggested that adult patients with neuroblastoma have a better prognosis than children with these tumors. We have examined the clinical features of eight adults with neuroblastoma and related these data to tumor histopathology and immunohistochemistry using an antibody to neuron-specific enolase. The results show that when children and adults with neuroblastoma are compared by stage, adults do not have a better prognosis. Adults tend to have a different anatomic distribution of primary tumor sites, with more frequent extra-abdominal sites than are seen in children. Neuroblastomas arising in adults are similar to those seen in children by containing neuron-specific enolase, an enzyme associated with cells of neuroectodermal origin. These findings show that adult neuroblastomas are similar to their childhood counterparts in clinical behavior and pathologic features.
An intraocular lacrimal gland choristoma which was at first mistaken for a medulloepithelioma (diktyoma) was studied with the electron microscope. The tumour did not contain cells with neuronal and glial differentiation as expected in a medulloepithelioma but it did contain acini, ducts and dilated ducts or cysts. The tumour, in fact, bore much resemblance to the lacrimal gland, in that it contained electron-dense serous granules, and at times, a myoepithelial cell was detected at the base of an acinus. The ductal and cystic elements were lined by a single layer of cells or by stratified or pseudostratified epithelium. An interesting, unexpected feature was the occurrence of a myoid band (composed of thin filaments with focal densities along their course) under the cell membrane of the epithelial cells adjacent to the lumen of the ducts and cysts.
Esthesioneuroblastoma is an uncommon nasal tumor originating from cells of neural crest origin. An increased awareness of this neoplasm by clinicians and pathologists has led to an increase in its diagnosis. In the Department of Otolaryngology--Head and Neck Surgery at the University of Virginia Medical Center, 22 cases have been treated. A review of the biologic course of these cases has allowed us to make certain recommendations for therapy. Patients with stage A and B disease are best treated with preoperative radiation therapy followed by craniofacial resection. Patients with stage C disease are treated with cyclophosphamide and vincristine before irradiation and craniofacial resection. If a significant response occurs these patients receive postoperative chemotherapy. We believe this treatment should result in five-year survivals in excess of 50%.
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A case of olfactory neuroblastoma in a 9 month-old girl is reported. Blindness and purulent meningitis were the first symptoms. Radiological examinations including CT scan revealed a sphenoidal tumor with penetration of the meninges. Surgery revealed an ethmoido-sphenoidal tumor mass extending into the pituitary fossea which consisted of malignant cells with the histological aspect of esthesioneuroblastoma. The rarity of this tumor in early childhood and its clinical polymorphism are emphasized.
In context with the first light and electron microscopic description of a spontaneous esthesioneuroepithelioma in a dog (10 year old female Labrador) general morpho- and etiopathogenetic problems concerning olfactory neoplasias were discussed. Because of its specific location and growth pattern, especially the development of true rosettes and pseudo-rosettes, the tumor was evaluated light microscopically as an esthesioneurogeneic one. The final classification was based upon ultrastructural peculiarities, as occurrence of ciliar structures, characteristic intercellular junctions, and the - although rare - appearance of dense cored vesicles.
Thirty-nine patients with esthesioneuroblastoma are reviewed. The presentation of the tumor, symptomatology, investigation, and treatment are discussed. A recommended treatment regimen is outlined. Histologic typing is valueless in predicting tumor behavior. An illustrative and difficult case of recurrent base of skull esthesioneuroblastoma is presented. The resection performed is described, and the problem of extradural oropharyngeal communication is discussed. The solution was to use a temporalis and galeal frontalis flap. Reconstruction was with an external and intraoral prosthesis. Optimal treatment in a fresh lesion is radical surgery with or without radiation therapy. Esthesioneuroblastoma is a rare and often misdiagnosed malignant tumor of the olfactory epithelium. Originally described by Bergen et al. in 1924 as "esthesioneuroepithelioma olfactif," it was introduced into the North American literature by Schall and Lineback in 1951. Since then, fewer than 200 cases have been collected. The various terms used to describe it--olfactory esthesioneuroblastoma, esthesioneurocytoma, and olfactory neuroblastoma--all denote origin from the neural crest. The sensory nerves of smell are short bundles of fibers that originate in the olfactory bulb and pass through the cribriform plate to the olfactory area of the nasal mucosa. This mucosa is located in the most superior part of both nasal fossae. Thus the usual primary sites of occurrence include the superior nasal cavity or nasal septum, and turbinates, the ethmoid, or the cribriform plate, although an extranasal site of origin has been suggested. Symptoms are usually progressive and range from nasal obstruction or epistaxis to diplopia, ocular pain, and headaches in the more advanced disease state.(ABSTRACT TRUNCATED AT 250 WORDS)
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A rare case of olfactory neuroblastoma presenting symptoms as a brain metastasis is reported. A 47-year-old-man was admitted to Yamagata City Hospital complaining of head heaviness and headache attack. Neurologically, bilateral slight choked disc and right anosmia were found. The otolaryngologist in this hospital pointed out a reddish-purple polypoid tumor in the right nasal cavity. But there were no episodes of nasal bleeding or obstruction before admission. CT scan on admission showed the tumor density in the right upper nasal cavity and ethmoid sinus, which partially infiltrated into the orbital cavity, and enhanced heterogenous high density mass in the inner basal portion of the right frontal lobe. Carotid angiogram revealed a small tumor blush in the ethmoid region and avascular mass lesion in the right frontal region. On the 11th hospital day, surgical biopsy of the nasal tumor was performed by the otolaryngologist and the evident tumor cells in the fibrous tissue were observed. So the endonasal removal of the tumor and the total removal of the intracranial tumor were achieved. On operation, the continuity of intracranial tumor and nasal tumor was not observed. The histological pictures of both tumors were similar and the diagnosis was olfactory neuroblastoma. From histological and operative findings, we concluded the intracranial tumor was metastatic lesion from nasal origin. CT scan taken after the operation showed the tumor rested only in the right ethmoid sinus.(ABSTRACT TRUNCATED AT 250 WORDS)
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Esthesioneuroblastoma or olfactory neuroblastoma is a rare tumor of the nasal cavities and paranasal sinuses. In the majority of cases the cerebral involvement is secondary. Only ten cases have been reported in which the tumor was primarily intracranial. An eleventh case is reported and literature reviewed.
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The clinicopathologic features of 21 olfactory neuroblastomas are reviewed with emphasis on variables of potential diagnostic or prognostic value. The patients ranged in age from 9 to 66 years (median: 49 years); 57% were women. At the time of diagnosis, one tumor was Kadish stage A, 11 were stage B, and nine were stage C. Nineteen patients had extensive surgical resections, but in six instances these were considered incomplete. Six patients (29%) developed local recurrences and eight (38%) had metastases. Ten patients (48%) were alive and free of disease from 10 to 239 months (median: 58 months) after diagnosis. Seven (33%) died of tumor, with a duration of survival ranging from 1 to 142 months (median: 27 months). A fibrillary intercytoplasmic back-ground was the most useful diagnostic feature microscopically and was present, at least focally, in 18 of 21 tumors. A silver stain for neuronal processes was helpful when fibrils could not be seen on hematoxylin- and eosin-stained sections. Homer-Wright rosettes, when present, also aided in diagnosis but were well formed in only six tumors. The only clinical feature that correlated with prognosis was whether the neoplasm had been completely excised at initial presentation. Tumor necrosis was the only histologic feature correlating with prognosis, but neither association was statistically significant. Metastases, clinical stage, mitotic rate, and nuclear pleomorphism showed no relationship to survival in this series.
A transfacial approach and the use of a bone flap allows exeresis of large tumors of middle and posterior third of face and of base of skull. The procedure can be used alone or associated with a neurosurgical approach to the lesions. This wide surgical approach increases the possibility of a complete excision under conditions of strict hemostasis. Replacement of the bone flap at the end of surgery restores skeletal reliefs ad integrum with excellent esthetic results. The technique is described in detail and several examples of its use outtined.