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)

Glia-derived nexin (GDN), also known as protease nexin I, is a serine protease inhibitor of deduced relative molecular mass 41,700, identified in conditioned media of glioma cells by its neurite-promoting activity. GDN can promote neurite outgrowth in vitro from neuroblastoma cells, sympathetic neurons and hippocampal neurons (L. Farmer et al., manuscript in preparation). In vivo, GDN is constitutively expressed in all parts of the olfactory system, where axonal regeneration and neurogenesis occur continuously throughout life. This observation indicates that GDN could be important for axonal regeneration in vivo. To investigate this possibility, we have taken advantage of the fact that damage to nerves in the peripheral nervous system leads to their regeneration, whereas in the central nervous system no such regeneration can occur. Here we report that after lesion of the rat sciatic nerve there is a large transient increase in the amount of GDN messenger RNA and of released GDN. The cells showing GDN immunoreactivity are mainly localized distal to the lesion site. These results further support the suggestion that GDN is important for axonal regeneration in vivo, and indicate that protease inhibitors could have a role in Wallerian degeneration and peripheral nerve regeneration.
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PMID:Induction of glia-derived nexin after lesion of a peripheral nerve. 268 11

Sinonasal neoplasms and neoplasm-like proliferations composed of light microscopically poorly differentiated or undifferentiated, small- to medium-sized cells cause considerable diagnostic confusion. Lesions in this category include lymphoepithelioma (undifferentiated carcinoma), olfactory neuroblastoma, small-cell undifferentiated (oat cell) carcinoma, sinonasal undifferentiated carcinoma, malignant melanoma, pituitary adenoma, lymphoid hyperplasia, malignant lymphoma, plasmacytoma, lymphomatoid granulomatosis, rhabdomyosarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, Ewing's sarcoma, and synovial sarcoma. Many of these lesions can be definitively diagnosed based on light microscopic features alone, but, in some instances, additional techniques such as immunohistochemistry are of value. The authors review the pertinent clinicopathologic features of the above lesions, with emphasis on light microscopic, immunohistochemical, and ultrastructural features of particular utility in differential diagnosis.
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PMID:"Undifferentiated" neoplasms of the sinonasal region: differential diagnosis based on clinical, light microscopic, immunohistochemical, and ultrastructural features. 269 5

Fifteen patients with olfactory neuroblastoma were treated during the 17-year period of 1969 to 1986. Data was analyzed with respect to age at presentation, sex, presenting signs and symptoms, stage, and results of treatment. Age ranged from 4 to 67 years with the median age being 27 years. Median follow-up was 8 years. Local control was achieved in nine of nine patients or 100% with successful surgical resection, i.e., minimal residual disease, followed by postoperative radiation therapy (45 to 65 Gy) was employed. There were no distant failures when the primary site was controlled. Regional lymph node metastases were infrequent: only 13% (two of 15 patients) presented with positive nodes. Three of four patients treated initially with surgery alone had a local recurrence, two of which were successfully salvaged by combined therapy. There were four patients treated with radiation therapy alone: three had persistent disease after radiation therapy, and one patient was controlled with 65 Gy. Olfactory neuroblastoma has a propensity to recur locally when treated with surgery alone. The authors' experience suggests excellent local control can be achieved with surgery immediately followed by radiation therapy. Thus the authors recommend planned combined treatment for all resectable lesions.
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PMID:Olfactory neuroblastoma. 272 May 89

Olfactory neuroblastoma is a malignant, slow growing, neurogenic tumor that originates in the olfactory mucosa of the upper nasal cavity. We report a case of olfactory neuroblastoma that presented with exuberant hyperostosis of the facial bone during the course. This unusual radiological finding has been discussed with literature review.
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PMID:[A case of olfactory neuroblastoma with exuberant hyperostosis]. 281 Aug 30

Primitive malignant melanoma of the nasal fossae is rare and amelanotic melanoma of the same site is highly unusual. The present work reports a case where positive diagnosis was only possible by means of ultrastructural study. Giving evidence of the premelanosomes, this technique made it possible to differentiate from other types of cancers (in particular from olfactory neuroblastoma, non differentiated small cell carcinomas and lymphoma). Although in all cases we are dealing with malignancies, the need for a precise histological diagnosis is fundamental to work up an adequate therapeutic protocol. Given the difficulties in getting an electronic microscope in any hospital, the method used in the present case is quite significant since it made precise diagnosis possible even on bioptic fragments fixed in non buffered formalin. Finally some problems regarding diagnosis and treatment linked to diagnosis by means of electronic microscopy are discussed.
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PMID:[Primary amelanotic melanoma of the nasal fossa. Importance of ultrastructural study for diagnosis]. 281 54

The olfactory neuroblastoma or esthesioneuroblastoma is a rare neuroectodermal tumor originating from the olfactory neuroepithelium, which can metastasis via cerebrospinal fluid pathways. In the present case of an extensive nasal malignancy with cervical lymph node metastases in a 75-year old woman, its difficult histology alternatively led to a diagnosis of anaplastic carcinoma and non-Hodgkin lymphoma. The patient died from complications following spread of the tumor to the spinal cord and cauda equina. Review of the literature shows that this tumor is notorious for its chameleonic character. In view of its clearly demonstrated malignancy an aggressive therapeutic approach is advocated.
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PMID:Olfactory neuroblastoma with spinal metastasis--a problem in diagnosis. 302 77

Seven patients with olfactory neuroblastoma were treated, using the staging classification proposed by Kadish (stage A two patients, stage B one, stage C three, and local recurrence one patient). Treatment was not uniform. Radiation therapy was employed initially as the only treatment modality in three patients who had stage A, B, and C, respectively. In one instance, it was used to treat a local recurrence after surgical treatment. Surgery and radiation therapy was employed in three patients: one with stage A and two with stage C. Chemotherapy was used as an adjuvant to radiation and surgery in one patient with stage C, and in three individuals, it was used for advanced disease. Four of the seven patients are alive without evidence of disease from 23 months to 16 years 8 months after treatment. A review of 11 series from the recent medical literature is presented. It is essential to follow these patients for long periods of time because of the slow growth and the propensity of local recurrence that some of these tumors have. The actual tendency in the management of this neoplasm seems to be combined therapy with surgery and radiation. The role of adjuvant chemotherapy is not well established, but has been recommended for locally advanced disease.
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PMID:Olfactory neuroblastoma. Observations on seven patients treated with radiation therapy and review of the literature. 305 35

A 2-year-old boy presented with sudden blindness and epistaxis resulting from olfactory neuroblastoma with intracranial extension. This disease is uncommon in children, with only 27 cases reported in patients less than 17 years of age. This article reviews the clinical and histological features of this tumor and discusses treatment. Therapy has generally consisted of surgery and radiation. Our child and 2 others treated with radiation and combination chemotherapy (including cyclophosphamide and anthracyclines) have done well with follow-ups of up to 9 1/2 years. Chemotherapy is an important form of adjuvant therapy in this disease.
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PMID:Olfactory neuroblastoma (esthesioneuroblastoma) in a 2-year-old boy. 315 27

Esthesioneuroblastoma is an uncommon tumor arising from the olfactory epithelium of the nasal vault. Differentiation from other tumors is often difficult, but techniques like immunocytochemistry and electron microscopy can help to distinguish a neuroblastoma from a malignant lymphoma with certainty. A patient is presented in whom difficulty was experienced in deciding on the diagnosis; the pathological data are presented to illustrate why confusion about the finite diagnosis can occur despite the use of these techniques. The initial diagnosis was a malignant lymphoma of the poorly differentiated lymphocytic type. The disease responded well to chemotherapy with cyclophosphamide, vincristine, procarbazine, and doxorubicin (COPA). When tumor recurred, rebiopsy of the small cell tumor was considered to be esthesioneuroblastoma, and the patient was treated with cyclophosphamide, vincristine, dacarbazine, and radiotherapy. The patient is now disease-free and has been without evidence of disease for more than 2.5 years; it is more than 5 years since the initial diagnosis of a small cell malignant neoplasm was made. This report illustrates the problems of diagnosis and treatment of this rare condition.
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PMID:Esthesioneuroblastoma or malignant lymphoma? A case report. 318 32

We present a case of giant olfactory neuroblastoma with special reference to a new operative approach in cases of dumbbell-shaped tumor in the anterior cranial fossa. Our new approach is characterized as follows: resection of the bilateral orbital roof and frontal sinus en bloc, following bifrontal conventional craniotomy. Accordingly, retraction of the frontal lobe is minimized and it is possible to obtain, with ease, a good operative field extending to the region adjacent to the tuberculum sellae. Using this technique combined with the transsphenoidal approach, even in the case of marked extracranial downward extension of a dumbbell-shaped tumor, it should be possible to remove the tumor totally. This approach should open a way to a wide range of application.
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PMID:[Operative approach to a dumbbell-shaped tumor in the anterior cranial fossa. A case of olfactory neuroblastoma]. 321 Dec 81


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