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Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

A case of olfactory neuroblastoma occurring in a 3-year-old girl is reported. The rarity of the lesion in early childhood is stressed and discussed with the clinico-pathological characteristics of the tumour, which are apparently more aggressive the younger the patient. Olfactory neuroblastoma should be considered as a possible diagnosis regardless of the age of the patient.
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PMID:Infantile olfactory neuroblastoma. A clinicopathological study with review of the literature. 38 19

Olfactory neuroblastoma is a malignant neoplasm with a varied biological behavior. Its clinical course is unpredictable and there is no correlation between its microscopic features and biological behavior. The present study deals with light and ultrastructural characteristics of two cases of olfactory neuroblastoma of the nasal cavity. In one patient, the definitive diagnosis was established on the basis of ultrastructural features of the lesion. The most consistent fine structural findings were the presence of intracytoplasmic densecored neurosecretory granules, "true" and "pseudo-" rosettes, and the neuritic processes emanating from the tumor cells. On the basis of their biochemical, histochemical, and ultrastructural characteristics, olfactory neuroblastomas are similar to neuroblastomas arising from the adrenals or sympathetic nervous system. These findings, therefore, support the hypothesis that olfactory neuroblastomas are most likely of neural crest origin and thus belong to a group of neoplasms collectively known as "apudomas" or neurocristomas. The literature review strongly favors combined surgery and postradiation as the most effective treatment of olfactory neuroblastoma.
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PMID:Olfactory neuroblastoma (esthesioneuroblastoma): a light and ultrastructural study of two cases. 38 58

Olfactory neuroblastoma is an uncommon malignant tumor originating in the olfactory epithelium in the roof of the nose. It usually produces nasal obstruction and secondary sinus disease and is often mistaken by the physician for a nasal polyp. Olfactory neuroblastoma has been observed to cause death by distant metastasis or by invasion through the cribriform plate and secondary meningitis in most instances, and is generally associated with a low cure rate. During the past eight years, we have treated three patients with olfactory neuroblastoma by a combination of extensive local surgery using the lateral rhinotomy approach with ethmoidectomy and medial partial maxillectomy, combined with either preoperative or postoperative irradiation therapy. From a review of the literature we have tried to draw meaningful conclusions regarding treatment.
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PMID:Olfactory neuroblastoma. Management and prognosis. 80 2

Nasal neuroblastoma, esthesioneuroblastoma, is frequently difficult to distinguish from the more common poorly differentiated epidermoid carcinoma of the nasal cavity and nasopharynx. We present a simple alternate method to electron microscopy, formaldehyde-fume-induced fluorescence, to demonstrate biogenic amine granules in neoplastic cells. This method is more specific and more sensitive, since it reveals the presence of biogenic amines, not merely membrane-bound granules, and it deals with larger quantities of tissue, thus avoiding some of the sampling errors inherent in electron microscopy. We also describe the histochemical relationship of this tumor to other neural crest neoplasms.
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PMID:Fume-induced fluorescence in diagnosis of nasal neuroblastoma. 124 26

Seventeen patients with olfactory neuroblastoma seen at the Massachusetts General Hospital and Massachusetts Eye and Ear Infirmary from 1941 to 1971 are presented. Data on age, sex, symptoms, physical, and roentgenographic findings, and therapeutic results are evaluated. A system of pretherapy staging is proposed in which for Group A, the tumor is limited to the nasal cavity; in group B, the tumor is localized to the nasal acvity and paranasal sinuses; and in group C, the tumor extends beyond the nasal cavity and paranasal sinuses. Olfactory neuroblastoma is a radioresponsive, and, to a limited extent, radiocurable tumor and varies in aggressiveness. Of 17 patients in this series, 13 or 76% were alive without disease following treatment by surgery, irradiation, and combination of these two methods. Uncontrolled primary lesions with or without metastases accounted for all therapeutic failures. A treatment policy for this disease is presented.
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PMID:Olfactory neuroblastoma. A clinical analysis of 17 cases. 126 Jun 76

Diagnostic classification of poorly differentiated, round cell, primitive neuroectodermal neoplasms, including Ewing's sarcoma, peripheral neuroepithelioma, Askin's tumor, and esthesioneuroblastoma, is challenging to the surgical pathologist using conventional histopathologic approaches because of very similar and overlapping morphologic and cytologic features. Furthermore, distinguishing these neoplasms from neuroblastoma, embryonal rhabdomyosarcoma, small cell osteogenic sarcoma, and non-Hodgkin's lymphoma can be difficult. This paper describes and reviews the cytogenetic and molecular genetic changes in these tumors and demonstrates how the ability to detect these changes has enabled a greater understanding of the histogenesis, classification, diagnosis, and prognosis of these neoplasms.
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PMID:Cytogenetic and pathologic aspects of Ewing's sarcoma and neuroectodermal tumors. 133 Aug 77

Olfactory neuroblastoma (esthesioneuroblastoma) is an extremely rare tumour arising from the olfactory epithelium of the nasal cavity close to the cribriform plate. Most institutions will have little experience in recognising the clinical and histological features, or management of this tumour and reliance is placed on researching the literature when the individual patient presents. This study reviews seven patients with olfactory neuroblastoma treated at the Queensland Radium Institute from 1971 to January 1990. The overall local control rate in this series is 57% (four of seven patients) and 43% of patients (three of seven) remain alive. Conservative surgery and post-operative radiotherapy is recommended for early disease and more radical surgery with post-operative radiotherapy for advanced disease.
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PMID:Olfactory neuroblastoma--management of a rare tumour at the Queensland Radium Institute and literature review. 181 31

Esthesioneuroblastoma is a rare tumor in children, and the correct diagnosis may be difficult, as is demonstrated in this case report. A 5-year-old girl was diagnosed with this tumor, which was incurable and behaved like a neuroblastoma, sending metastases to the bone marrow and invading the cranium and the spinal canal.
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PMID:A child with esthesioneuroblastoma with metastases to the spinal cord and the bone marrow. 199 Feb 59

We report a 16-year-old boy with esthesioneuroblastoma that presented with a unilateral tumor extending to the maxillary sinus and periorbital region. Despite initial therapy with gross resection, 5,682 cGy to the tumor bed and chemotherapy, the patient subsequently had a rapid local recurrence with distant metastases. Immunocytochemical, ultrastructural, cytogenetic, and molecular techniques were performed to determine if this tumor was biologically similar to childhood neuroblastoma. Urinary excretion of vanillylmandelic acid (VMA) and homovanillic acid (HVA) were markedly elevated. Chromogranin and neuron specific enolase immunostaining of tumor cells was positive, as seen in neuroblastoma. Electron microscopic studies showed cells that were closely packed and connected by occasional cell junctions. The cell cytoplasm contained moderate amounts of filaments and microtubules. Numerous electron dense granules were observed; however, these granules lacked distinct nucleoids and generally reacted strongly for acid phosphatase, indicating a lysosomal rather than a secretory function. Tumor cells contained near-pseudotetraploid chromosomes, with all chromosomes represented at least three times, and chromosome 5 was present in multiples of eight. Clonal structural abnormalities included 2q+ and 5q+ and multiple double minutes. Northern blot analysis revealed both c-myc and N-myc expression; however, N-myc amplification was not demonstrated, and c-myc expression appeared increased, unlike cases of rapidly progressive neuroblastoma. These results suggest that despite biologic similarities to neuroblastoma in catecholamine excretion and some ultrastructural features, molecular genetic abnormalities differ in this comparatively aggressive case of estesioneuroblastoma.
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PMID:Cytogenetic and molecular evaluation of clinically aggressive esthesioneuroblastoma. 202 81

Expression of insulin-like growth factor I (IGF-I) mRNA by some tumor cell lines of neuroectodermal origin has been described. To further explore the significance of IGF-I mRNA expression in these tumors, a more extensive analysis was performed. Most (9 of 10) neuroectodermal tumor cell lines with a t(11;22) translocation (primitive neuroectodermal tumor [PNET], Ewing's sarcoma, esthesioneuroblastoma) expressed IGF-I mRNA, whereas 0 of 15 cell lines without the translocation (PNET, neuroblastoma) expressed IGF-I. Furthermore, inasmuch as all neuroblastoma (12 of 12) cell lines examined expressed IGF-II RNA, the pattern of IGF expression could distinguish between these closely related tumors. CHP-100, a PNET cell line with the t(11;22) translocation, was shown to secrete both IGF-I protein and an IGF binding protein, IGFBP-2. This cell line also expressed the type I IGF receptor mRNA, and blockade of this receptor by a monoclonal antibody (alpha IR3) inhibited serum-free growth. These data demonstrate that IGF-I expression is a property of neuroectodermal tumors with a t(11;22) translocation and that interruption of an IGF-I autocrine loop inhibits the growth of these tumor cells.
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PMID:Insulin-like growth factor I expression by tumors of neuroectodermal origin with the t(11;22) chromosomal translocation. A potential autocrine growth factor. 217 8


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