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)

Primary neuroblastomas obtained before therapy from 36 patients were studied to determine the frequency of tumors expressing a specific glycosphingolipid, GD2 ganglioside. Total tissue gangliosides were purified by a new partition method, quantitated, and analyzed by high-performance thin-layer chromatography. All 36 neuroblastoma tumors, representing all clinical stages, contained GD2 ganglioside. The mean relative and absolute concentrations of GD2 were substantial (12% of the total tissue gangliosides and 50 nmol/g of tissue) and were independent of the clinical stage of the tumor. In contrast, 6 samples of related but more differentiated tumors (ganglioneuroblastoma and ganglioneuroma) had little or no detectable GD2 (less than or equal to 1.5% of total gangliosides and less than or equal to 4 nmol/g of tissue). These results suggest that GD2 is a sensitive marker for neuroblastoma tissue and may be an excellent target antigen for immunotherapy of this tumor.
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PMID:Expression of GD2 ganglioside by untreated primary human neuroblastomas. 394 Feb 9

MAb were derived from mice immunized with cells of the human neuroblastoma line IMR-32. Five hybridomas were selected according to their selective binding to human cell lines, tumors and normal tissues. One of them, CE7, reacted with all sympatho-adrenomedullary cells (neuroblastoma, ganglioneuroblastoma, ganglioneuroma, pheochromocytoma, adrenal medulla, sympathetic ganglion cells). Weak cross-reactivities were observed with melanocytes and with some human melanoma and glioma cell lines. The antigen recognized by CE7 was markedly expressed on neuroblastoma tumors of all histological grades, independently of the adrenergic or cholinergic nature of these cells. MAb derived from clones AD2, BC1, BC4 and CB10 bound variably to some, but not to all, neuroblastoma cells. By using these MAb, 3 phenotypes of neuroblastoma lines could be distinguished. The binding profiles of these types, however, showed no correlation with origin of the cell lines or stage of the disease.
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PMID:Production and characterization of monoclonal antibodies against human neuroblastoma. 394 22

The authors report a case of malignant peripheral nerve sheath tumor arising in an adrenal ganglioneuroma in an adult. This is the first such case occurring in the absence of a history of childhood neuroblastoma treated with radiation, and provides evidence that such a transformation can occur spontaneously. The neoplasm demonstrated a highly malignant biologic behavior with rapid growth, local recurrence, and metastasis.
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PMID:Malignant peripheral nerve sheath tumor arising in an adrenal ganglioneuroma in an adult male homosexual. 395 8

The phenomenon of familial neuroblastoma is discussed in the context of case reports describing disseminated neuroblastoma in two of three half-brothers who share a common unaffected mother and who each have a different father. This family's cytogenetics proved to be unremarkable; also, the mother's peripheral blood DNA did not show tumorigenic capacities in transfection-nude mice experiments. An analysis of reported cases permits an updated examination of the clinical features of this entity and defines the limits of genetic counseling of families of all neuroblastoma patients. Multiple primaries are a hallmark of familial neuroblastoma. Most diagnoses are made in the first 18 months of life and at ages that fall within 12 months of the age of diagnosis of the other affected family member. Difficulties in determining the incidence and penetrance of an inherited susceptibility to neuroblastoma derive from undiagnosed tumors that have undergone regression or spontaneous maturation to benign ganglioneuroma, as well as from early deaths or long-term treatment complications that preclude reproduction and multigenerational pedigrees. Nevertheless, the risk of neuroblastoma in siblings or offspring of the large majority of persons with neuroblastoma appears to be less than 6%. Recent observations concerning chromosomal aberrations and oncogenes in embryonal malignancies are presented in an integrated model of tumorigenesis that corresponds to clinical experience.
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PMID:Familial neuroblastoma. Case reports, literature review, and etiologic considerations. 395 26

Neuroblastoma is one of the malignant childhood tumors which have a dismal prognosis, but is known to occasionally mature into benign histology spontaneously. Two cases with either stage III or IV neuroblastoma, whose tumors had not been extirpated at the initial operation, were treated by intra-aortic PGE1 infusion combined with oral papaverine and conventional anti-malignant chemotherapy. This therapeutic modality was effective in one case, where the tumor decreased in size and was removed in a second exploratory operation. The histology of the extirpated tumor was benign ganglioneuroma. In this case, however, distant bone metastases were found after the second operation. The role of PGE1 in the mechanism of bone metastasis remains to be determined.
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PMID:[Intra-aortic PGE1 infusion combined with anti-malignant chemotherapy in the treatment of advanced neuroblastoma]. 404 Jul 36

Neuroblastoma is one of the commoner tumors of infancy and childhood. There is great variation in the histological picture and even within one tumor. One unique feature is the apparently high rate of spontaneous regression, particularly during the first year of life. There is also a tendency for neuroblastoma to mature to the more benign ganglioneuroma and recent in vitro studies suggest that a serum factor may influence this process. Approximately 90 percent of patients with neuroblastoma excrete abnormally high quantities of various catecholamines, thus providing a useful diagnostic tool and a means for evaluating the effect of therapy. Treatment requires a multidisciplinary team approach involving a surgeon, radiotherapist and chemotherapist. Prognosis is influenced by a number of host factors and the most important of these seem to be the patient's age at diagnosis and the extent of the disease, although some children with widespread disease appear to have a particularly good prognosis. It is difficult to evaluate the influence of chemotherapy on survival in patients with neuroblastoma but it has not been of great significance. The unique biologic characteristics of this tumor require further study in the hope of providing more effective therapy.
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PMID:Recent advances in neuroblastoma. 462 80

Ultrastructural study of neuroblastoma group tumors including 7 neuroblastomas, 4 ganglioneuroblastomas, and 2 ganglioneuromas was performed by using both TEM and SEM. Tumor cells showed a wide variation comparable to the developmental stages of nerve cells and were classified into four types according to the neuritic process projections; namely apolar, monopolar, bipolar, and multipolar. Neuroblastoma was composed of small cells of apolar, monopolar, and bipolar types with few multipolar cells. Tumor cells ganglioneuroblastoma and ganglioneuroma were multipolar type. SEM observation demonstrated characteristic varicosities in the elongated neuritis processes, and by TEM examination the dilated portions of these varicosities revealed disruption or disappearance of parallel runnings of the microtubules and microfilaments. TEM examination demonstrated presence of Schwann cells in ganglioneuroblastoma and ganglioneuroma, and satellite cells and perineurial cells in ganglioneuroma. The hitherto undescribed cells with caveolar structure which are thought to be a precursor of these stromal elements were disclosed in ganglioneuroblastoma. In order to evaluate the maturation and prognosis of tumors of this group, stromal differentiation seems to be equally important as ganglionic differentiation because both stromal elements and tumor cells have a common origin of neural crest.
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PMID:Transmission and scanning electron microscopic studies on the tumors of neuroblastoma group. 628 64

Thirteen human neuroblastoma and six Wilms' tumor biopsies have been analyzed for neuron specific enolase (NSE). THe relative activity of NSE in the neuroblastomas (including ganglioneuroblastoma and ganglioneuroma) ranged from 28% to 62.5% of total enolase activity. The corresponding figures for the Wilms' tumors were 1% to 4.5%. It appears that NSE can serve as a biochemical marker for neuroblastoma and be useful in the differential diagnosis of neuroblastoma and Wilms' tumor.
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PMID:Neuron specific enolase: a marker for differential diagnosis of neuroblastoma and Wilms' tumor. 628 8

The Verner-Morrison syndrome has been described in 19 previous patients with ganglioneuroma and ganglioneuroblastoma but never neuroblastoma. Its occurrence following treatment of a neuroblastoma with chemotherapy with maturation of the tumor has only been reported on one previous occasion. Our case suggests that vasoactive intestinal polypeptide may be used not only as a diagnostic indicator for the presence of a neural crest tumor but also as a marker to monitor maturation of the tumor and indicate an improving prognosis.
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PMID:Vasoactive intestinal peptide producing neuroblastoma. 629 96

Two hundred and two benign and malignant soft tissue lesions were studied for the presence of S-100 protein by means of the peroxidase-antiperoxidase technique on formalin-fixed, paraffin-embedded tissue. Virtually all benign nerve sheath tumors (neurofibroma, neurilemoma, and granular cell tumor) contained numerous immunoreactive S-100-positive cells. Only one-half (18 of 36) of malignant schwannomas contained the protein, suggesting that its presence is an expression of differentiation in Schwann cell tumors. S-100 protein was not identified within pure neuroblastic tumors (neuroblastoma, neuroepithelioma) but could be identified within rare cells of the ganglioneuroblastoma and within the Schwann cell component of ganglioneuroma. It was also identified within most melanocytic tumors (cellular blue nevus, clear cell sarcoma, and melanoma). In fact, its constant presence in melanoma indicates that it may prove to be an independently reliable method for diagnosing amelanotic forms. It is also sporadically present within a variety of mesenchymal lesions including lipoma, liposarcoma, synovial chondromatosis, chondrosarcoma, fibromatosis, histiocytosis X, and chordoma. Although S-100 protein is highly characteristic of neural crest-derived tumors, it is not restricted to them and, consequently, must be interpreted cautiously. It may prove helpful in select situations such as the distinction of (a) benign nerve sheath tumors from other benign mesenchymal tumors such as fibrous histiocytomas, (b) cellular neurilemomas from malignant schwannomas, (c) malignant schwannomas from conventional fibrosarcoma (d) malignant melanomas from many carcinomas, and, possibly (e) juvenile xanthogranulomas from histiocytosis X.
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PMID:Value of S-100 protein in the diagnosis of soft tissue tumors with particular reference to benign and malignant Schwann cell tumors. 631 Feb 27


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