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)

Seven monoclonal antibodies (mAbs) reactive with ganglioside II3(NeuAc)2-LacCer (GD3) were generated; four of these mAbs (DMAb-21, DMAb-22, DMAb-23, and DMAb-24) by immunizing mice with GD3 adsorbed to Salmonella minnesota and the remaining three (DMAb-7, DMAb-8, and DMAb-17) with melanoma line SK-MEL 28, which contains 1.4 nmol sialic acid of GD3 per mg protein. The specificities of the mAbs were defined by high-performance thin-layer chromatography (HPTLC) immunostain and solid-phase radioimmunoassay (SP-RIA) with a panel of purified gangliosides. DMAb-7 and DMAb-8 reacted with GD3, IV3(NeuAc)2nLcOse4Cer(3',8'-LD1), and very weakly with IV3(NeuAc)2II3NeuAcGgOse4Cer (GT1a), but not with II3NeuAc-LacCer (GM3), II3NeuAcGgOse3Cer(GM2), II3NeuAcGgOse4Cer (GM1), II3NeuAc, IV3NeuAcGgOse4Cer (GD1a), II3(NeuAc)2GgOse3(GD2), II3(NeuAc)2GgOse4Cer (GD1b), IV3NeuAcII3(NeuAc)2, GgOse4Cer(GT1b), suggesting the binding epitope to be a terminal tetrasaccharide NeuAc alpha 2-8NeuAc alpha 2-3Gal beta 1-4(Glc or GlcNAc). DMAb-7 and DMAb-8 were used to investigate the expression of GD3 on cultured human tumor cells of neuroectodermal origin. Thirteen of 19 gliomas, 3 of 5 medulloblastomas, 5 of 5 neuroblastomas, 2 of 2 melanomas, and 1 of 3 teratomas were shown to react with DMAb-8 and/or DMAb-7 by cell surface-RIA (CS-RIA) and immunofluorescence (IF) assays. HPTLC and densitometric analysis confirmed these results, as positive immunostains in the GD3 region were obtained with oligoganglioside fractions from 9 glioma, 1 medulloblastoma, 2 neuroblastoma, 1 melanoma, and 1 teratoma cell line. Glioma cell line U-105 MG and medulloblastoma cell line Daoy contain GD3 as shown by HPTLC immunostain analysis of extracts, although GD3 was undetectable on the cell surface as determined by CS-RIA and IF.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:GD3 expression by cultured human tumor cells of neuroectodermal origin. 260 39

A group of 2,576 autopsies of children of the age of 0 to 15 years (made between 1978 and 1987) comprised 14 congenital (neonatal) tumours (among 93 tumorous lesions). Teratomas were the most frequent congenital tumours followed by neuroblastomas (4 and 3 cases). Neuroblastomas grew from cervical and thoracoabdominal sympathicus and from the right adrenal medulla. The most frequent and most extent hematogenic dissemination concerned liver, microscopical examination found dissemination in various organs as well as a lymphatic spread to regional lymph nodes (in 2 cases). One case was characterized by an exclusive continual destructive growth in retroperitoneum and posterior mediastinum with 2 macroscopical secondaries in skeleton. Pathognomic differentiated structures were always found by light microscopy. Differential diagnosis of round cell tumours of infancy was discussed with a concise exposure of symptomatology and autoptic findings of neuroblastomas according to congenital cases from literature.
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PMID:[Congenital neuroblastomas]. 279 Oct 61

We reviewed the autopsy, clinical, and radiographic records of 39 patients with metastatic skeletal disease (age range 18 months-20 years). There were 11 different primary tumors responsible for skeletal metastases, of which neuroblastoma was the most common (16 patients). Other commonly occurring tumors giving rise to secondary skeletal deposits were rhabdomyosarcoma (seven), teratoma-teratocarcinoma (four), and Wilms tumor (three). Overall patient survival ranged from 2 weeks to 72 months, with an average of 10.2 months. Those patients presenting without skeletal metastases at the time of diagnosis of the primary tumor had a survival time averaging 16.2 months (range 3-72 months), whereas those patients presenting initially with skeletal metastases at the time of diagnosis of the primary tumor had a survival time of 8.5 months (range 0.5-23 months).
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PMID:Metastatic skeletal disease in the pediatric population. 298 4

A total of 452 cases of childhood malignant solid tumors were treated over the last twenty years at the National Children's Hospital. These included 175 cases of neuroblastoma, 64 cases of Wilms' tumor, 65 cases of malignant lymphoma, 45 cases of soft tissue sarcoma, 31 cases of hepatoma, 20 cases of malignant teratoma, 17 cases of testicular tumor, 7 cases of ovarian tumor and 28 cases of other forms of malignant solid tumor. Bone metastasis was observed in 62 of 175 cases of neuroblastoma, 3 of 64 cases of Wilms' tumor, one of 65 cases of malignant lymphoma, 4 of 45 cases of soft tissue sarcoma, one case of pulmonary blastoma and one case of osteogenic sarcoma, giving a total occurrence of bone metastasis in 72 of the 452 cases. The main sites of bone metastasis in neuroblastoma were the skull (61.4%), femur (56.8%), orbit (27.3%) and spine (22.7%). The average values of serum calcium and alkaline phosphatase activity showed no significant difference. The patients with bone metastasis were treated with a combination of radiation therapy and intensive chemotherapy, resulting in temporary improvement. The survival of patients with stage IV neuroblastoma with bone metastasis was worse than that of similar patients without bone metastasis.
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PMID:[Bone metastasis of malignant solid tumors in childhood]. 303 15

Ten children who underwent allogeneic (n = 5) or autologous (n = 5) bone marrow transplantation (BMT) for chronic myelogenous leukaemia (n = 2), acute lymphoblastic leukaemia (n = 1), acute myelogenous leukaemia (n = 2), severe aplastic anaemia (n = 2), malignant histiocytosis (n = 1), neuroblastoma (n = 1) and teratoma (n = 1) were assessed for endocrinological function. Transplant preparative regimens consisted of high-dose cyclophosphamide, high-dose cyclophosphamide in combination with high-dose busulphan, high-dose melphalan as well as BACT (BCNU, cytarabine, cyclophosphamide and 6-thioguanine) chemotherapy. None of the patients received total body irradiation (TBI). Median survival following BMT was 37 months (range 7-115). Growth hormone deficiency was present in only one patient; none of the patients had abnormal thyroid or adrenocortical function. This is in contrast to previous reports in which growth hormone deficiency and abnormal thyroid and adrenocortical function occurred in a much higher percentage of patients after BMT conditioned with TBI.
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PMID:Endocrine function after bone marrow transplantation without the use of preparative total body irradiation. 304 94

Fifty-seven patients with advanced malignant tumours were treated with ifosfamide (Holoxan) and mesna (Uromitexan) in our department from November 1979 to December 1984. This series comprised eight cases of soft tissue sarcoma, nine cases of ovarian carcinoma, five cases of non-seminomatous testicular tumour, 11 cases of bronchogenic carcinoma, three cases of renal carcinoma, seven cases of non-Hodgkin's lymphoma, two cases of skeletal fibrosarcoma, two cases of breast carcinoma, one case each of Ewing's tumour, prostatic carcinoma, seminoma, plasma cell tumour, multiple myeloma, malignant teratoma, nasopharyngeal carcinoma, Wilms's tumour, neuroblastoma and mycosis fungoides. Out of these 57 cases, 53 were evaluable. There were five complete remissions and 20 partial remissions, corresponding to a total response rate of 47%. The overall median survival time (MST) of the 53 evaluable patients was 7.5 months. The responders had a longer survival time (MST 10 months) than the non-responders (MST 4.75 months) (p greater than 0.05). Analysis of the results according to sex, age, dosage of ifosfamide and degree of histological differentiation of the tumour cells failed to show any influence of these factors on the therapeutic results. The response rate to ifosfamide found in this study might be related to the histological origin of the tumours and to whether the primary tumours had been resected. The non-seminomatous testicular tumours, non-Hodgkin's lymphomas and ovarian carcinomas showed a high response rate. The response rate was higher in the group in which the primary tumour had been resected (61%) than in the non-resected group (12%) (except the non-Hodgkin's lymphoma). The side-effects of this regimen were moderate. Dyspepsia, nausea, vomiting, myelodepression, dizziness, and alopecia were common. Cystitis could be prevented nearly completely by concomitant administration of mesna, when given correctly, for preventing side-effects of ifosfamide on the urinary system (haemorrhagic cystitis, etc.).
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PMID:Treatment of advanced malignancies with ifosfamide under protection with mesna. 313 Mar 16

This paper describes the immunohistochemical staining properties of four monoclonal antibodies (MAbs) (CF, EB, AD, and KB) which had been previously shown to be specific for purified neuron-specific enolase (NSE) by a solid-phase radioimmunoassay. In this study, the authors immunostained a spectrum of normal and neoplastic neuronal, "neuroendocrine," and nonneuronal tissues fixed in formalin and embedded in paraffin. Positivity was generally restricted to normal neuronal structures and neuronal tumors, including adrenal neuroblastoma, ganglioneuroblastoma, olfactory neuroblastoma, pheochromocytoma, carotid body paraganglioma, duodenal gangliocytic paraganglioma, and teratoma with neuroepithelial components. Three staining patterns of the normal or neoplastic neuronal structures were observed: two MAbs (CF and EB) stained predominantly the nerve fibers (axoplasm); one (AD) stained predominantly the cell bodies (perikaryon); and one (KB) stained both the axoplasm and the perikaryon. "Neuroendocrine" tumors such as pulmonary small cell carcinoma, pancreatic islet cell tumor, thyroid medullary carcinoma, and carcinoid tumors from various locations showed a variable staining pattern. Tumor cells undergoing mitotic division were usually positive regardless of type. Normal structures other than neuronal or "neuroendocrine," including normal glial cells, were negative. The authors also studied a range of glial cell tumors with MAbs CF and AD as well as with Dako polyclonal antiserum to NSE. The results showed that CF stained the axonal fibers in the normal white matter surrounding these tumors; it did not stain the tumor cells or the perikarya of neurons in the surrounding normal gray matter. AD stained the glioma cells as well as the perikarya and dendrites of neurons in the surrounding normal gray matter; it did not stain the axonal fibers in the surrounding normal white matter. By contrast, the polyclonal antiserum stained all of these structures. The high degree of staining specificity of the MAbs should prove them to be valuable in immunohistochemical diagnosis of tumors as well as in further understanding the role of NSE in neuronal differentiation.
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PMID:Immunohistochemical characterization of a set of monoclonal antibodies to human neuron-specific enolase. 328 44

The sonograms and medical records of 49 patients were reviewed to determine if there is a characteristic sonographic appearance for certain neck masses. Neck masses included: inflammatory masses (12), noninflammatory masses (23), and thyroid masses (14). Four entities including thyroglossal duct cyst, fibromatosis colli, cystic hygroma, and multiple lymphadenopathy showed characteristic sonographic appearance. Thyroglossal duct cysts were seen as midline or slightly off midline cystic masses. A sinus tract extending superiorly was nicely demonstrated in one patient. The mass in fibromatosis colli (neonatal torticollis) appeared as a well defined mass clearly within the sternocleidomastoid muscle, uniformly echogenic, but less echogenic than the normal surrounding muscle, without good through-transmission. A cystic or primarily cystic mass with linear septations was the characteristic finding of the cystic hygroma. Multiple lymphadenopathy demonstrated multiple discrete, oval, relatively hypoechoic masses along the cervical lymphatic chain. The sonographic appearance of inflammatory masses was variable, being either inhomogeneously echogenic or of mixed echogenicity. Hemangiomas were either echogenic with cystic vascular spaces or linear septations or relatively homogeneously echogenic. Intrinsic thyroid masses could be distinguished from extrinsic masses in most cases. The demonstration of calcification in a mass was useful in narrowing the differential diagnosis. Although it is not a specific finding, the presence of calcification highly suggests a neoplastic lesion, particularly neuroblastoma or teratoma. Not only can the location, extent, and internal characteristics of a mass be determined, but in certain entities, the sonographic appearance is characteristic and an accurate diagnosis can be made.
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PMID:Sonography of neck masses in children. 351 42

The great vessels' interspace, in the pediatric retroperitoneum, deserves special attention during abdominal sonographic examination. By rotating the child into the right anterior oblique position, the full length of this interspace is demonstrated. Normally, it has a uniform appearance. When invaded by disease, the sonographic pathology can be identified and differentiated from the surrounding structures. The studies were gathered from experience with 1658 retroperitoneal sonographic examinations. Fourteen children were found with disease involving the great vessels' interspace: 4 patients with lymphoma, 3 patients with sympathetic ganglioneuroblastoma, 2 patients with retroperitoneal rhabdomyosarcoma, and one case each, respectively, of adrenal neuroblastoma, Wilms' tumour, clear cell carcinoma of the kidney, retroperitoneal teratoma and Toxocara canis.
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PMID:The sonographic evaluation of the great vessels' interspace in the pediatric retroperitoneum. 352 17

Two children with ganglioneuroblastomas in different locations underwent fine needle aspiration (FNA). Clinically, the tumor in the presacral area was diagnosed as a benign teratoma and the retroperitoneal tumor as a neuroblastoma. Both tumors were correctly diagnosed preoperatively as ganglioneuroblastomas by FNA cytology. The smears showed the characteristic Homer-Wright rosettes, ganglion cells and fibrillar material.
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PMID:Fine needle aspiration cytologic diagnosis of ganglioneuroblastoma. 367 62


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