Gene/Protein Disease Symptom Drug Enzyme Compound
Pivot Concepts:   Target Concepts:
Query: UMLS:C0027819 (neuroblastoma)
 27,800 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Computed tomography (CT) and 123I- or 131I-meta-iodo-benzyl-guanidine (MIBG) scintigraphy were compared for accuracy in tumor detection in 47 patients with neuroectodermal neoplasms. MIBG concentration was found in 12 of 13 pheochromocytomas, 12 of 12 neuroblastomas, 5 of 9 carcinoids, and 1 of 4 glomus tumors. MIBG uptake was not observed in medullary thyroid carcinomas, oat-cell carcinomas, Merkel tumors, 1 gastrinoma, and 2 unclassified neuroectodermal neoplasms. With regard to the different tumor manifestations, the sensitivity in detecting pheochromocytomas, neuroblastomas, and carcinoids was 87%, 77%, and 100% for CT, and 83%, 100% and 71% for MIBG scintigraphy, MIBG scintiscan was superior in the detection of small adrenal pheochromocytomas (less than 1 cm diameter) and in the depiction of small bone metastases and bone marrow infiltration from neuroblastoma. In all, 25 cycles of high-dose MIBG therapy were performed in eight patients with surgically incurable tumors (4 malignant pheochromocytomas, 1 neuroblastoma, 3 carcinoids). The total therapeutic activity applied was 3.55-43.29 GBq 131I-MIBG. Tumor kinetics of MIBG were investigated before and during treatment. One patient with metastatic pheochromocytoma has been in complete remission for a follow-up period of 36 months since completion of treatment, and another patient is in partial remission. Tumor reduction or no change was observed in four patients. Two patients died of non-concentrating recurrence and metastases.
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PMID:[Diagnosis and therapy of neuroectodermal tumors]. 253 1

Synaptophysin, an Mr 38,000 integral membrane glycoprotein of neurotransmitter vesicles, has been identified in diverse primary neuroendocrine (NE) tumors of both neural and epithelial origin (Wiedenmann and co-workers, Proc Natl Acad Sci USA 1986; 83: 3500-3504). In the present study, metastases of several types of NE tumors, including medullary thyroid carcinoma, gastrinoma, insulinoma, small (oat) cell carcinoma of the lung, gastrointestinal carcinoid, and neuroblastoma, were examined for the presence of synaptophysin by immunocytochemistry, with the use of tissue sections as well as centrifuged cell suspensions and by immunoblotting of tumor proteins. The results show that expression of synaptophysin can be maintained during formation of metastases. Therefore, the authors propose that synaptophysin antibodies be used for the positive identification of metastatic NE tumors, notably in differential diagnosis. The possible implications of these findings for tumor diagnosis are discussed.
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PMID:Synaptophysin identified in metastases of neuroendocrine tumors by immunocytochemistry and immunoblotting. 311 96

Pituitary adenylate cyclase activating polypeptide (PACAP) is a newly discovered neuropeptide which exists in two biologically active forms: PACAP-38 consisting of 38 amino acids and PACAP-27, a peptide corresponding to the N-terminal 27 amino acids of PACAP-38. Both PACAPs are derived from a 176 amino acid precursor (preproPACAP) which in addition gives rise to a 29 amino acid peptide, designated PACAP-related peptide (PRP). The presence of the three preproPACAP-derived peptides (PACAP-38, PACAP-27 and PRP) in tumour tissue from nine patients with VIP-producing tumours (pancreatic carcinoma, neuroblastoma, ganglioneuroma and pheochromocytoma) and eleven patients with non-VIP-secreting tumours (gastrinoma, glucagonoma, somatostatinoma, neuroblastoma) was examined by specific radioimmunoassays. In seven out of the nine VIP-secreting tumours elevated concentrations of all the three preproPACAP-derived peptides were found compared with normal tissue, while the concentrations in the non-VIP-secreting tumours were within the normal range. PACAP-38 was in all cases the dominating peptide, the concentration ranging from 41 to 3606 pmol/g. When tumour extracts were fractionated on Sephadex G50 column, tricine gel electrophoresis or reverse-phase HPLC immunoreactive components corresponding to synthetic PACAP-38, PACAP-27 and human PRP were identified, suggesting that preproPACAP was fully processed. Immunocytochemical examination showed PACAP-immunoreactive cells in the tumour tissue which also stained for VIP. This co-localization of PACAP and VIP was confirmed by double-staining experiments on the same sections, demonstrating PHM/VIP mRNA and PACAP-immunostaining in the same cells.
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PMID:PreproPACAP-derived peptides occur in VIP-producing tumours and co-exist with VIP. 857 31

Combining anatomical and functional imaging can improve sensitivity and accuracy of tumor diagnosis and surveillance of pediatric malignancies. MRI is the state-of-the-art modality for demonstrating the anatomical location of brain tumors with contrast enhancement adding additional information regarding whether the tumor is neuronal or glial. Addition of SPECT imaging using a peptide that targets the somatostatin receptor (Octreoscan) can now differentiate medulloblastoma from a cerebellar pilocytic astrocytoma. Combined MRI and Octreoscan is now the most sensitive and accurate imaging modality for differentiating recurrent medulloblastoma from scar tissue. CT is the most common imaging modality for demonstrating the anatomical location of tumors in the chest and abdomen. Addition of SPECT imaging with either MIBG or Octreoscan has been shown to add important diagnostic information on the nature of tumors in chest and abdomen and is often more sensitive than CT for identification of metastatic lesions in bone or liver. Combined anatomical and functional imaging is particularly helpful in neuroblastoma and in neuroendocrine tumors such as gastrinoma and carcinoid. Functional imaging with MIBG and Octreoscan is predictive of response to molecularly targeted therapy with 131I-MIBG and 90Y-DOTA-tyr3-Octreotide. Dosimetry using combined anatomical and functional imaging is being developed for patient-specific dosing of targeted radiotherapy and as an extremely sensitive monitor of response to therapy. Both MIBG and Octreotide are now being adapted to PET imaging which will greatly improve the utility of PET in medulloblastoma as well as increase the sensitivity for detection of metastatic lesions in neuroblastoma and neuroendocrine tumors.
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PMID:Combining anatomic and molecularly targeted imaging in the diagnosis and surveillance of embryonal tumors of the nervous and endocrine systems in children. 1858 Oct 60

Our preliminary experience of Somatostatin Receptor Positive Tumour Scintigraphy (SRPTS) in the management of some rare neuroendocrine tumours is highlighted. Six patients were evaluated using SRPTS. A single patient each with Zollinger-Ellison syndrome, recurrent medullary carcinoma of thyroid and Stage IV neuroblastoma. Two patients with phaeochromocytoma, and one patient with suspected insulinoma were evaluated. SRPTS was useful in three of the six patients studied i.e. patient with gastrinoma, recurrent medullary carcinoma of thyroid and metastatic neuroblastoma. SRPTS although expensive is a useful and cost-effective approach of rare endocrine tumours and it role as a first line tool in the diagnosis is discussed.
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PMID:Preliminary report on somatostatin receptor imaging in rare endocrine tumours. 1983 44