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)

Catecholamines are biogenic amines that play an important role in the nervous system. Some catecholamines have been used as tumor makers of phenochromocytoma, paraganglioma and neuroblastoma. The analysis of total catecholamine metabolites should be useful for one-shot screening of multiple aspects of diseases; however, it is difficult to do this, because the catecholamine metabolites are divided into three groups: five amines, one amino acid and three carbonic acids. Catecholamines and small molecules were separated from plasma proteins by an internal-surface reversed-phase column (protein-coated octadeyclsilica column) and were analyzed by liquid chromatography (LC)/mass spectrometry (MS) using electrospray ionization time-of-flight MS. Using a reversed-phase column and hydrophilic mobile phases, we succeeded in the separation of nine catecholamines, all of which had similar structures. These nine substances were eluted in the following order: norepinephrine, epinephrine, normetanephrine, dopamine, metanephrine, 3,4-dihydroxyphenylalanine, vanillomandelic acid, 3,4-dihydroxyphenylacetic acid and homovanillic acid. The reproducibility of this method was acceptable. The highest coefficient of variation was 7.4%. In addition, various types of compounds were separated from and detected in plasma proteins by applying LC/MS. The plasma direct injection method, which uses an internal-surface reversed-phase column and an ion-pair reagent, allowed us to separate small molecules from plasma proteins. MS detected some compounds that high-performance LC could not succeed in separating and detecting with UV detection. We think that the method can be applied to find new markers in neuroblastoma, by comparing the plasma of patients with that of normal infants. The method can be also used to help in making a diagnosis of other diseases and finding their new makers.
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PMID:Pretreatment and one-shot separating analysis of whole catecholamine metabolites in plasma by using LC/MS. 1679 60

A significant diversity of tissue types interface at the base of the skull and contribute to the diagnostic challenges of skull base surgical pathology. Advances in surgical technique now permit biopsy and resection of lesions previously termed "inoperable." Retrospective review was made of all pathology specimens from skull base surgeries performed at the University of California Davis Medical Center from 1990 to 1996. Surgical biopsies and resections were performed on 186 patients who had 33 distinctive diagnoses. Any preoperative biopsy or tissue from referring institutions was reviewed prior to skull base surgery. One hundred eighteen patients had benign lesions, the most frequent of which were pituitary adenoma (55) and acoustic neuroma (27). Other benign lesions included angiofibroma, meningioma, fibrous dysplasia, and paraganglioma. Sixty-eight patients had malignant tumors, 32 of which were squamous cell earcinoma. Other malignancies included salivary carcinomas, basal cell carcinoma, neuroblastoma, melanoma, and several sarcomas. Unexpected findings were two metastatic carcinomas and five inflammatory lesions. Nearly 1500 intraoperative consultations were performed to establish resection margins and less commonly to confirm the diagnosis. The discrepancy rate between the intraoperative and final diagnosis was 1.8%. Immunohistochemistry and/or electron microscopy was utilized in 44% of the specimens to confirm the diagnosis. Surgical pathology is an essential ingredient to a successful skull base surgery program. Pathologists are involved in both pre- and intraoperative decisions. The diversity of lesions that arise from the skull base often has overlapping histologies that require careful attention to morphology and the use of ancillary studies for accurate diagnosis. The need for frequent intraoperative interpretations contributes to the significant challenge for the surgical pathologist.
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PMID:Surgical pathology of the skull base: a 7-year experience. 1717 Oct 65

Paragangliomas may have composite forms in which they combine features of a typical paraganglioma with those of a neural component consisting of either neuroblastoma, ganglioneuroblastoma, or ganglioneuroma. These variants are rare and generally located in the adrenal. Herein, we describe a retroperitoneal, extra-adrenal composite paraganglioma-ganglioneuroma of a 57-year-old woman. Radiological evaluation revealed a nodular mass with apparent pancreatic location, with findings suggestive of an endocrine tumor, yielding the diagnosis of a pancreatic endocrine tumor. At histology the neoplasm, strictly adhering to the external surface of the pancreatic gland but well demarcated, displayed a main central region with typical paraganglioma features and cells arranged in cords and in a nesting "zellballen" pattern, positive for neuroendocrine markers, and a distinct peripheral area consisting of dense bundles of wavy spindled Schwann cells, with scattered ganglionic cells.
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PMID:Extra-adrenal composite paraganglioma with ganglioneuroma component presenting as a pancreatic mass. 1959 1

Treatment with (131)I-metaiodobenzylguanidine (MIBG) has been introduced to the management of neuroendocrine tumors (NET) nearly 30 years ago. It provides efficient internal radiotherapy of chromaffin tumors (neuroblastoma, pheochromocytoma, and paraganglioma), but also of carcinoid and other less frequent tumors. Although for various NET types the role of this treatment form decreased by the emergence of peptide receptor radionuclide therapy, (131)I-MIBG still remains the primary radiopharmaceutical for targeting chromaffin tumors with outstanding efficiency. Results in neuroblastoma with overall response rates around 30% in refractory or recurrent diseases have been improved by combinations with chemotherapy, radiosensitizers, and autologous stem cell support. For adult chromaffin tumors, that is, pheochromocytoma and/or paraganglioma, (131)I-MIBG therapy is currently the most efficient nonsurgical therapeutic modality and applies for inoperable, disseminated disease. The antisecretory effect with powerful palliation of symptomatic disease (response rate: 75%-90%) should also be considered when judging treatment benefit. The results in carcinoid tumors are less pronounced, primarily achieving arrest of tumor growth, and most importantly effective functional control. With the presence of peptide receptor radionuclide therapy, (131)I-MIBG remains the alternative radionuclide in this tumor entity, for example, for patients with renal impairment. Another worthwhile mentioning indication-although less prevalent-are metastatic medullary thyroid carcinomas, especially if functioning. These patients are good candidates for this treatment form in the absence of reasonable surgical options and presence of diagnostic MIBG uptake. This article outlines the current status, results, and methodological improvements of (131)I-MIBG therapy.
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PMID:131I-metaiodobenzylguanidine therapy of neuroblastoma and other neuroendocrine tumors. 2011 83

Nuclear medicine can image some tumors by means of receptor specific radiopharmaceuticals, and offers the possibility to characterize cancer through the detection of its receptor expression. This is the case of neuroendocrine tumours (NETs), that are visualized by different radiolabelled somatostatin analogues that bind 5 distinct somatostatin receptor types (named sstr1-5) that show different tissue distribution. The subtypes sstr2 and sstr5 are the most commonly expressed in NETs. Until now the most widely used radiolabelled somatostatin analogue for planar and single photon emission computed tomography (SPECT) has been [(111)In]pentetreotide, because of its commercial availability. Other analogues labelled with gamma emitting radionuclides are [(99m)Tc]EDDA/HYNIC-TOC, [(99m)Tc]P829, [(111)In]DOTA-lanreotide, [(111)In]DOTA-NOC-ATE, [(111)In]DOTA-BOC-ATE. However, these compounds have not been successful for the routine use. Moreover, NETs express various receptors that can be depicted by different radiopharmaceuticals, such as [(123)I]VIP and [(111)In]GLP-1. Besides this, some precursors of the catecholamines metabolism, as meta-iodo-benzyl-guanidine (MIBG), labelled with (123)I or (131)I, accumulates in neuroendocrine tissues, in particular those of sympathoadrenal lineage. MIBG scintigraphy is currently indicated for neuroblastoma, paraganglioma and phaeocromocitoma. An impressive technological progress has been achieved recently with PET and, in particular, with the development of hybrid instrumentations (PET/CT) combining nuclear imaging with radiological imaging providing both functional and morphologic information. Among positron emitting tracers, the [(18)F]FDG is the most diffuse in oncology, but other more effective tracers are available for NETs, such as the analogues labelled with 68Ga. The diagnostic sensitivity and accuracy of these technology is superior to that of gamma emitting radiopharmaceuticals, but the fact that they are not still registered limits their use in the clinical practice. This overview summarizes the state of art of NETs imaging, focusing the attention mainly on gamma-emitting tracers.
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PMID:Imaging of neuroendocrine tumours with gamma-emitting radiopharmaceuticals. 2016 82

Familial paraganglioma/pheochromocytoma (PGL/PCC) is genetically heterogenous with mutations in three of the four subunits of the heterotetrameric mitochondrial complex II enzyme succinate dehydrogenase (SDH) being causally responsible for the majority of cases. In addition to PGL/PCC an array of non-paraganglial tumors have been described in affected individuals. We present a 30-year follow-up on the family of a deceased patient who synchronously developed malignant neuroblastoma (NBL), PCC, and renal cell carcinoma (RCC). Other family members with late onset disease have come to our attention, and molecular study revealed a mutation in the SDHB gene. Despite the embryologic relationship, NBL has been seen in only two previous patients with familial PGL/PCC, both with deletions of the SDHB gene. Review of the literature suggests the lack of a reported association between NBL and familial PGL/PCC may be an ascertainment bias. We further suggest that study of the SDH genes in NBL survivors who develop secondary solid tumors, particularly RCC, may correct this bias, and provide for more effective and comprehensive tumor screening in this patient population.
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PMID:Paraganglioma, neuroblastoma, and a SDHB mutation: Resolution of a 30-year-old mystery. 2050 30

Extraadrenal paragangliomas are most commonly found in the carotid body and are also found with lower frequency in the CNS. These lesions are derived from the sympathoadrenal lineage of neural crest cells. Here, the authors report a rare case of a composite paraganglioma with ganglioneuromatous components found at the filum terminale in a patient who presented with a brief history of low-back pain and paresthesias in the inguinal region. Immunohistochemical analysis of the resected lesion revealed admixed elements of neuroendocrine and neuroblastoma lineages, indicating the presence of divergent differentiation of sympathoadrenal progenitor cells. This case represents a unique opportunity to understand the cell fate of sympathoadrenal progenitor cells. Here, the authors propose that paragangliomas at the filum terminale can revert to a neural crest cell precursor fate, giving rise to divergent neoplastic populations.
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PMID:Composite ganglioneuroma-paraganglioma of the filum terminale. 2051 59

Primary retroperitoneal neoplasms are uncommon in children. Retroperitoneal neoplasms are either mesodermal, neurogenic, germ cell ectodermal or lymphatic in origin. In general, primary retroperitoneal neoplasms in children have different spectrum and prevalence compared to those in adults. Neuroblastoma, rhabdomyosarcoma, benign teratoma and lymphoma are the common retroperitoneal neoplasms. In this review, the clinical and CT futures of common retroperitoneal neoplasms in children are described. Coarse, amorphous, and mottled calcification are very common in neuroblastoma. Paraganglioma tends to show marked and early enhancement and may present with clinical symptoms associated with the excess catecholamine. Sarcomas are often very large and have heterogeneous appearance. Imaging cannot be reliably used to identify the type of retroperitoneal sarcomas due to overlapped radiographic features. In children, lipoblastoma is the most common lipomatous tumor in the retroperitoneum. The percentage of visible fat in tumor varies depending on the cellular composition of the lesion. The CT characteristics of teratoma are quite variable, which may be cystic, solid, on a combination of both. Typically teratoma appears as a large complex mass containing fluid, fat, fat-fluid level, and calcifications. Lymphoma is often homogeneous on both enhanced and unenhanced CT scans. Necrosis and calcification are rare on CT. In conclusion, making a final histological diagnosis of retroperitoneal tumor base on CT features is not often possible; however, CT can help to develop a differential diagnosis and determine the size and extent of the retroperitoneal neoplasms.
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PMID:CT characteristics of primary retroperitoneal neoplasms in children. 2059 98

Metaiodobenzylguanidine (MIBG) is a tracer that selectively targets neuroendocrine cells. On this basis, radiolabeled iodinated-MIBG (I-131-MIBG) has been introduced as a molecular nuclear therapy in the management of neuroendocrine tumors, including neuroblastoma, pheochromocytoma, paraganglioma, neuroendocrine carcinomas, and other rare neuroendocrine tumors. Extensive work has been addressed to develop I-131-MIBG therapy: doses, therapeutic schemes, and efficiency. In this paper, we present an overview on I-131-MIBG therapy, with main focus on different aspects how to perform this treatment.
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PMID:I-131-MIBG therapies. 2205 46

The purpose of this systematic review was to investigate the effects of therapeutic radiopharmaceuticals in patients with different types of advanced neuroendocrine tumour (NETs). A literature search was carried out in MEDLINE and EMBASE from January 1998 to November 2010. The Cochrane Library (to Issue 10, 2010) and the Standards and Guidelines Evidence Inventory of Cancer Guidelines, including over 1100 English-language cancer guidelines from January 2003 to June 2010, were also checked. No existing systematic reviews or clinical practice guidelines based on a systematic review or randomised controlled trials focusing on this topic were found. Twenty-four fully published articles were abstracted and summarised: 16 articles focused on five peptide receptor radionuclide therapy ((111)In-DTPAOC, (90)Y-DOTALAN, (90)Y-DOTATOC, (90)Y-DOTATATE, and (177)Lu-DOTATATE) and eight focused on (131)I-MIBG treatment. Limited evidence from a historical comparison of studies in one centre supported that (177)Lu-DOTATATE might be associated with greater clinical outcomes compared with (90)Y-DOTATOC or (111)In-DTPAOC. The severe toxicities for (177)Lu-DOTATATE included hepatic insufficiency in 0.6%, myelodysplastic syndrome in 0.8% and renal insufficiency in 0.4% of patients in this study. Insufficient evidence suggested efficacy of (131)I-MIBG in adult NET patients, but the overall tumour response rate from (131)I-MIBG was 27-75% for malignant neuroblastoma, paraganglioma or pheochromocytoma. Haematological toxicities were the main severe side-effects after (131)I-MIBG and 4% of patients developed secondary malignancies in one study. To date, peptide receptor radionuclide therapy seems to be an acceptable option and is relatively safe in adult advanced NET patients with receptor uptake positive on scintigraphy, but patients' renal function must be monitored. (131)I-MIBG may be effective for malignant neuroblastoma, paraganglioma or pheochromocytoma, but its side-effects need to be considered. No strong evidence exists to support that one therapeutic radiopharmaceutical is more effective than others. Well-designed and good-quality randomised controlled trials are required on this research topic.
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PMID:Radionuclide therapy in neuroendocrine tumours: a systematic review. 2222 16


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