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)

The prognosis of children with neuroblastoma (NB) is dependent upon the patient's age at diagnosis, the location of the primary tumor, and histologic tumor cell differentiation. These characteristics, as well as the presumption that NB results from clonal expansion of primitive cells involved in sympathetic nervous system (SNS) development, predict that a model of tumorigenesis based upon normal fetal SNS histogenesis might indicate tumor progenitor status and define biologic and clinical behavior. Immunohistochemistry and in situ hybridization were used to examine a panel of marker gene products predicted or shown to be expressed during SNS development in the normal human fetal SNS from 8 to 24 weeks' gestational age. A similar analysis was performed in a selection of clinical NB tumors, and the results were compared. In a subset of differentiating, often extra-adrenal NB tumors in patients who frequently had a favorable outcome; advancing morphologic tumor cell differentiation spatially paralleled an advancing fetal extra-adrenal chromaffin marker gene expression phenotype (ie, increasing TrkA, TrkC, TH, IGF-2, and neuron-specific enolase expression but a lack of phenylethanolamine N-methyltransferase expression). In these tumors, expression of gene products associated with normal fetal sympathetic ganglionic differentiation (ie, Bcl-2, HNK-1, and neuropeptide Y) was lost with morphologic tumor cell differentiation. In contrast, undifferentiated tumors, the majority of which were high stage, adrenal in origin, and prognostically unfavorable, displayed marker expression characteristics mirroring that of an early fetal ganglionic lineage. Thus, we show that morphologic differentiation in stroma-poor NB tumors, long held as an important prognostic feature in tumor grading systems, often corresponds to an extra-adrenal chromaffin rather than a ganglion cell or adrenal medullary chromaffin phenotype. Understanding the biology of extra-adrenal chromaffin tissues may provide an explanation for the clinically less aggressive nature of differentiating NB tumors and suggest potential mechanisms for spontaneous regression and/or treatment response.
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PMID:A developmental model of neuroblastoma: differentiating stroma-poor tumors' progress along an extra-adrenal chromaffin lineage. 894 Dec 12

Four cases of mixed neuroendocrine-neural tumors composed of pheochromocytoma and neuroblastoma elements (including ganglioneuroma and ganglioneuroblastoma) were studied for the presence of catecholamine-synthesizing enzymes, neuroendocrine markers, and peptide hormones with clinicopathological correlations. Paroxysmal hypertension with hypercatech olaminemia was observed in 3 patients. One patient had an extremely elevated level of dopamine. The location of the tumor was in the adrenal glands in 2 patients and in the retroperitoneum in the other 2. Numerous electron-dense granules in the cytoplasm and neural processes with abundant neurotubules were characteristic of mixed neuroendocrine-neural tumors. Immunohistochemical study revealed that catecholamine-synthesizing enzymes were present in both components of the pheochromocytoma and neuroblastoma group, but phenylethanolamine N-methyltransferase was detectable only in epinephrine-producing tumors. Chromogranin and neurofilament immunoreactivities were present in both components; however, the intensity of chromogranin immunoreactivity was stronger in pheochromocytoma than in the other components. In contrast, neurofilament positivity was stronger in the neuroblastoma group than it was in pheochromocytoma. Multiple peptide hormones were immunoreactive in both components. Neuropeptide Y and met-enkephalin-positive cells were numerous in both; cells containing vasoactive intestinal peptide and somatostatin were less common but were comparatively more frequently found in ganglion cells than in pheochromocytoma cells.
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PMID:Adrenal and retroperitoneal mixed neuroendocrine-neural tumors. 3235 42