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Query: UMLS:C0027651 (tumor)
 685,946 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Presentation of a 48-y old woman who developed a neuroendocrine tumor of the nasal cavities. This lesion progressed rapidly despite an extensive resection and repeated chemotherapy. The patient refused radiotherapy. Before her death, 28 months later, she exhibited a paraneoplastic Cushing-like syndrome. At autopsy, restricted to the brain, there was a 5 cm diameter tumor invading the frontal area without alteration of the hypothalamus or the pituitary gland. Routine histology and electron microscopy confirmed the neuroendocrine nature of the tumor. Immunohistochemistry revealed the tumor to be positive only for neurone specific enolase, negative for S-100 protein, neurofilament and ACTH. The pituitary gland was positive for most usual hormones (GH, PRL, TSH, LH, FSH) but only few cells were slightly positive for ACTH. Many Crooke cells were observed. These findings suggest that the tumor secreted an ACTH-like substance (not detected actually by immunochemistry) that stimulated the activity of the adrenal cortex but inhibited normal production of ACTH at the pituitary gland level.
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PMID:[Neuroendocrine tumor of the nasal cavity (esthesioneuroblastoma). Apropos of a case with paraneoplastic Cushing's syndrome]. 362 28

A tumor with the clinical and light microscopic appearance of an oligodendroglioma that occurred in the lateral ventricles of a 25-year-old man is described. On further study this tumor proved to have the ultrastructural features typical of neuroendocrine tumors, and the presence of neuron-specific enolase was demonstrated by immunoperoxidase staining. This unusual presentation of a neuroendocrine tumor, which was entirely amitotic and free of atypia, raises important questions concerning both the true incidence of such cerebral differentiated neuroblastomas and their biologic behavior. The importance of electron microscopy and immunostaining techniques, which should be used more frequently to uncover additional cases of this tumor, is stressed.
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PMID:Differentiated cerebral neuroblastoma: a tumor in need of discovery. 399 44

Cell lines (COLO 320 DM and COLO 320 HSR), established from a human neuroendocrine tumor, contain an amplified cellular oncogene (c-myc). We have previously shown that the homogeneously staining regions (HSRs) of a marker chromosome in the COLO 320 HSR cells that evolved in culture from COLO 320 DM cells contain amplified c-myc. Molecular hybridization in situ has now been used to demonstrate that the HSRs are on both arms of what was once an X chromosome. We also show that amplified c-myc copies are present in the isolated double minute chromosomes (DMs) of the COLO 320 DM cells that were characteristic of the tumor cells initially established from the patient. The results suggest that the amplified c-myc appeared first as DMs and was subsequently transposed to engender HSRs on an X chromosome. The initial COLO 320 tumor cell may have acquired two "early replicating" (i.e., active) X chromosomes and lost the "late replicating" (i.e., inactive) X.
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PMID:Evolution of karyotypic abnormalities and C-MYC oncogene amplification in human colonic carcinoma cell lines. 400 96

A 56-year-old woman presented with a sudden, severe hemorrhage per rectum. Angiography localized a jejunal tumor, which was excised. Light microscopy suggested a neuroendocrine tumor, but neither a smooth muscle tumor nor a lymphoma could be excluded. Electron microscopy showed dense cored, single membrane bound secretory granules 150--220 nm in diameter; myofilaments were not observed. Biochemical analysis of tumor tissue yielded considerable amounts of catecholamines. VMA, 5-HIAA, and metanephrines. These combined ultrastructural and biochemical observations establish the diagnosis of neuroendocrine tumor; however, in this case neither type of information is sufficiently specific to define the tumor as either a paraganglioma or a carcinoid. Although paraganglia and mucosal endocrine cells in the GI are currently thought to constitute distinct cell types, they share numerous structural and functional properties, and they are both thought to be part of the APUD cell system. These parallels and similarities are shared by the neoplasms derived from them which often display features of both. In the absence of specific granule types or specific substances isolated from tumor tissue, only the application of specific immunocytochemistry techniques may allow the precise "functional" classification of such tumors.
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PMID:Neuroendocrinoma of the jejunum: electron microscopic and biochemical analysis. 615 53

Two human neuroendocrine tumor cell lines derived from a colon carcinoma contain either numerous double minute chromosomes (COLO 320 DM) or a homogeneously staining marker chromosome (COLO 320 HSR). We found amplification and enhanced expression of the cellular oncogene c-myc in both COLO 320 DM and HSR cells, and we were able to show that the homogeneously staining regions of the COLO 320 HSR marker chromosome contain amplified c-myc. From previous and present karyotypes, it appears that the homogeneously staining regions reside on a distorted X chromosome. Therefore, amplification of c-myc has been accompanied by translocation of the gene from its normal position on chromosome 8 (8q24). Because double minute chromosomes were features of primary cultures from the original tumor, it seems reasonable to suspect that amplification of c-myc may have contributed to tumorigenesis.
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PMID:Homogeneously staining chromosomal regions contain amplified copies of an abundantly expressed cellular oncogene (c-myc) in malignant neuroendocrine cells from a human colon carcinoma. 630 Aug 69

Enolase is a glycolytic enzyme widely distributed in each mammalian tissue and consists of three distinct subunits alpha, beta, and gamma. In the brain enolase exhibits three dimetric isozymic forms: alpha alpha, alpha gamma and gamma gamma. The gamma protein subunit has recently been found to be identical with the nervous system-specific and species-nonspecific protein, 14-3-2; therefore, alpha gamma and gamma gamma types of enolase were characterized as neuron-specific enolase (NSE). NSE has been also detected in the pituitary gland, thyroid gland, adrenal medulla and pancreas, all of which contain neuroendocrine cells. Recently NSE was observed by immunostaining or radioimmunoassay in neuroendocrine tumor such as glucagonomas, insulinomas, gut carcinoids, medullary thyroid carcinomas or neuroblastomas. Furthermore, small cell carcinoma of the lung which has been known to frequently exhibit neuroendocrine properties was found to produce NSE. In this paper NSE as a tumor marker in various cancers was evaluated by immunostaining or enzyme immunoassay which was developed by a co-worker Kato. The data revealed that serum NSE was clinically useful as a tumor marker, especially a monitoring marker of disease extent. NSE productions were also observed in adenocarcinoma of the colon or the lung and large cell carcinoma of the lung as well as small cell carcinoma of the lung and the esophagus, all of which were considered to share the biochemical features of neuroendocrine tumor. The evidence challenges a speculation that small cell carcinoma of the lung has an origin separated from the other histological types of lung carcinoma. In this meaning NSE is an important tumor marker for both clinical medicine and basic research.
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PMID:[Neuron-specific enolase as a new tumor marker]. 634 46

The histogenesis of six mediastinal tumors was investigated ultrastructurally and immunohistochemically using monospecific antibodies against intermediate filament proteins. Four of the tumors, showing different appearances by light microscopy, displayed desmosomes and cytoplasmic tonofilaments, by electron microscopy, compatible with an epithelial thymoma. These cases also showed keratin positivity by immunofluorescence microscopy. One spindle cell tumor showed zonula adherens-type junctions, prominent collections of intermediate filaments, and abundant cytoplasmic neurosecretory granules consistent with a neuroendocrine tumor. In this tumor, neurofilaments could be demonstrated by immunofluorescence microscopy, a feature also consistent with a neuroendocrine tumor. One malignant tumor, lacking tonofilaments and desmosomes but showing a few primitive junctions, did not contain keratin but showed vimentin positivity. This suggests a mesenchymal origin and a diagnosis of primitive sarcoma. These cases illustrate the diagnostic usefulness of electron microscopy and immunohistochemical evaluation of intermediate filaments.
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PMID:Mediastinal tumors: ultrastructural and immunohistochemical evaluation of intermediate filaments as diagnostic aids. 668 33

Gastrointestinal neuroendocrine tumors are rare neoplasms that cause classic clinical syndromes because of the excess secretion of specific gastrointestinal hormones. The two most important tumors clinically are insulinomas and gastrinomas. The clinical management of patients with these disorders usually involves the localization and surgical removal of the responsible tumor. Many radiological techniques can be used for tumor localization, including preoperative and intraoperative ultrasound, endoscopic ultrasound, CT, MRI, radionuclide scanning, angiography, and venous sampling. However, there are conflicting claims as to the relative accuracy of these procedures, and many of these investigations are difficult to justify because of their high cost, degree of invasiveness, or lack of precise anatomic information that is obtained. If surgical resection of a neuroendocrine tumor is planned, intraoperative sonography should always be used to detect occult nonpalpable tumors and to discern the relationship of the tumor to vital adjacent pancreatic ductal anatomy. The choice of preoperative imaging is more controversial, and depends on the clinical problem, local expertise, and availability of imaging techniques. Sonography and contrast-enhanced helical CT are the most commonly used preoperative imaging methods, because of their relatively low cost and widespread availability. Radionuclide scanning with a somatostatin analogue, which is a relatively new procedure, may be valuable in patients with symptoms of tumor recurrence.
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PMID:Imaging of gastrointestinal neuroendocrine tumors. 757 79

Diagnosis of ectopic acromegaly was made in a 21-year-old female patient who 3 years before had undergone a right pneumectomy for a disseminated bronchial carcinoid. Plasma growth hormone-releasing hormone (GHRH) concentrations were markedly elevated (6440 ng/l; normal value < 100 ng/l), as were serum GH (187 micrograms/l; normal < 5 micrograms/l) and plasma insulin-like growth factor I (IGF-I) levels (6.7 U/ml; normal < 2 U/ml). Retrospective immunohistochemical examination of the carcinoid tumor was positive for GHRH and the tumoral content of GHRH was 2130 ng/g wet weight. Subcutaneous treatment with octreotide was begun and first resulted in a profound inhibition of GH hypersecretion, normalization of plasma IGF-I and only partial reduction of GHRH concentrations. However, the initial dose of 3 x 100 micrograms had to be increased gradually to 4 x 750 micrograms because of a progressive deterioration of the hormonal control. After 15 months of intermittent therapy, octreotide was administered by continuous sc infusion. This treatment improved compliance, allowed the daily dose of octreotide to be reduced to 1500 micrograms and normalized serum GH levels. A near-normalization of the plasma IGF-I concentrations was also obtained, whereas the suppression of plasma GHRH concentrations remained incomplete. Despite favorable evolution of the endocrine parameters, intramedullar metastases were diagnosed and required radiation therapy. This observation emphasizes the superiority of continuous over intermittent administration of octreotide in the treatment of ectopic acromegaly. It also shows that the somatostatin analog acts more at the pituitary level to inhibit GH secretion than at the site of the neuroendocrine tumor.
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PMID:Subcutaneous octreotide treatment of a growth hormone-releasing hormone-secreting bronchial carcinoid: superiority of continuous versus intermittent administration to control hormonal secretion. 758 49

Early and late results of proximal pancreatoduodenectomy were determined in a personal and consecutive series of 100 patients (64 men, 36 women, mean age 51.9 years). Final diagnoses were chronic pancreatitis in 35, idiopathic bile duct stricture in 1, carcinoma of the head of pancreas in 27, and other periampullary tumors in 37 (duodenal carcinoma 11, ampullary carcinoma 11, neuroendocrine tumor 10, cholangiocarcinoma 5). Mean follow-up period was 30.5 months (range 3.5-132.0 months). Resection was conventional (including distal gastrectomy) in 39 patients and conservative (retaining the stomach, pylorus, and duodenal cap) in 61 patients. Resection for inflammatory disease caused greater operative blood loss (mean 2.29 versus 1.75 L; p = 0.054) and a longer operative time (6.2 versus 5.2 hours; p = 0.040) than resection for neoplastic disease. There were four operative deaths, two from leakage of the pancreatic anastomosis; another two patients survived pancreatojejunostomy leaks. Twenty patients developed postoperative complications, seven of whom required reoperation. Good pain relief was obtained in 76% of patients with chronic pancreatitis, but five required completion distal pancreatectomy at a mean 22.8 months after the first resection. Mean survival of patients with pancreatic cancer was 13.2 months. Sixteen patients with other periampullary cancers are still alive 41.6 months after the operation.
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PMID:Resection of the pancreatic head with or without gastrectomy. 763 97


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