Gene/Protein Disease Symptom Drug Enzyme Compound
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Query: UMLS:C0027627 (metastases)
 103,950 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Diabetes mellitus, steatorrhea, cholelithiasis and a tumor distorting the duodenum prompted a work-up for somatostatinoma in a 52-year-old man. The responses of pancreatic B-cells but not of A-cells to nutrient stimuli were inhibited, and growth-hormone release was suppressed, suggesting somatostatin resistance in some target tissues. Plasma somatostatin-like immunoreactivity ranged from 9000 to 13,000 pg per milliliter (normal: 88+/-8, mean +/- S.E.M.) and was distributed in four molecular forms, including free somatostatin. The primary tumor contained 5 microgram of somatostatin-like immunoreactivity per milligram of wet tissue, distributed in three of the molecular forms noted in plasma. Plasma calcitonin was also elevated (4650 pg per milliliter; normal: less than 120). Immunocytochemical studies showed that cells of the primary tumor contained somatostatin and calcitonin but no other peptide hormones. Only somatostatin was present in the metastases. Somatostatin was localized electron microscopically in all secretory granules, irrespective of size and shape, whereas calcitonin was present only within a single subpopulation of small granules in the same cells.
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PMID:Somatostatinoma syndrome. Biochemical, morphologic and clinical features. 37 80

The effects of octreotide in vivo and in vitro on hormone release, in vivo [123I]Tyr3-octreotide scanning, and in vitro [125I]Tyr3-octreotide autoradiography were compared in five patients with endocrine pancreatic tumors. [123I]Tyr3-octreotide scanning localized the primary tumor and/or previously unknown metastases in four of the five patients. The patient with a negative scan had an insulinoma that did not respond to octreotide in vivo. No Tyr3-octreotide-binding sites were subsequently found at autoradiography of the tumor, whereas somatostatin-14 receptors were present at a high density. In parallel, culture studies with the cells prepared from this adenoma showed that insulin release was not affected by octreotide, while both somatostatin-14 and -28 significantly suppressed hormone release. Culture studies of the tumor cells from two gastrinomas showed a dose-dependent inhibition of gastrin release by octreotide. Octreotide exerted direct antiproliferative effects in one of these gastrinomas, which had been shown to be rapidly growing in vivo. Both gastrinomas had specific somatostatin receptors, as measured by in vitro receptor autoradiography. Somatostatin release by the cultured somatostatinoma cells from one of these patients was suppressed by octreotide. In conclusion, 1) the [123I]Tyr3-octreotide scanning procedure is valuable in the localization of primary endocrine pancreatic tumors as well their often clinically not yet recognized metastases; 2) the in vitro detection of somatostatin receptors in those tumors that were also visualized in vivo after injection of [123I] Tyr3-octreotide indicates that the ligand binding to the tumor in vivo indeed represents binding to specific somatostatin receptors; and 3) the parallel between the presence of somatostatin receptors on tumors and in in vivo and in vitro effects of octreotide on hormonal release from these tumors indicate that a positive scan predicts a good suppressive effect of octreotide on hormonal hypersecretion by these tumors.
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PMID:Parallel in vivo and in vitro detection of functional somatostatin receptors in human endocrine pancreatic tumors: consequences with regard to diagnosis, localization, and therapy. 216 29

Endocrine pancreatic tumors are slowly growing neuroendocrine neoplasms with a malignant potential which may cause symptoms such as hypoglycemia, multiple ulcers, diarrhea, flush, hyperglycemia and skin rash. A prospective study was performed on 84 patients with endocrine pancreatic tumors. In 59 patients (70%) the tumors were malignant. Of the 84 patients, 23 had insulinomas, 25 gastrinomas, 20 nonfunctioning tumors, 14 the WDHA syndrome, 1 somatostatinoma and 1 glucagonoma. The median age at diagnosis was 53 years and the median delay from first symptom to diagnosis was 2 years. The most common site of the pancreatic primary tumor was the tail (41%), and metastases were most frequently located in the liver (60%) and lymph nodes (44%). Plasma chromogranin A + B was elevated in 94%, serum pancreatic polypeptide (PP) in 74%, plasma neurotensin in 67% and serum gastrin in 62%. Serum HCG-alpha and -beta subunits were elevated in 41 and 30% respectively, all except 3 having a verified malignant tumor. The median survival from first symptom and diagnosis was 14.2 and 8.7 years respectively. Patients with MEN-1 had a significantly better survival from diagnosis than sporadic cases (median 15.1 versus 5.8 years). Patients who received interferon after failing chemotherapy had a significantly better survival than those given chemotherapy alone (5-year survival 65 and 50% respectively).
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PMID:Neuroendocrine pancreatic tumors. Clinical findings in a prospective study of 84 patients. 247 25

To define the course of malignant pancreatic islet cell tumors, 20 patients seen over 14 years with these neoplasms were reviewed. The 12 men and 8 women ranged in age from 22 to 76 years, with a mean of 52. Seven functional tumors included three insulinomas, two glucagonomas, one gastrinoma, and one somatostatinoma. One insulinoma was associated with a multiple endocrine neoplasia type I (MEN-I) syndrome. The 13 patients with nonfunctioning tumors had abdominal pain (3), jaundice (2), and steatorrhea (2). Seven had a palpable abdominal mass. Diagnosis of malignancy was based on local invasion (4), distant metastases (15), or both (1). One patient had percutaneous biopsy of a hepatic metastasis. All others had laparotomy for diagnosis and/or treatment. Each patient had a single tumor except the patient with MEN-I syndromes, who had multiple tumors throughout the pancreas. The head was involved in seven patients, the body in seven, and the tail in five. Operations included six curative and three palliative resections, five biliary diversions (two with concomitant enteric bypass), and five biopsies. Palliative resections were done for hormonal or local symptoms such as gastrointestinal (GI) bleeding and pain. Multiple chemotherapeutic agents were used, but the best results were obtained with DTIC (50% response). Four patients had radiation for liver, brain, or bone metastasis, with some improvement. Of five patients who had curative resections, four are alive 15 to 144 months, with a mean of 75 months. One died six years after diagnosis. Of the remaining 15 patients who had liver metastasis, seven patients are alive 8 to 168 months later, with a mean of 87 months.(ABSTRACT TRUNCATED AT 250 WORDS)
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PMID:Islet cell carcinoma of the pancreas. 254 52

A case of malignant somatostatinoma is reported in a patient with long-standing dermatitis herpetiformis and coeliac disease. The patient had non-specific abdominal pain of several years duration and came to attention because of weight loss despite strict adherence to a gluten-free diet. Plasma somatostatin levels were raised, and laparotomy showed a pancreatic tumour with metastases, which on histology, electron microscopy and immunohistochemistry proved to be a somatostatinoma. After a promising initial response to streptozotocin, she died 30 months later. This is the first reported occurrence of a somatostatinoma in a patient with coeliac disease, adding to the growing list of neoplastic complications in this condition.
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PMID:Malignant pancreatic somatostatinoma in a patient with dermatitis herpetiformis and coeliac disease. 289 27

Insulinoma, glucagonoma, gastrinoma (Zollinger-Ellison syndrome), vipoma, somatostatinoma and a tumor that secretes human pancreatic polypeptide are the primary endocrine-secreting tumors of the pancreas. hormones are produced by specific tumor cell types and cause a variety of dramatic clinical pictures. Diagnosis often requires hormone assays. Computerized tomography may be helpful. Definitive surgical treatment is possible, but metastases may be present.
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PMID:Primary endocrine-secreting pancreatic tumors. 610 68

Somatostatin-like immunoreactive polypeptides with approximate molecular weights of 1,500-2,000 (peak I), 2,500-3,500 (peak II), and 10,000-15,000 (peak III) were isolated from the peripheral plasma and from extracts of tumor tissue of a patient with a pancreatic somatostatinoma and hepatic metastases. The peak I component had approximately the same molecular charge as somatostatin, but the peak II component was appreciably more basic. The peak III component was heterogeneous with respect to charge and dissociated into smaller immunoreactive polypeptides during isoelectric focusing or after treatment with strongly denaturing solvents. In plasma, the levels of the larger molecular weight components (peak II and peak III), relative to that of the somatostatin-size component (peak I), were higher than in tissue extracts, suggesting a lower rate of clearance of the larger polypeptides from the circulation. In response to a nutrient stimulus, the peak I component increased to a greater extent than the larger components. Plasma levels of larger immunoreactive polypeptides relative to the peak I component in patient's plasma were higher after complete resection of the pancreatic tumor than preoperatively, suggesting that the rate of release of these putative precursor forms from the metastases was greater than from the primary tumor.
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PMID:Characterization of somatostatin-like components in the tumors and plasma of a patient with a somatostatinoma. 610 68

Ultrasonographic imaging was performed on 49 patients who had pancreatic islet cell tumors, including examples of all six known tumor varieties. In beta cell insulinomas, ultrasonography was of little value for tumor visualization, because these tumors tend to be small and the patients frequently are obese. On the other hand, ultrasonography was very effective for evaluating the nonbeta cell tumors, detecting both primary pancreatic neoplasms and hepatic metastases in patients with glucagonomas, somatostatinoma, non-functioning tumors, and the WDHA syndrome. In detection of the Zollinger-Ellison syndrome, ultrasonography compares favorably with previously reported gastrinoma detection by angiography and CT scanning.
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PMID:Ultrasonic investigation of pancreatic islet cell tumors. 630 3

A case of Von Recklinghausen's disease with duodenal somatostatinoma is reported. A search of the world's literature revealed 27 patients with Von Recklinghausen's disease associated with an immunohistologically proved duodenal somatostatinoma. Twenty-nine cases of duodenal somatostatinoma not associated with Von Recklinghausen's disease and 32 cases of pancreatic somatostatinomas have been identified for comparison. While their histology may be similar in many respects, the duodenal and pancreatic somatostatinomas show significant differences, especially in hormonal and growth behaviors. In contrast to its pancreatic counterpart, the duodenal somatostatinoma is frequently associated with Von Recklinghausen's disease, is seldom associated with a recognizable "somatostatin syndrome," often contains psammoma bodies, and is less frequently associated with demonstrable metastases at the time of operation.
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PMID:Von Recklinghausen's disease associated with duodenal somatostatinoma: contrast of duodenal versus pancreatic somatostatinomas. 774 81

A case of a pancreatic somatostatinoma in a 54 year old male is presented. A pancreatic carcinoma with hepatic metastases were primary diagnosed, and the patient had a palliative choledochoduodenostomy and gastroenteroanastomosis. As he was still alive four years later, the histological samples were reevaluated. Immunohistochemically the tumor was found positive for somatostatin, neuron-specific enolase and pancreatic polypeptide. Symptoms, diagnosis, pathology and treatment in relation to somatostatinomas are discussed.
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PMID:[Somatostatinoma in the pancreas]. 806 87


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