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Query: UMLS:C0677930 (primary tumor)
 20,210 document(s) hit in 31,850,051 MEDLINE articles (0.00 seconds)

Generally, the results of liver transplantation for metastatic liver disease have not been favorable. One exception has been the unique group of neuroendocrine tumors, the slow growth of which allows liver transplantation to effectively palliate and control symptoms. We report two cases: (a) A 51-year-old man who underwent orthotopic liver transplantation and resection of the pancreatic primary tumor for a nonfunctioning malignant neuroendocrine tumor with features of both carcinoid and islet-cell glucagonoma remains symptom-free and without evidence of tumor recurrence at 13 months follow-up. (b) A 47-year-old man who underwent orthotopic liver transplantation and Whipple resection for a metastatic islet-cell tumor in the head of the pancreas is fully recovered at 5 months follow-up.
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PMID:Liver transplant for metastatic neuroendocrine tumor. 169 94

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

In the Pittsburgh series of 1,000 orthotopic liver transplants (OLTx), from January 1981 to July 1987, the indication for transplantation in five patients consisted of unresectable hepatic metastases arising from endocrine tumors of gastrointestinal origin: glucagonoma, two patients; carcinoid, two, and gastrinoma, one patient. Three patients underwent resection of the primary tumor (two distal pancreatectomies and one ileal resection) at the time of the hepatic transplantation. All patients underwent extensive nodal dissection. Three of the five patients are alive with no evidence of residual disease after a median follow-up study of 12 months. Hepatic transplantation broadens the concept of radical excision of tumor and may be considered as a potential therapeutic approach for some highly selected instances of unresectable hepatic metastases arising from endocrine tumors of the intestine and pancreas.
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PMID:Transplantation of the liver for metastatic endocrine tumors of the intestine and pancreas. 253 98

In this study, liver metastases from a patient with a pancreatic glucagonoma producing the syndrome have been investigated histologically, ultrastructurally, and immunocytochemically. A comparison has also been made between the metastases and the primary pancreatic tumor investigated in a parallel study. In the metastatic tissue, glucagon-, pancreatic polypeptide (PP)-, and somatostatin-containing cells were found together with a majority of cells without any immunoreactivity. Glucagon-positive cells were much more numerous than PP- and somatostatin-immunoreactive cells. As in the primary tumor, double immunogold staining of ultrathin sections demonstrated the co-existence of glucagon and PP immunoreactivities in most of the granulated cells, but PP immunolabeling was often faint, so that it probably could not be revealed by the PAP method in light microscopical sections. Such a finding, together with the histological and ultrastructural features, is consistent with an ontogenic and phylogenetic primitiveness of the metastatic cell population.
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PMID:A malignant tumor of the pancreas producing glucagonoma syndrome: immunocytochemistry and ultrastructure of liver metastases and comparison with the primary tumor. 254 78

Seven patients with clinical features of the glucagonoma syndrome, including the characteristic rash, diabetes mellitus, and weight loss, were examined by CT. Computed tomography demonstrated a primary pancreatic tumor in all patients, and, in four, hepatic metastases were identified. The primary tumors, relatively large and solid in nature, varied in size from 2.5 to 6 cm in maximum diameter. The tumor was found in the tail of the pancreas in three patients and in the head of the pancreas in four. No obliteration of adjacent perivascular or peripancreatic fat planes was observed in any patient. Calcification was present in the primary tumor in three patients and in the hepatic metastases in one. All tumors that were studied angiographically were found to be hypervascular. In contrast to insulinomas, which are frequently quite small when clinically diagnosed, glucagonomas appear to attain considerable size prior to being clinically apparent. Thus, we conclude that CT ought to become the mainstay in the identification, localization, and staging of these tumors.
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PMID:CT evaluation of glucagonomas. 298 9

A patient with the classic manifestations of the glucagonoma syndrome and hepatic metastases is presented. Clinical improvement occurred after removal of the primary tumor by distal pancreatectomy and splenectomy. No further antitumor therapy has been needed over a 15 month postoperative follow-up despite metastatic disease.
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PMID:Metastatic glucagonoma. improvement after surgical debulking. 624 50

Glucagon-producing tumors of the pancreas are among the rarest forms of islet cell tumors. Two patients are described in whom the characteristic dermatitis, glucose intolerance, weight loss, and anemia of the glucagonoma syndrome were due to a metastasizing islet cell carcinoma. In both, removal of the primary tumor with a distal pancreatectomy brought marked relief of all clinical symptoms for 1- and 2-year periods. Because streptozocin, the usual chemotherapeutic agent for these tumors, is quite toxic and frequently unsuccessful dimethyltriazenoimidazole carboximide (DTIC) was used for recurrence after operation. The first patient began taking DTIC when his rash reappeared and his immunoreactive glucagon (IRG) level rose to 6,000 pg/ml (normal, less than 200 pg/ml). Within 3 months, his rash was gone, and IRG level was 75 pg/ml. The second patient developed a neoplastic gastrocolic fistula and was extremely emaciated. With DTIC, the fistula healed, and he gained weight and returned to work. His IRG level has decreased from 2,975 to 200 pg/ml. No side-effects were noted during chemotherapy. Temporary palliation of malignant glucagon-producing neoplasms can be achieved by cytoreductive surgery. When the life-threatening symptoms of this syndrome recur, DTIC chemotherapy seems indicated because of its safety and effectiveness.
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PMID:Operative and chemotherapeutic management of malignant glucagon-producing tumors. 626 42

Four patients with metastatic glucagonoma are described. Angiography demonstrated a small avascular primary tumor of the tail of the pancreas in one patient and large hypervascular tumors of the pancreatic head in the other three. Liver metastases, were hypervascular in all four. Including our 4 with 21 cases from the literature, glucagonomas show a 92% incidence of increased tumor vascularity--thus increasing the likelihood of successful angiographic diagnosis. The awareness of clinically subtle or atypical glucagonomas and use of plasma glucagon determination are important factors leading to early diagnosis of these neoplasms. Since angiography can localize the tumor, assess its extent, and detect hepatic metastases, it is essential to the detailed evaluation of glucagonomas.
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PMID:Glucagonoma and its angiographic diagnosis. 630 75

From the non-B-cell tumors of the pancreas the gastrinoma (Zollinger-Ellison-syndrome) is the best known. The glucagonoma and the vipoma are less known and less common. The diagnosis of these syndroms, produced by overproduction of hormones, is a clinical one. The radiodiagnost has to localize the primary tumor and to exclude metastases. The x-ray findings of the glucagonoma in 3 patients and of the vipoma in 1 patient are described.
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PMID:[Radiodiagnosis of glucagonoma and vipoma, 2 rare endocrine pancreatic tumors]. 633 Aug 2

This paper reports one case with glucagonoma syndrome that resection of the primary glucagonoma in the pancreas was done. There was metastasis one in liver and lymph node. The definitive diagnosis was established by immunohistochemical analysis. The patient by immunohistochemical analysis. The patient remained well for 27 months and gained 5 kg of weight. Another experiences of diagnosis and treatment of 8 cases of malignant glucagonoma collected in chinese literature were reviewed. The clinical specialty of glucagonoma syndrome. diagnosis and management were discussed suggesting two points: on diagnosis the clinical specialty is a important clues: palliative primary tumor resection may be significant clinical improvement and prolonged survival.
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PMID:[Glucagonoma syndrome report of one case and review of the chinese literature]. 772 Apr 33


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