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)

Clinical features, diagnostic accuracy of imaging study, and the outcome of surgical treatment for endocrine tumors of the pancreas were analyzed in 12 patients who underwent surgery for the past 16 years and 11 months. The 12 cases were classified into two groups: functioning tumors in 8 (insulinoma in 7 including one malignant case, and malignant glucagonoma in one), and nonfunctioning tumors in 4 including 3 malignant cases. In functioning tumors, tumor size was 2 cm or less in 6 benign cases, but 5 cm or more in 2 malignant cases. In nonfunctioning tumors, tumor size was larger, ranging from 3.5 to 8.0 cm. Diagnostic accuracy for localization of functioning tumors was 66.7% for US, 75.0% for CT, 66.7% for endoscopic retrograde pancreatography (ERP), and 50.0% for selective angiography, while all nonfunctioning tumors could be detected by any diagnostic imagings. Percutaneous transhepatic portal venous sampling for immunoreactive insulin was very helpful to localize insulinoma. Stenosis or obstruction of the main pancreatic duct on ERP and arterial encasement on angiography highly suggested a malignant tumor. Even for malignant cases with liver metastasis, resection of the primary tumor with debulking of metastatic disease or intraarterial infusion chemotherapy was considered to prolong patient prognosis.
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PMID:[Diagnosis and surgical treatment for endocrine tumors of the pancreas]. 813 24

Neuroendocrine pancreatic tumors are neoplasms derived from APUD cells, characterized by hyperincretion of several peptides of hormonal activity. The incidence of these tumor is low. They are usually classified according to the predominant secreted peptide: gastrinoma, insulinoma, VIPoma, glucagonoma. Insulinoma is the most frequent endocrine pancreatic tumor, characterized by a peculiar clinical picture due to insulin action. This neoplasm is prevalently benign (90%), and may cause symptoms due to hypo-glycemia such as epilepsy, asthenia, deep coma, dizziness, hunger and epigastric pain. Surgery still constitutes the principal therapy for insulinoma treatment, but an accurate tumor identification is necessary. Selective arteriography of the pancreas and new diagnostic investigations as intraoperative US, selective sampling of pancreatic veins with insulin Quick-RIA, aid the diagnosis and more precise localization of the tumor. When surgical therapy is not practicable, for diffuse metastases, octreotide has an inhibitory effect upon hormone release, and may be combined with chemotherapy for controlling clinical symptoms. We review the clinical records of 2 patients from our Institute, who had hyper-insulinism due to benign insulinomas of the tail of the pancreas. Surgical treatment was performed with enucleation of the neoplasms.
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PMID:[Pancreatic insulinomas]. 817 52

The authors report their experience with octreotide in 20 patients (median age 57 years, 10 M, 10 F) from 1984 to 1991; 16 had metastatic APUDoma: 1 PPoma with VIPoma, 1 glucagonoma, 5 gastrinoma including 1 associated to PP-oma, 9 mid-gut carcinoid; 3 patients had multiple-endocrine neoplasia type I (MEN-I) with Zollinger-Ellison syndrome (ZES) and 1 patient a non-metastatic VIPoma. Octreotide (200-750 micrograms/day) was administered bid or tid with regular laboratory controls and morphological assessment. There was a striking improvement of symptoms, particularly in the carcinoid group (reduction of flushing in all patients and of diarrhoea in 3/5), in the patient with gastrinoma + acromegaly (regression of congestive heart failure) and in the patient with non-metastatic VIPoma. The hormonal markers were markedly reduced, particularly gastrin, PP (except in the patient with PPoma + VIPoma), VIP, GH and Somatomedin-C and urinary 5HIAA in 4/9 patients with carcinoid. There was only one partial regression of metastases (gastrinoma) and 4 apparent stabilizations of tumour growth, in the 16 metastatic cases. Among them, 4 patients died: 1 glucagonoma, 1 PPoma + VIPoma, 2 mid-gut carcinoids after a treatment of 5, 16, 30, 36 months, respectively. The patient with acromegaly + ZES died after 6 years of treatment at age 81. A patient with prolactinoma, resected insulinoma, hyperparathyroidism and ZES was not improved by a short course of octreotide (hypoglycemia); he died later of recurrent insulinoma. In conclusion, octreotide is a useful drug to control most of the symptoms related to gut endocrine tumours; it may inhibit tumour growth.
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PMID:Use of octreotide in the treatment of digestive neuroendocrine tumours. Seven year experience in 20 cases including 9 cases of metastatic midgut carcinoid and 5 cases of metastatic gastrinoma. 826 71

During the early 1980s liver transplantation (LTx) was expected to be a promising therapeutic option for patients with primary or secondary tumors confined to the liver. Because of disappointing results owing to death from early recurrence, LTx is currently restricted to well selected patients with small primary tumors and, in the case of liver metastases, to those with metastases of gastroenteropancreatic (GEP) tumor origin only. In our series of 300 liver transplantations four patients with GEP tumor metastases underwent LTx. The primary tumors were one neuroendocrine kidney tumor, one glucagonoma of the pancreas, and two cases of carcinoids of the pancreas. Because of local metastatic lymph node involvement upper gastrointestinal exenteration followed by LTx was performed in two patients. No patient survived beyond 33 months after LTx. Three patients died from tumor recurrence. In one patient who died from fungal sepsis autopsy revealed spine metastases that had been missed before LTx. Our dismal results do not compare well with promising data published previously by others for this particular patient group. Under the pressure of an increasing donor organ shortage, patients with GEP tumor metastases should be selected carefully for LTx.
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PMID:Results of liver transplantation for gastroenteropancreatic tumor metastases. 858 17

The glucagonoma syndrome is a rare disorder characterized by weight loss, necrolytic migratory erythema (NME), diabetes, stomatitis, and diarrhea. We identified 21 patients with the glucagonoma syndrome evaluated at the Mayo Clinic from 1975 to 1991. Although NME and diabetes help identify patients with glucagonomas, other manifestations of malignant disease often lead to the diagnosis. If the diagnosis is made after the tumor is metastatic, the potential for cure is limited. The most common presenting symptoms of the glucagonoma syndrome were weight loss (71%), NME (67%), diabetes mellitus (38%), cheilosis or stomatitis (29%), and diarrhea (29%). Although only 8 of the 21 patients had diabetes at presentation, diabetes eventually developed in 16 patients, 75% of whom required insulin therapy. Symptoms other than NME or diabetes mellitus led to the diagnosis of an islet cell tumor in 7 patients. The combination of NME and diabetes mellitus led to a more rapid diagnosis (7 months) than either symptom alone (4 years). Ten patients had diabetes mellitus before the onset of NME. No patients had NME clearly preceding diabetes mellitus. Increased levels of secondary hormones, such as gastrin (4 patients), vasoactive intestinal peptide (1 patient), serotonin (5 patients), insulin (6 patients, clinically significant in 1 only), human pancreatic polypeptide (2 patients), calcitonin (2 patients) and adrenocorticotropic hormone (2 patients), contributed to clinical symptoms leading to the diagnosis of an islet cell tumor before the onset of the full glucagonoma syndrome in 2 patients. All patients had metastatic disease at presentation. Surgical debulking, chemotherapy, somatostatin, and hepatic artery embolization offered palliation of NME, diabetes, weight loss, and diarrhea. Despite the malignant potential of the glucagonomas, only 9 of 21 patients had tumor-related deaths, occurring an average of 4.91 years after diagnosis. Twelve patients were still alive, with an average age follow-up of 3.67 years.
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PMID:The glucagonoma syndrome. Clinical and pathologic features in 21 patients. 860 27

The previously unreported ultrasonographic (US) features of liver metastases of pancreatic glucagonoma and of pancreatic acinar cell carcinoma are described. They present as complex masses with hyperechoic solid component, containing echo-free cystic areas; these sonographic features markedly differ from the echo-poor US pattern of the much more common metastases of pancreatic ductal carcinoma. Survival from diagnosis of liver metastases was 45 months in the patient with pancreatic glucagonoma and 23 months in the patient with acinar cell carcinoma. These survivals were much longer than the expected survival of patients with pancreatic ductal carcinoma metastatic to the liver. The US finding of highly reflective lesions in the liver, containing echo-free cystic areas, should alert one that the primary pancreatic tumor has a histotype different from ductal carcinoma. Such US findings could affect the decision to resect the pancreatic tumor and its liver metastases, if histology confirms a malignancy less aggressive than ductal carcinoma.
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PMID:Sonographic features of liver metastases from pancreatic glucagonoma and acinar cell carcinoma. Case reports. 866 36

Islet cell carcinoma (ICC) of the pancreas is a rare, indolent malignancy associated with higher resectability rate and better survival than ductal carcinoma. This retrospective study presents results of surgical treatment from a single institution. From 1985 through 1993 a total of 64 patients (36 men, 28 women) were surgically treated for ICC. Ages ranged from 22 to 80 years (median 55 years) with a median postoperative follow-up of 39 months (range 10-97 months). Of the 64 patients, 30 (47%) had functioning and 34 (53%) nonfunctioning tumors. Gastrinoma (n = 11) followed by glucagonoma (n = 6) and insulinoma (n = 4) were the most common functioning tumors. In the patients undergoing a laboratory study, 67% of the nonfunctioning tumors had elevated peptide hormone levels. Potentially curative resections were performed in 17 patients (26%), palliative procedures in 35 (55%), and exploratory laparotomy alone in 12 (19%). One patient (2%) died within 30 days after operation. Symptomatic improvement was achieved in 96% of patients with a mean duration of 22 months. Three- and five-year survivals were 66% and 49%, respectively. In patients with curative resection, the disease-free survival at 3 years was 53% (95% CI: 32-86%). The presence of diffuse hepatic metastases was a predictor of poor survival at 3 years (74% versus 58%;p = 0.05); there was no statistically significant difference in survival between functioning and nonfunctioning groups (p > 0.1). Although curative resection for ICC is rare, meaningful palliation can be achieved in most patients with rare mortality and acceptable morbidity.
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PMID:Islet cell carcinoma of the pancreas. 867 66

Islet cell tumors are rare pancreatic or peripancreatic neoplasms that produce and secrete hormones to a variable degree. These tumors are best divided on clinical grounds into those that produce a recognizable, clinically evident endocrine syndrome (ie, functioning) and those that exhibit no clinical evidence of hormone production (ie, clinically silent). Clinically silent tumors produce symptoms due to mass effect because of their large size. They are often partially cystic or necrotic. Functioning islet cell tumors usually manifest earlier in the course of the disease because of the distinctive signs and symptoms of the associated endocrine syndrome. Clinically silent and functioning tumors cannot be histologically distinguished reliably even with the use of immunohistochemical stains. Insulinoma and gastrinoma, the two most common functioning lesions, are typically small homogeneous masses. Other functioning islet cell tumors include glucagonoma, somatostatinoma, vipoma, and adrenocorticotropic hormone-producing tumor. Larger tumors are associated with calcification, cystic degeneration and necrosis, and a more aggressive behavior (local and vascular invasion as well as distant metastases). There are many different techniques for detection and characterization of these lesions that are usually chosen according to the radiologist's experience and preference. Treatment and prognosis of these lesions depend on the hormone produced, their size, and their behavior.
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PMID:Islet cell tumors of the pancreas: clinical, radiologic, and pathologic correlation in diagnosis and localization. 908 84

Although quite rare, the islet cell tumors present an important challenge to the clinician because of their protean manifestations and potential lethality. Early diagnosis is essential and depends on recognition of the classic and variant clinical syndromes followed by confirmation of elevated peptide levels by radioimmunoassay. Medical control of the hormonal syndrome with agents such as diazoxide for insulinoma, omeprazole for gastrinoma, and octreotide for vipoma and glucagonoma allows an orderly and thorough investigation for associated endocrinopathies and comorbid medical conditions. Localization and staging of the tumors are important because they may be small and occult, widely metastatic, or multifocal in the context of multiple endocrine neoplasia type I (MEN I) syndrome. Computed tomography, visceral angiography, endoscopic ultrasonography, and indium-labeled octreotide scanning are the most useful preoperative imaging techniques. Surgical exploration that includes intraoperative ultrasonography remains an essential localization technique for occult tumors, particularly insulinomas and gastrinomas. For all patients other than some with advanced metastatic disease or MEN I syndrome, an aggressive surgical approach with the intent of complete and curative tumor excision is indicated. Surgical cure is possible in most insulinomas, a substantial proportion of gastrinomas, and some patients with the other more rare and malignant islet cell tumors. At present, adjuvant medical therapies for unresectable malignant disease have limited efficacy. However, a variety of newer and innovative tumor localization techniques, operative strategies, and nonoperative treatment modalities hold considerable promise for the attainment of higher cure rates and improved palliation.
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PMID:Islet cell tumors. 929 76

The authors describe their experience with liver transplantation (OLT) for metastatic endocrine tumors (MET) in order to determine reasonable indications for OLT in patients with this disease. Removal of the primary lesion and subsequent liver transplantation were performed in two separate procedures in all patients except one. Only those patients suffering from objective tumor progression and symptoms with no evidence of extrahepatic spread after complete work-up (including endoscopic ultrasonography (US) and 123I-labeled Tyr3-octreotide body scanning) underwent liver transplantation. Fifteen patients were referred for liver transplantation. Seven patients were excluded either because of stability of liver metastases (n = 3), extrahepatic spread, general contraindication (n = 2), or feasibility of aggressive surgical resection (n = 2). Liver transplantation was undertaken in eight patients with carcinoid tumor (n = 4), gastrinoma (n = 3) and glucagonoma (n = 1). Three patients did not survive the surgical procedure itself, whereas two additional patients died from chronic rejection or from recurrent disease. Three patients who received transplants for metastatic carcinoid tumor are alive without biochemical or imaging evidence of disease recurrence at 6, 15, and 52 months. The best indication for transplantation seems to be patients with metastases restricted to the liver and unresponsive to adjuvant therapy after aggressive surgical resection including excision of the primary lesion and reduction of hepatic metastases. In such highly-selected patients, liver transplantation remains a high-risk operation, but it can yield long-term survival.
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PMID:Metastatic endocrine tumors: is there a place for liver transplantation? 934 51


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