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

The history, clinical signs and radiographic and ultrasonographic findings in 16 dogs with pancreatic neoplasia were reviewed retrospectively. Thirteen of the dogs had islet cell carcinoma compatible with insulinoma, one had a pancreatic adenocarcinoma and two had secondary invasion of the pancreas, one by a gastric carcinoma and one by an intestinal lymphoma. The clinical signs in the 13 dogs with insulinoma included collapse in 10 dogs, ataxia in seven, weakness in five, and seizures in two. Two of the 16 dogs had jaundice due to biliary obstruction by the primary tumour or metastases. The sensitivities for pancreatic neoplasia were three of 16 (19 per cent) for radiography and 12 of 16 (75 per cent) for ultrasonography; the sensitivities for metastasis were two of 11 (18 per cent) for radiography and six of 11 (55 per cent) for ultrasonography. Biliary obstruction was detected by ultrasonography in both affected dogs.
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PMID:Ultrasonography of pancreatic neoplasia in the dog: a retrospective review of 16 cases. 853 34

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

Gastrointestinal endocrine tumors consist of pancreatic endocrine neoplasms and carcinoid tumors. Except for insulinoma, a majority of gastrointestinal endocrine tumors are malignant. With improved medical treatment of syndromes of hormonal excess, growth of the primary tumor and metastatic spread has increasingly become an important determinant of long-term survival. Although few randomized, prospective data are available given the rare occurrence of malignant gastrointestinal endocrine tumors, surgery appears to be the only potentially curative treatment for malignant endocrine tumors, and complete resection of localized or regional nodal metastases provides the highest probability of cure. Surgery may also be the most effective treatment for hepatic metastases if most or all of the tumor can be resected, and patients with solitary, localized metastatic disease appear to benefit most. Symptoms from extensive metastases may respond to chemotherapy or octreotide. Gastrointestinal endocrine tumors are generally indolent, slow-growing neoplasms, and when symptoms are adequately controlled, patients can live comfortably and productively for many years with metastatic disease.
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PMID:Gastrointestinal endocrine cancers and nodal metastasis: biologic significance and therapeutic implications. 878

Because SRS identifies 90% of hepatic metastatic disease and the addition of other studies (ultrasonography, C.T. MRI, and selective mesenteric angiography) identities only 4% more, the identification of a primary lesion with SRS obviates for the most part the use of further investigations. If SRS is negative, additional studies should only be undertaken if surgery is contemplated. Because SRS may only localize 60%-70% of primary gut NETs, an additional 10%-15% may be identified by undertaking additional studies. The most sensitive test, STIR-MRI, should be undertaken next, but because it is not widely available, pancreatic protocol CT scan is almost as effective in identification of a primary lesion. If a primary gastrinoma cannot be identified by SRS or STIR-MRI, endoscopic ultrasonography should be undertaken because duodenal gastrinomas are often minute and multicentric. A similar strategy applies for insulinomas because up to 40% cannot be located by SRS and the majority are located in the pancreatic head. Thus, STIR-MRI followed by endoscopic ultrasonography is the most appropriate course. Although calcium provocation-angiography is highly effective in the identification of insulinomas, it is significantly more invasive and should be used only as a last resort. Of particular interest is the observation that in the study of gastrinomas, SRS altered clinical management in almost 50% of patients. This reflected the ability of SRS not only to identify the primary tumor location but clarify equivocal localization results generated by conventional imaging studies. It thus seems that the simplicity, superior sensitivity, high specificity, and cost-effectiveness of SRS mandate that it be the imaging modality in patients with gastrinomas. Because the cost of an SRS is $1800 and may obviate the need for multiple other topographic studies that are at least as expensive, the fiscal dictates further warrant the use of this study as the initial topographic investigation. These observations are probably applicable to all gut NETs, although the likelihood of primary identification in the instance of insulinoma patients may be somewhat less. The timely and cost-effective establishment of the type of NET, its primary site, and the detection metastatic spread will enable determination of the appropriate management strategy.
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PMID:Approaches to the diagnosis of gut neuroendocrine tumors: the last word (today). 902 13

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

A survey of UK patients receiving the drug diazoxide, revealed 40 patients with insulinoma on this treatment. Mean age (+/- SD) was 67 +/- 18 years, and 74% were female. Duration of treatment was 7 +/- 6 years (range 1-22). Most (55%) patients were treated with diazoxide because of tumour non-localisation (including failed previous surgery). Metastatic disease (20%) and poor surgical risk (10%) were other indications. Side-effects (notably fluid retention and hirsutism) were common (47%) but not troublesome. Treatment was highly effective--59% were symptom free and 38% had only occasional symptoms. Only one patient had frequent hypoglycaemia despite treatment. We conclude that diazoxide is effective in the management of insulinoma. Side-effects are common but not problematic. Treatment should be considered for all patients not cured by surgery, or unsuitable for surgical treatment.
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PMID:Diazoxide treatment for insulinoma: a national UK survey. 949 74


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