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)

Computed tomography (CT) and 123I- or 131I-meta-iodo-benzyl-guanidine (MIBG) scintigraphy were compared for accuracy in tumor detection in 47 patients with neuroectodermal neoplasms. MIBG concentration was found in 12 of 13 pheochromocytomas, 12 of 12 neuroblastomas, 5 of 9 carcinoids, and 1 of 4 glomus tumors. MIBG uptake was not observed in medullary thyroid carcinomas, oat-cell carcinomas, Merkel tumors, 1 gastrinoma, and 2 unclassified neuroectodermal neoplasms. With regard to the different tumor manifestations, the sensitivity in detecting pheochromocytomas, neuroblastomas, and carcinoids was 87%, 77%, and 100% for CT, and 83%, 100% and 71% for MIBG scintigraphy, MIBG scintiscan was superior in the detection of small adrenal pheochromocytomas (less than 1 cm diameter) and in the depiction of small bone metastases and bone marrow infiltration from neuroblastoma. In all, 25 cycles of high-dose MIBG therapy were performed in eight patients with surgically incurable tumors (4 malignant pheochromocytomas, 1 neuroblastoma, 3 carcinoids). The total therapeutic activity applied was 3.55-43.29 GBq 131I-MIBG. Tumor kinetics of MIBG were investigated before and during treatment. One patient with metastatic pheochromocytoma has been in complete remission for a follow-up period of 36 months since completion of treatment, and another patient is in partial remission. Tumor reduction or no change was observed in four patients. Two patients died of non-concentrating recurrence and metastases.
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PMID:[Diagnosis and therapy of neuroectodermal tumors]. 253 1

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

The clinical presentation of gastrinoma has changed since the original description by Zollinger and Ellison in 1955. Tumors currently found are smaller, extrapancreatic or extraintestinal in location, and frequently occult within lymph nodes. The incidence of hepatic metastases on initial presentation has decreased. In addition, the clinical course of patients with tumor in lymph nodes is benign, suggesting that more patients than were previously thought are now candidates for cure. Improved knowledge of the anatomic location of gastrinomas has enhanced our ability to find and remove them at laparotomy. As a result of these factors, more patients are being cured than ever before, and in the future, cure rate may be even higher. On the basis of these recent advances, the optimal treatment of gastrinoma is surgical excision for cure.
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PMID:Gastrinoma. New medical and surgical approaches. 256 61

Recently a number of surgeons have recommended radical resection of gastrinomas in Zollinger-Ellison syndrome (ZES). We have developed a useful technique for preoperative localization of gastrinomas--the selective arterial secretin injection test (SASI)--and we recommend an intraoperative secretin test (IOS) for deciding the radicality of resection of gastrinomas. Here the results of SASI and IOS tests in 11 patients with ZES are examined and compared with the results of other techniques. The SASI test localized gastrinomas in all of the patients, while the sensitivity of ultrasonography, computed tomography, arteriography, or portal venous blood samplings was between 1/11 and 5/11. On the basis of the results of the SASI test, radical resection of gastrinoma was performed in four patients (three pancreatoduodenectomies and one extirpation). After pancreatoduodenectomy, immunohistologic study of the specimen revealed multiple microgastrinomas and lymph node metastases in two patients and the coexistence of a microgastrinoma and a gastinoma in one patient. The IOS test was useful in the estimation of the advisability of radicality, and in two patients total gastrectomy was not performed because of the results of the IOS test. These four patients are well and have returned to work, and their serum gastrin levels are below 35 pg/mL. Thus we believe SASI and IOS tests are helpful for planning curative resection of gastrinomas.
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PMID:Curative resection of multiple gastrinomas aided by selective arterial secretin injection test and intraoperative secretin test. 258 84

In the management of gastrinoma, a variety of preoperative tests for tumor localization are recommended. Their value in predicting surgical outcome is not well defined. We reviewed the preoperative investigation and operative outcome in 23 patients to assess the predictive value of localization before surgery. With results of laparotomy used as reference, the efficacy of computed tomography, angiography, ultrasonography, and magnetic resonance imaging, individually and combined for primary and metastatic disease, was determined. All investigative modalities were poor for the detection of primary gastrinomas, with individual sensitivities in the range of 20% to 29%. Their combined sensitivity was 53%, with a negative predictive value of only 38%. The detection of metastatic disease was better, with a combined accuracy of 83%. At laparotomy, tumor was identified in 19 of 23 (83%) patients. In 14 patients with negative preoperative workup, primary gastrinoma was found in 10 of 14 (71%) and resected for long-term cure in three of 14 (21%) patients. In all nine patients with positive preoperative test results, tumor was found and was resected for cure in four (44%) patients. In conclusion, preoperative localization of primary gastrinoma is unreliable, and a negative workup does not preclude cure. Therefore, despite the availability of effective palliative antiulcer therapy, all patients exclusive of those with multiple endocrine neoplasia, type I, or metastasis should undergo laparatomy with curative resection in mind.
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PMID:Gastrinoma: the predictive value of preoperative localization. 268 59

The treatment of patients with Zollinger-Ellison syndrome (ZES) has undergone dramatic evolution during the past decade. Although initially regarded as an incurable tumor, resection of gastrinoma for potential cure has been reported in 30% to 40% of selected patients in recent series. Conversely, although definitive control of acid hypersecretion is achieved by total gastrectomy, histamine (H2)-receptor antagonists and the newly introduced agents omeprazole and somatostatin analogues allow effective medical therapy of gastric acid overproduction. Confirmation of the diagnosis is best achieved with the I.V. secretin stimulation test, and tumor localization techniques are mandatory to identify candidates for operative tumor resection. Intraoperative sonography and careful exploration are required for tumor removal; successful tumor resection is associated with prolonged survival. The majority of patients (60%) are still found to have malignant disease at the time of diagnosis, but 10-year overall survival commonly exceeds 40%. The presence of multiple endocrine neoplasia type I (MEN-I) is seen in 10% to 25% of patients; correction of hypercalcemia alone may have therapeutic benefit in some ZES patients, and while gastrinoma resection is rarely possible, MEN-I patients demonstrate prolonged survival. The choice of medical rather than surgical therapy for acid hypersecretion depends on the suitability of each patient for careful and repeated endoscopic and chemical studies, versus the likelihood of a successful postoperative outcome. Socioeconomic, geographic, and related medical factors in each case may dictate the form of long-term antisecretory therapy. Exploration for possible tumor resection is indicated for virtually all patients who have no documented metastatic disease.
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PMID:Current diagnosis and management of Zollinger-Ellison syndrome. 268 66

Fourteen hormone-producing gastrointestinal tract tumors were tested for their content of somatostatin (SRIH) receptors, using receptor autoradiography and in vitro binding assay with tumor homogenates. All four gastrinomas tested had high levels of SRIH receptors, as did two of five insulinomas and four of five vasoactive intestinal peptide-producing tumors. Receptor visualization was obtained with two different radioligands, either a SRIH-28 analog, [125I]-[Leu8,D-Trp22,Tyr25]SRIH-28, or a SRIH octapeptide, the [125I]Tyr3 derivative of SMS 201-995 [H-DPhe-Cys-Phe-DTrp-Lys-Thr-Cys-Thr(ol)], [125I]204-090. In both cases receptors were localized over the tumor cell area only. Biochemical and pharmacological analyses of one insulinoma and two vipomas revealed saturable, high affinity binding sites with pharmacological specificity for SRIH. However, differences in receptor affinity of selected SRIH analogs, in particular SRIH-28 and SRIH octapeptides, were found between the insulinomas and the two other tumor types, vipoma and gastrinoma. The presence of SRIH receptors on various hormone-producing gastrointestinal tumors suggests that at least part of the beneficial effects of chronic therapy with SRIH analogs may be mediated through such membrane-bound receptors located on the tumor itself. SRIH receptor measurement may be of prognostic value in assessment of the therapeutic efficacy of SRIH analogs. They may also be of diagnostic value, if used as in vivo markers for the localization of small hormone-producing gastrointestinal tumors or their metastases.
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PMID:Hormone-producing gastrointestinal tumors contain a high density of somatostatin receptors. 282 49

The gastrinoma remains the only endocrine-producing pancreatic tumor for which early surgical intervention is not uniformly advised. It should be possible after 30 years to determine the factors that appear to influence survival. Forty patients who underwent surgery 10 years or more ago were reviewed in an effort to identify factors at operation and the type of surgical procedure that influence survival. Because the total number of patients is small, conclusions based on statistics are not justified; however, certain trends have become clear. Favorable factors at surgery were the presence of a verified submucosal duodenal tumor, the presence of multiple endocrine neoplasia, or the failure to find tumor. Unfavorable factors were the presence of liver and/or lymph node metastases and/or a verified sporadic, nonfamilial pancreatic tumor. Thirty-four of the 40 patients underwent total gastrectomy, and 18 of these patients also underwent hemipancreatectomy, while six underwent other procedures less than total gastrectomy. The 10-year survival rate was 50%. The long-term quality of life in surviving patients who underwent total gastrectomy supports the original recommendation of total gastrectomy.
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PMID:Gastrinoma: factors influencing prognosis. 285 58

The advent of the histamine H2-receptor antagonists and the renewed interest in curative surgery in patients with gastrinoma have made the differentiation between benign and malignant tumors of critical importance. An analysis of 65 patients with gastrinoma followed for an average of 93 months revealed two distinct clinical groups: those with and those without hepatic tumors at initial examination or operation. Among the 14 patients with hepatic tumors, 12 had multiple liver metastases from pancreatic or duodenal primary tumors, and 2 had primary hepatic gastrinomas. Ten of the 14 patients (71 percent) died from tumor progression, and the total tumor-related mortality for this group was 79 percent. In contrast, only 1 of 15 patients (7 percent) with tumor in the lymph nodes died from a tumor-related cause (recurrent ulcer hemorrhage), and none died from tumor progression. Only a single patient with lymph node metastases at initial exploration went on to the development of liver metastases, which was found incidentally at autopsy 313 months later. Among 23 patients with either primary tumors only or no tumors found at laparotomy, there was only one tumor-related death and no deaths from tumor spread. Life-table analysis demonstrated a significantly decreased length of survival for patients with liver tumor compared with those without liver involvement. Multiple endocrine adenopathy syndrome was not a significant factor in survival. Serum gastrin levels were likewise nondiscriminatory. Six of 52 patients (12 percent), including three with tumor in the lymph nodes, were apparently cured by excision of all gastrinoma recognized at laparotomy. The cure rate was 23 percent for patients without multiple endocrine adenopathy syndrome or liver metastases. Hepatic metastases is a definitive marker for clinically malignant disease and portends a poor prognosis. Patients with gastrinoma confined to the lymph nodes uncommonly follow a malignant clinical course. Such patients have at least a 20 percent probability of surgical cure if they do not have multiple endocrine adenopathy syndrome.
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PMID:Benign and malignant gastrinoma. 285 72

Forty-six patients with the gastrinoma syndrome were divided into 2 categories: 1) benign sporadic gastrinoma (n = 30), and 2) gastrinoma with metastases to liver (n = 16). Thirteen of the 46 patients had multiple endocrine neoplasia type I syndrome. Serum gastrin levels in patients fasted overnight were determined by RIA using antisera directed toward the NH2- and COOH-terminals of heptadecapeptide gastrin (G17) and the NH2-terminus of the triacontatetrapeptide (G34). These results were compared with findings in 50 normal subjects. In the normal subjects, the mean COOH-terminal gastrin-17 level was higher [65 +/- 8 (+/- SEM) pg/ml] than the NH2-terminal gastrin-17 level (11 +/- 0.2 pg/ml) and lower than the NH2-terminal gastrin-34 level (134 +/- 20 pg/ml). The levels of NH2-terminal gastrin-17 were higher in patients with metastatic disease than in those with benign gastrinoma, whereas the COOH-terminal gastrin-17 and the NH2-terminal gastrin-34 levels were similarly high in both groups. The mean ratio of NH2-terminal gastrin-17 to COOH-terminal gastrin-17 was less than 1 in normal subjects (0.22 +/- 0.02) and benign gastrinoma patients (0.2 +/- 0.04), and it was 2.2 +/- 0.41 in the patients with metastatic gastrinoma. An NH2 to COOH gastrin-17 ratio greater than 1 was found in 13 of 16 patients with metastatic gastrinoma, but in none of the patients with benign gastrinoma or normal subjects. Similar results were found in multiple endocrine neoplasia type I patients with benign and metastatic disease. A high NH2 to COOH gastrin-17 ratio is suggestive of metastatic gastrinoma. In 4 patients with metastatic gastrinoma, the NH2 to COOH gastrin-17 ratio fell in parallel with the response to chemotherapy.
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PMID:Evaluation of NH2-terminus gastrins in gastrinoma syndrome. 287 76


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