(dtpa-phe(1))-octreotide and Multiple-Endocrine-Neoplasia-Type-1

Neuroendocrine tumors (NETs) are a potentially lethal component of multiple endocrine neoplasia type 1 (MEN 1). Somatostatin receptor scintigraphy (SRS) can be used to localize NETs and evaluate patients for extraduodenopancreatic disease; its utility in managing MEN 1 is undefined.. All patients with MEN 1 evaluated by SRS from April 1994 to November 1997 are reported. SRS findings were correlated with other imaging studies and operative findings.. Thirty-seven SRS studies were performed in 29 patients with MEN 1. SRS identified occult tumor in 36% (4/11) of patients with only biochemical evidence of NET; 2 patients went on to resection. SRS showed tumor in 79% (15/19) of patients with computed tomography (CT)-demonstrated tumor; 30% (6/20) of the SRS lesions were occult on CT. Conversely, 55% (16/29) of CT-identified lesions were occult on SRS. SRS found distant disease in 21% (6/29) of patients. In patients who had previous operations, SRS found tumor in 40% (4/10) of patients, again with both new positive and false-negative results compared with other imaging. SRS also had 3 important false-positive results, including 1 patient who had laparotomy with no tumor identified.. SRS is useful in identifying otherwise occult NETs in patients with MEN 1 and can substantially alter management. However, SRS also has significant false-positive and false-negative results that demand correlation with other studies.

Topics: Adult; Aged; Female; Humans; Male; Middle Aged; Multiple Endocrine Neoplasia Type 1; Octreotide; Pentetic Acid; Prospective Studies; Radionuclide Imaging; Radiopharmaceuticals; Receptors, Somatostatin

Gastric carcinoids are of increasing clinical concern because they may develop in hypergastrinemic states, especially with the increased chronic use of potent acid suppressants that can cause hypergastrinemia. However, gastric carcinoids are difficult to diagnose. Somatostatin receptor scintigraphy (SRS) has a high sensitivity and specificity for localizing carcinoids in other locations. The purpose of this study was to determine whether SRS could localize gastric carcinoids.. Two groups of patients with Zollinger-Ellison syndrome (ZES) with hypergastrinemia, each having a different increased risk of developing gastric carcinoids, were studied. One hundred sixty-two consecutive patients with ZES were studied prospectively, with 39 having multiple endocrine neoplasia, type 1 (MEN-1) (high increased risk), and 123 not having MEN-1 (low increased risk). Patients were admitted to the hospital initially and then yearly, undergoing SRS with SPECT, upper gastrointestinal endoscopy, and Jumbo Cup biopsies of any gastric abnormalities, as well as random biopsies of the gastric body. Tumor localization studies were also performed. Both the results of the routine SRS interpretation and the results of a masked review, with particular attention to the stomach of high risk MEN-1 patients, were correlated with the gastric biopsy results.. Gastric SRS localization was positive in 19 (12%) of 162 patients. Sixteen patients had a gastric carcinoid, and 12 of these patients had SRS localization. The sensitivity of SRS in localizing a gastric carcinoid was 75%, with a specificity of 95%. Positive and negative predictive values were 63% and 97%, respectively.. SRS is a noninvasive method that can identify patients with gastric carcinoids with a reasonable sensitivity and a high specificity. SRS should prove useful in the treatment of patients with hypergastrinemic states that have an increased incidence of gastric carcinoids. In patients with MEN-1, one must realize that localization in the upper abdomen on SRS may be caused by a gastric carcinoid and not a pancreatic endocrine tumor.

Topics: Carcinoid Tumor; Case-Control Studies; Female; Humans; Indium Radioisotopes; Male; Middle Aged; Multiple Endocrine Neoplasia Type 1; Octreotide; Pentetic Acid; Predictive Value of Tests; Prospective Studies; Radiopharmaceuticals; Receptors, Somatostatin; Sensitivity and Specificity; Stomach Neoplasms; Tomography, Emission-Computed, Single-Photon; Zollinger-Ellison Syndrome

Topics: Adult; Carcinoid Tumor; Humans; Indium Radioisotopes; Male; Multiple Endocrine Neoplasia Type 1; Octreotide; Pentetic Acid; Radionuclide Imaging; Thymus Neoplasms

To determine the value of somatostatin-receptor scintigraphy in the localization of various endocrine gastrointestinal tumors, we compared the results obtained with this new technique with the results obtained with computed tomography and sonography. We could not find an overall advantage of somatostatin-receptor scintigraphy as compared to computed tomography or sonography in the localization of intestinal carcinoids (n = 13), gastrinomas (n = 12), functionally non-active endocrine pancreatic tumors (n = 8) and various other endocrine pancreatic tumors (n = 4). In 2 patients with endocrine pancreatic tumors however, the tumors were localized preoperatively only by somatostatin-receptor scintigraphy. Somatostatin-receptor scintigraphy may occasionally be helpful in the localization of gastrointestinal endocrine tumors if these tumors are not localized by conventional imaging studies. Somatostatin-receptor scintigraphy does not solve the problem to localize small endocrine tumors.

Topics: Biomarkers, Tumor; Carcinoid Tumor; Gastrinoma; Gastrointestinal Neoplasms; Humans; Indium Radioisotopes; Insulinoma; Multiple Endocrine Neoplasia Type 1; Octreotide; Pancreatic Neoplasms; Paraneoplastic Endocrine Syndromes; Pentetic Acid; Receptors, Somatostatin; Tomography, Emission-Computed, Single-Photon; Zollinger-Ellison Syndrome

In patients with the Zollinger-Ellison syndrome, which is either sporadic or integrated into multiple endocrine neoplasia type 1, accurate localization of all the tumours is difficult and may have therapeutic implications. In an attempt to improve this localization, somatostatin receptor scintigraphy using [111In-DTPA-D-Phe1]-octreotide was performed prospectively in 48 consecutive patients with the Zollinger-Ellison syndrome. Thirty of them had the sporadic type of this disease. Scintigraphic data were compared with data obtained by conventional imaging methods, and also, in 32 selected patients, with those obtained by endoscopic ultrasonography. Somatostatin receptor scintigraphy showed abnormal tracer uptake in 39 patients (81%), in whom it correctly identified 50 of the 60 tumoral sites (83%) previously localized by the other imaging methods. In 17 patients (35%) somatostatin receptor scintigraphy disclosed abnormal tracer uptake at 18 different tumoral sites: 14 were located in the abdomen, including four in the liver and eight in the duodenopancreatic area, and four outside the abdomen, including two in the mediastinum. Six of the ten tumoral sites which were not correctly identified by somatostatin receptor scintigraphy were located in the duodenopancreatic area. However, in the 20 patients for whom conventional techniques failed to visualize any tumour in the duodenopancreatic area, somatostatin receptor scintigraphy was positive in ten (50%) whereas endoscopic ultrasonography was only positive in five (25%). In our patients with the Zollinger-Ellison syndrome, somatostatin receptor scintigraphy appeared to be a useful new addition to the battery of tests used for tumour detection.

Topics: Bone Neoplasms; Duodenal Neoplasms; Female; Humans; Indium Radioisotopes; Liver Neoplasms; Male; Mediastinal Neoplasms; Middle Aged; Multiple Endocrine Neoplasia Type 1; Octreotide; Pancreatic Neoplasms; Pentetic Acid; Prospective Studies; Radionuclide Imaging; Receptors, Somatostatin; Ultrasonography; Zollinger-Ellison Syndrome