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

Eighty-five patients with endocrine pancreatic tumors associated with clinical syndromes of hormone excess were retrospectively analyzed regarding symptomatology, means of diagnosis, and results of surgical and medical treatment during follow-up of 3-18 years (median 8 years). The combination of angiography and computed tomography was most successful in pre-operative localization of both primary tumors and metastases. Surgery provided long term cure in 39 of 44 patients with benign islet cell lesions, the majority having insulinomas. Forty-one patients had malignant tumors, which at the time of diagnosis or operation were associated with liver and/or regional lymph gland metastases in 56% and 24%, respectively. Sixteen patients with metastatic disease and/or very large tumors were considered inoperable, 5 patients underwent palliative resection of their malignant tumors, while grossly radical tumor removal was accomplished in 20 patients. Long-term cure was achieved in 5 patients by excision of primary tumors and localized liver or lymph gland metastases. Half of the patients, particularly those with insulinoma, gastrinoma, or vipoma, showed response to streptozotocin, in combination with other cytostatics, for a median of 24 months or a response to interferon for a median of 10 months. The overall 5-year and 10-year survival among the patients with malignant islet cells tumors was 54% and 28%, respectively. Absence of liver metastases at time of operation/diagnosis, smaller size of the primary tumor, grossly radical tumor resection as well as response to medical therapy predicted the more favorable survival.
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PMID:Clinical characteristics, treatment and survival in patients with pancreatic tumors causing hormonal syndromes. 135 29

The effects of octreotide in vivo and in vitro on hormone release, in vivo [123I]Tyr3-octreotide scanning, and in vitro [125I]Tyr3-octreotide autoradiography were compared in five patients with endocrine pancreatic tumors. [123I]Tyr3-octreotide scanning localized the primary tumor and/or previously unknown metastases in four of the five patients. The patient with a negative scan had an insulinoma that did not respond to octreotide in vivo. No Tyr3-octreotide-binding sites were subsequently found at autoradiography of the tumor, whereas somatostatin-14 receptors were present at a high density. In parallel, culture studies with the cells prepared from this adenoma showed that insulin release was not affected by octreotide, while both somatostatin-14 and -28 significantly suppressed hormone release. Culture studies of the tumor cells from two gastrinomas showed a dose-dependent inhibition of gastrin release by octreotide. Octreotide exerted direct antiproliferative effects in one of these gastrinomas, which had been shown to be rapidly growing in vivo. Both gastrinomas had specific somatostatin receptors, as measured by in vitro receptor autoradiography. Somatostatin release by the cultured somatostatinoma cells from one of these patients was suppressed by octreotide. In conclusion, 1) the [123I]Tyr3-octreotide scanning procedure is valuable in the localization of primary endocrine pancreatic tumors as well their often clinically not yet recognized metastases; 2) the in vitro detection of somatostatin receptors in those tumors that were also visualized in vivo after injection of [123I] Tyr3-octreotide indicates that the ligand binding to the tumor in vivo indeed represents binding to specific somatostatin receptors; and 3) the parallel between the presence of somatostatin receptors on tumors and in in vivo and in vitro effects of octreotide on hormonal release from these tumors indicate that a positive scan predicts a good suppressive effect of octreotide on hormonal hypersecretion by these tumors.
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PMID:Parallel in vivo and in vitro detection of functional somatostatin receptors in human endocrine pancreatic tumors: consequences with regard to diagnosis, localization, and therapy. 216 29

We report herein a rare case of malignant insulinoma which recurred as multiple liver metastasis 8 years after the initial resection. The patient was a 51-year-old Japanese man who originally presented in 1985 at the age of 43 years suffering from general malaise and syncope. The initial surgery in 1985 involved complete enucleation of a 15 x 13 mm insulinoma located in the uncus of the pancreas. Histopathologically, the tumor was diagnosed as a benign adenoma (insulinoma) which was immunohistochemically stained with only the anti-insulin monoclonal antibody. Macroscopically, there were no signs of either invasion or metastasis. During the subsequent 7 years, he did not show any symptoms or significant abnormality in laboratory data. However, in 1993, the patient again experienced syncope with hypoglycemia and hyperinsulinemia. Ultrasonography revealed multiple echogenic lesions in the liver and a second laparotomy confirmed multiple hepatic metastases from insulinoma, the histopathological findings of which were similar to those of the primary tumor from 8 years before. The patient is currently being treated with streptozotocin and 5-fluorouracil via a catheter in the hepatic artery.
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PMID:Malignant insulinoma causing liver metastasis 8 years after the initial surgery: report of a case. 754 77

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

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

We describe an insulinoma of the pancreas in a 56-year-old patient, which showed insular-ductular differentiation in its liver metastasis. Although the primary tumor was uniformly endocrine in nature with insulin production, the metastasis contained two distinct cell types in organoid arrangement. One cell type was insulin-positive and was arranged in islet-like structures; the other was insulin-negative but distinctly pan-cytokeratin and cytokeratin 7 positive and arranged in ducts. In the primary tumor and the metastasis, the tumor cells were surrounded by a desmoplastic stroma. As to the histogenesis of the tumor and its metastasis, we discuss the following possibilities: (1) the tumor cells might derive from a common stem cell that matures into two phenotypically different cell lines, resembling the situation in embryogenesis and (2) one tumor cell type originates from the other by transdifferentiation (metaplasia). We conclude that the parallel occurrence of endocrine and ductal differentiation supports the concept that, under certain conditions, islet cells and ductular cells may also originate from islets and that mixed endocrine/exocrine pancreatic tumors do not necessarily arise from totipotent duct cells but might also have a primary endocrine cell origin.
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PMID:Insulinoma of the pancreas with insular-ductular differentiation in its liver metastasis--indication of a common stem-cell origin of the exocrine and endocrine components. 1146 96

Hepatoid carcinomas are uncommon extra-hepatic neoplasms exhibiting features of hepatocellular carcinoma and they are most frequently described in the stomach. We report a 64-year-old woman with a malignant insulinoma showing focal hepatoid differentiation and biochemical evidence of alpha-fetoprotein (AFP) production. The current case is the first malignant insulinoma with hepatoid differentiation. Resection of the primary tumor followed by regional embolization was peformed. The patient died 22 months after initial presentation. Thus, the presence of hepatoid differentiation in pancreatic tumor should be noted as the tumor may be associated with elevated AFP. The features of pancreatic hepatoid carcinomas are discussed.
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PMID:Malignant insulinoma with hepatoid differentiation: a unique case with alpha-fetoprotein production. 1174 56

Accurate preoperative detection, localization, and staging of the primary tumor and metastases are essential for the selection of appropriate candidates for surgery. In dogs with insulinoma, preoperative assessment usually is performed with transabdominal ultrasonography (US). There are no reports on the use of computed tomography (CT) for this purpose. The preoperative use of somatostatin receptor scintigraphy (SRS) recently has been advocated for the identification of insulinoma and gastrinoma in dogs, but its accuracy remains to be established. In this report US, CT, and single-photon emission computed tomography (SPECT) with [111In-DTPA-D-Phe1]-octreotide (a specific form of SRS) were compared for their effectiveness in detecting and localizing primary and metastatic insulinoma in dogs. Findings at surgery or postmortem examination served as control. Of 14 primary insulinomas, 5, 10, and 6 were correctly identified by US, CT, and SPECT, respectively. No lymph node metastases were detected by US or SPECT. CT identified 2 of 5 lymph node metastases but also identified 28 false-positive lesions. Two of 4 livers were found to be positive for metastases by 1 of the imaging techniques. US can be used for the initial evaluation of dogs with hypoglycemia. Although CT identifies most primary tumors, intraoperative inspection and palpation of the pancreas is still superior. SPECT appears as effective as US and CT in detecting insulinomas. Future developments in preoperative imaging techniques might improve current methods of canine insulinoma detection.
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PMID:Comparison of ultrasonography, computed tomography, and single-photon emission computed tomography for the detection and localization of canine insulinoma. 1571 42

Neuroendocrine tumors (NETs) can be small and situated almost anywhere throughout the body. Our objective was to investigate whether whole-body (WB) positron emission tomography (PET) with (11)C-5-hydroxytryptophan (5-HTP) can be used as a universal imaging technique for NETs and to compare this technique with established imaging methods. Forty-two consecutive patients with evidence of NET and a detected lesion on any conventional imaging (six bronchial, two foregut, 16 midgut, and two thymic carcinoids; one ectopic Cushing's syndrome; four gastrinomas; one insulinoma; six nonfunctioning endocrine pancreatic tumors; one gastric carcinoid, one paraganglioma; and two endocrine-differentiated pancreatic carcinomas) were studied. The WB-(11)C-5-HTP-PET examinations were compared with WB-computed tomography (CT) and somatostatin receptor scintigraphy (SRS). Tumor lesions were imaged with PET in 95% of the patients. In 58% of the patients, PET could detect more lesions than SRS and CT and equal numbers in 34%, whereas in three cases, SRS or CT showed more lesions. In 84% (16 of 19 patients), PET could visualize the primary tumor compared with 47 and 42% for SRS and CT, respectively. The surgically removed PET-positive primary tumor sizes were 6-30 mm. To conclude, this study indicates that WB-(11)C-5-HTP-PET can be used as a universal imaging method for detection of NETs. This study also shows that WB-(11)C-HTP-PET is sensitive in imaging small NET lesions, such as primary tumors, and can in a majority of cases image significantly more tumor lesions than SRS and CT.
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PMID:Whole-body (11)C-5-hydroxytryptophan positron emission tomography as a universal imaging technique for neuroendocrine tumors: comparison with somatostatin receptor scintigraphy and computed tomography. 1575 58


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