Pancreatic Somatostatinoma Research Articles

Pancreatic somatostatinoma diagnosed preoperatively: report of a case.

Somatostatinoma is a rare neoplasm of the pancreas. Preoperative diagnosis is often difficult. We report a 72-year-old woman with a pancreatic head tumor measuring 37 mm in diameter, and enlargement of the lymph nodes on the anterior surface of the pancreatic head and the posterior surface of the horizontal part of the duodenum. Laboratory data showed an elevated plasma somatostatin concentration. Examination of a biopsy specimen of the pancreatic head mass obtained by endoscopic ultrasound-guided fine needle aspiration (EUS-FNA) showed histopathological features of a neuroendocrine tumor. Immunohistochemical staining showed that the tumor cells were positive for somatostatin, leading to a preoperative diagnosis of pancreatic somatostatinoma. The patient underwent pylorus-preserving pancreaticoduodenectomy. The plasma somatostatin concentration decreased progressively after surgery. A rare case of pancreatic somatostatinoma with lymph node metastases was presented. Immunohistochemical analysis of a biopsy specimen obtained by EUS-FNA was useful for preoperative diagnosis.

Pancreatic somatostatinoma with obscure inhibitory syndrome and mixed pathological pattern

Somatostatinoma is a very rare neuroendocrine tumor that originates from D cells and accounts for less than 1% of all gastrointestinal endocrine tumors. The duodenum is the most frequent site for this tumor, followed by the pancreas. We here describe a 46-year-old Chinese woman who developed pancreatic somatostatinoma presenting with the characteristic "inhibitory" syndrome, but the symptoms were obscure and seemingly uncorrelated. This case is also unique for its large tumor size and mixed pathological pattern. Distal pancreatectomy was performed, and the patient has remained well since operation. As the syndromes of somatostatinoma may be obscure and atypical, clinicians should review all clinical findings to obtain an accurate diagnosis. Aggressive surgery is preferred to improve the survival.

FNAC diagnosis of pancreatic somatostatinoma

Somatostatinomas are rare pancreatic endocrine neoplasms (PEN). We present a case of a PEN in a 63-year-old lady having diabetes mellitus, cholelithiasis, steatorrhea, weight loss, indigestion, nausea and fatigue. Ultrasonography revealed a large calcified mass occupying the liver, pancreas and emboli in the splenic vein. Fine needle aspiration cytology (FNAC) of the metastatic mass in the liver showed features of neuroendocrine tumor. Correlating the clinical picture, computed tomography and cytological findings, a diagnosis of pancreatic somatostatinoma was suggested. Serum somatostatin levels and biopsy findings confirmed the same. The patient was put on chemotherapeutic agents and octreotide and is doing well after two years of follow-up. We discuss the FNAC findings of pancreatic endocrine tumors and its differentiating features from hepatoma and other small round cell tumors.

Diagnosis and treatment of pancreatic somatostatinoma: a case report

Diagnosis and treatment of pancreatic somatostatinoma: a case report

Pancreatic somatostatinoma and tuberous sclerosis: case report of an exceedingly rare association

smooth muscle. A common blood supply should be present. It should be lined by alimentary-tract epithelium. Rarely, respiratory-tract epithelium, in conjunction with alimentary-tract epithelium, has been noted. In adults, diagnosis is very difficult before surgery. Gastric duplication cysts are usually asymptomatic. However, signs and symptoms may include epigastric pain, abdominal mass, or vomiting. Rarely, GI bleeding, pancreatitis, peritonitis, malignancy, or even acute abdomen may result. Often, a GDC may be mistaken as a pancreatic pseudocyst, which is more common. Normal levels of pancreatic enzymes and no personal history of pancreatitis may suggest a gastric duplication cyst. Unlike GDC, the pancreatic pseudocyst is generally associated with repeated attacks of pancreatitis. They lack an epithelial lining and contain amylase-rich fluid. US, CT, and EUS may help in the diagnosis. In our case, the diagnosis was made before surgery by EUS-guided cyst aspiration and ERP. Surgical resection led to a satisfactory outcome. This case provides a fascinating opportunity to explore embryogenesis of the alimentary tract, as well as highlighting the usefulness of EUS and ERP in preoperative diagnosis.

Fine‐needle aspiration cytology of pancreatic somatostatinoma: The importance of immunohistochemistry for the cytologic diagnosis of pancreatic endocrine neoplasms

Pancreatic somatostatinoma is a rare pancreatic endocrine neoplasm representing as little as 1% of pancreatic endocrine neoplasms (PENs). The histologic features of this tumor are like those of other PENs, except that it commonly forms acinar structures and often has cells with abundant, granular cytoplasm. We have recently encountered two of these neoplasms sampled by endoscopic ultrasound (EUS)-guided fine-needle aspiration (FNA). We discuss the cytologic and immunohistochemical findings of these two cases and the cytologic similarities these neoplasms share with pancreatic acinar-cell carcinoma (PACC). We review the cytologic features of PEN and PACC and discuss the importance of cell block immunohistochemistry in the diagnosis of pancreatic neoplasia sampled by EUS-guided FNA.

Metastatic somatostatinoma of the chest wall

A 72-year-old male was diagnosed as having a thoracic tumor in the left chest wall five years after resection of primary pancreatic somatostatinoma. The tumor was suggestive of metastasis to the chest wall or to the left lung from the previously resected somatostatinoma based on the abnormally elevated serum somatostatin level. Percutaneous biopsy confirmed that the lesion was an islet cell tumor and thoracotomy demonstrated metastasis to the left third rib without involvement of the left lung. Our case represents a rare documentation of somatostatinoma metastatic to the chest wall for which complete resection was performed.

Pancreatic somatostatinoma associated with obstructive jaundice, pancreatic and biliary Kaposi's sarcoma in a man with neurofibromatosis

Pancreatic somatostatinoma associated with obstructive jaundice, pancreatic and biliary Kaposi's sarcoma in a man with neurofibromatosis

超音波ドプラー法が診断に有用であったすいソマトスタチノーマの１例

超音波ドプラー法が診断に有用であったすいソマトスタチノーマの１例

Reciprocal changes in progression of gastric mucosa-associated lymphoid tissue lymphoma and pancreatic somatostatinoma

Somatostatin is reported to inhibit the proliferation of various kinds of cells, including lymphocytes, through its receptors ( 1 Chiba T. Yamada T. Gut somatostatin. in: Walsh J.H. Comprehensive Endocrinology. Gut Peptides. Raven Press, New York1994: 123-146 Google Scholar ). Octreotide, a somatostatin analogue, has been used for the treatment of lymphomas, although its effectiveness is unclear ( 2 Witzig T.E. Letendre L. Gerstner J. et al. Evaluation of a somatostatin analog in the treatment of lymphoproliferative disorders results of a phase II north central cancer treatment group trial. J Clin Oncol. 1995; 13: 2012-2015 Crossref PubMed Scopus (34) Google Scholar ). We have cared for a patient with a low-grade mucosa-associated lymphoid tissue (MALT) lymphoma of the stomach accompanied by pancreatic somatostatinoma. Here we report that during the clinical course there were reciprocal changes in the plasma somatostatin concentration and the progression of the lymphoma, suggesting an inhibitory effect of plasma somatostatin on the proliferation of gastric MALT lymphoma.

A Case of Pancreatic Somatostatinoma Found Endoscopically with Metastasis to the Duodenal Periampulla

Abstract:A case of pancreatic somatostatinoma with duodenal metastasis is reported. The presenting feature was severe right hypochondrial pain with cholecystolithiasis and mild diabetes mellitus. Duodenogastroscopy revealed a small polypoid lesion at the duodenal peri‐ampulla of the major papilla. A biopsy specimen demonstrated a neuroendocrine tumor. Selective gastroduodenal arteriography disclosed a spherical hypervascular lesion in the pancreaticoduodenal arcade. Pancreatoduodenectomy was performed and pathological examination demonstrated pancreatic islet cell tumor and metastasis to the duodenal peri‐ampulla. Immunocytochemical examinations demonstrated intra‐cytoplasmic somatostatin and calcitonin in both tumors. The patient's basal plasma somatostatin level was 4 times higher than the normal range, however her plasma calcitonin level was within the normal range. After the operation, plasma somatostatin levels were within the normal range and her clinical course was good. In this case endoscopy was very useful in detecting the early stage duodenal metastasis of pancreatic somatostatinoma.

Pancreatic somatostatinoma: A case report and review of the literature

A 56-year-old man underwent distal pancreatectomy, splenectomy, and partial resection of the splenic flexure of the colon because of tumor in the tail of pancreas and the splenic hilus. The patient presented with symptoms of general malaise, anorexia, weight loss, mild diarrhea, and borderline diabetes mellitus, although there was no cholelithiasis. The diagnosis remained unclear until immunohistochemical studies of the resected specimen revealed somatostatin and synaptophysin, suggesting a somatostatinoma. Twenty-three reported cases of pancreatic somatostatinoma are reviewed and their clinical features discussed. The role of immunohistochemical studies in the diagnosis of somatostatinoma is described.

Pancreatic Somatostatinoma; Presentation with Recurrent Episodes of Severe Hyperglycaemia and Ketoacidosis

A 47-year-old woman was admitted on four occasions over a four-year period with severe hyperglycaemia associated with marked ketoacidosis. She had weight loss with hepatomegaly and ultrasonography indicated a pancreatic tumour which was shown to be a somatostatinoma. Resection resulted in prolonged survival. The biochemical and morphological features of this rare tumour are presented, and an explanation for the unusual presentation of a somatostatinoma with episodes of ketoacidosis is given.

Pancreatic somatostatinoma: abundance of somatostatin-28(1-12)-like immunoreactivity in tumor and plasma.

In the present study we characterized and compared the relative amounts of the different molecular forms of somatostatin-14 like immunoreactivity (S-14 LI) and of somatostatin-28(1-12) like immunoreactivity (S-28(1-12) LI) in extracts of tumor and peripheral plasma of a patient with a pancreatic somatostatinoma. Tissue and plasma were chromatographed on Sephadex G-50 columns equilibrated with 6 M urea. Immunoreactivity in the eluting fractions was assayed with two separate, region specific RIAs using antibodies R149 (S-14 LI) and S309 (S-28(1-12)LI). RIA R149 recognizes the 6-8 and 14 regions of the S-14 sequence and detects S-14, S-28, and prosomatostatin, an approximately 14,000 mol wt precursor for the two peptides. RIA S309 recognizes the 2-11 segment of S-28 and reacts with S-28, S-28(1-12), and higher mol wt S-28(1-12) LI but not S-14. Total tumor S-14 LI was 190 pmol/mg protein and consisted of three peaks of immunoreactivity of apparent 14,000 mol wt (14K S-14 LI), 3,200 mol wt (3.2K corresponding to S-28) and 1,600 mol wt (1.6K corresponding to S-14). The three peaks comprised, respectively, 7%, 57%, and 36% of total S-14 LI. Total tumor S-28(1-12) LI was 594 pmol/mg protein and eluted as four major peaks of immunoreactivity as follows: peak I (mol wt 15,000, 10% of total S-28(1-12) LI); peak II (mol wt 8,000, 20% of S-28(1-12) LI), peak III (corresponding to S-28, 19% of S-28(1-12) LI); peak IV (corresponding to S-28(1-12), approximately 50% of total S-28(1-12) LI). Total plasma concentration of S-14 LI was 714 pM, being made up of the three peaks found in tumor but in the following relative amounts (14K S-14 LI, 22%; 3.2K, 29%; 1.6 K, 49%). Plasma S-28(1-12) LI was 4 times higher (2879 pM) than S-14 LI and contained immunoreactivity corresponding to each of the four peaks found in the tumor. 1) The tumor and plasma concentrations of S-28(1-12) LI were greater than that of S-14 LI. 2) Both tumor and plasma S-14 LI and S-28 LI were heterogeneous and comprised species corresponding not only to S-14 but also S-28, S-28(1-12), prosomatostatin, and other higher mol wt forms of S-28.(ABSTRACT TRUNCATED AT 400 WORDS)

A Case of Duodenal Somatostatinoma: Diagnostic Usefulness of Calcium-Pentagastrin Test

A duodenal somatostatinoma is described in a patient suffering from a tumoral obstruction of the bile duct. The diagnosis was based upon histologic and immunocytochemical characterization of tumoral fragments and later confirmed by immunochemical analysis of liver metastases. Basal peripheral somatostatin immunoreactivity levels were normal until liver failure developed. A hypersomatostatinemia was induced by combined injection of calcium and pentagastrin, which was not the case in normal volunteers or in patients with disseminated pancreatic adenocarcinoma. This test was also positive in a case of generalized pancreatic somatostatinoma 4 yr after streptozotocin treatment. It was concluded that the calcium-pentagastrin test might be useful in the diagnosis of somatostatinomas.

Human somatostatin I: sequence of the cDNA.

RNA has been isolated from a human pancreatic somatostatinoma and used to prepare a cDNA library. After prescreening, clones containing somatostatin I sequences were identified by hybridization with an anglerfish somatostatin I-cloned cDNA probe. From the nucleotide sequence of two of these clones, we have deduced an essentially full-length mRNA sequence, including the preprosomatostatin coding region, 105 nucleotides from the 5' untranslated region and the complete 150-nucleotide 3' untranslated region. The coding region predicts a 116-amino acid precursor protein (Mr, 12.727) that contains somatostatin-14 and -28 at its COOH terminus. The predicted amino acid sequence of human somatostatin-28 is identical to that of somatostatin-28 isolated from the porcine and ovine species. A comparison of the amino acid sequences of human and anglerfish preprosomatostatin I indicated that the COOH-terminal region encoding somatostatin-14 and the adjacent 6 amino acids are highly conserved, whereas the remainder of the molecule, including the signal peptide region, is more divergent. However, many of the amino acid differences found in the pro region of the human and anglerfish proteins are conservative changes. This suggests that the propeptides have a similar secondary structure, which in turn may imply a biological function for this region of the molecule.

Characterization of somatostatin-like components in the tumors and plasma of a patient with a somatostatinoma.

Somatostatin-like immunoreactive polypeptides with approximate molecular weights of 1,500-2,000 (peak I), 2,500-3,500 (peak II), and 10,000-15,000 (peak III) were isolated from the peripheral plasma and from extracts of tumor tissue of a patient with a pancreatic somatostatinoma and hepatic metastases. The peak I component had approximately the same molecular charge as somatostatin, but the peak II component was appreciably more basic. The peak III component was heterogeneous with respect to charge and dissociated into smaller immunoreactive polypeptides during isoelectric focusing or after treatment with strongly denaturing solvents. In plasma, the levels of the larger molecular weight components (peak II and peak III), relative to that of the somatostatin-size component (peak I), were higher than in tissue extracts, suggesting a lower rate of clearance of the larger polypeptides from the circulation. In response to a nutrient stimulus, the peak I component increased to a greater extent than the larger components. Plasma levels of larger immunoreactive polypeptides relative to the peak I component in patient's plasma were higher after complete resection of the pancreatic tumor than preoperatively, suggesting that the rate of release of these putative precursor forms from the metastases was greater than from the primary tumor.

Calcitonin-Producing Pancreatic Somatostatinoma

A pancreatic somatostatinoma metastatized to the liver was detected in a 70-yr-old woman presenting with chronic diarrhea, steatorrhea, pancreatic insufficiency, diabetes mellitus, and achlorhydria. At immunocytochemistry, most tumor cells stored both somatostatin and calcitoninlike substances. Chromatography of acid extracts of the tumor on G50 Sephadex gave two distinct peaks coeluting with cyclic ovine somatostatin and human calcitonin, respectively, thus ruling out the hypothesis of a single cross-reacting molecule synthetized by the neoplastic cells. When the tumor was extracted at neutral pH, larger molecular forms of the above components were found, which accounted for less than 20% of the total immunoreactivity. Gel permeation of plasma showed that the circulating calcitonin- and somatostatinlike components consisted of three and four different forms, respectively, including components of molecular weights similar to those of the reference peptides. Inhibition curves and immunoadsorption experiments indicated that the large forms were immunologically similar, if not identical, to the corresponding standard preparations. The present case illustrates the occasional ability of neoplastic somatostatin cells of pancreas to synthetize simultaneously components immunologically related to somatostatin and calcitonin. These two inappropriate secretions could account for the symptoms displayed by this patient.

Pancreatic somatostatinoma: histologic, clinical, and angiographic features

Pancreatic somatostatinoma: histologic, clinical, and angiographic featuresAJ Gerlock, Jr, CA Muhletaler, S Halter and V GoncharenkoAudio Available | Share

Calcitonin Secretion by a Pancreatic Somatostatinoma

Calcitonin Secretion by a Pancreatic Somatostatinoma