National Cancer Institute®
Last Modified: July 1, 2002
UI - 11963761
AU - Hempfling W; Rust C; Furst H; Aust DE; Reiser M; Jacob K; Engelhardt D
TI - [57-year-old patient with collapse during automobile driving]
SO - Internist (Berl) 2002 Feb;43(2):259-62
AD - Medizinische Klinik II, Klinikum Grosshadern, Ludwig-Maximilians-Universitat, Marchioninistrasse 15, 81377 Munchen.
UI - 11966750
AU - Scott A; Hinwood D; Donnelly R
TI - Radio-frequency ablation for symptom control in a patient with metastatic pancreatic insulinoma.
SO - Clin Endocrinol (Oxf) 2002 Apr;56(4):557-9
AD - Division of Vascular Medicine, Nottingham University, Derbyshire Royal Infirmary, London Road, Derby DE1 2QY, UK. firstname.lastname@example.org
Malignant insulinomas are very rare endocrine tumours with a variable clinical course. We describe a 51-year-old man who had two large insulinomas resected from the body of the pancreas and 19 years later, having again become symptomatic, was found to have hepatic metastases. Medical treatment with diazoxide and octreotide failed to control his symptoms, but repeated hepatic embolization effected both symptomatic and biochemical improvements for a further 5 years. When symptoms recurred but further embolization failed to control his symptoms the hepatic metastases were treated by outpatient percutaneous radio-frequency ablation. He remains symptom-free 18 months later and levels of insulin and pro-insulin have nearly normalized. The survival, with liver metastases, for 27 years in a man with a malignant insulinoma has not been described previously. Malignant insulinoma may follow a rather indolent course and symptoms respond well to locally destructive therapies. Hepatic embolization is less traumatic than hepatic lobe resection and radio-frequency ablation offers an alternative if vascular access to the tumour is no longer possible.
UI - 12049533
AU - Kouvaraki MA; Lee JE; Shapiro SE; Gagel RF; Sherman SI; Sellin RV; Cote
TI - GJ; Evans DB Genotype-phenotype analysis in multiple endocrine neoplasia type 1.
SO - Arch Surg 2002 Jun;137(6):641-7
AD - Department of Surgical Oncology, The University of Texas MD Anderson Cancer Center, 1515 Holcombe Blvd, Houston, TX 77030, USA.
HYPOTHESIS: Multiple endocrine neoplasia type 1 (MEN 1) syndrome is an autosomal dominant disorder caused by germline mutations in the MEN1 gene and characterized by multiple endocrine tumors, most notably in the parathyroid glands, pituitary, and pancreas. The syndrome demonstrates variable expressivity and considerable genetic heterogeneity. Patient data were examined for possible associations between genotype and phenotype. DESIGN: We reviewed recorded medical data from 1975 to 2001 on patients with MEN 1 and compared specific types and locations of MEN1 gene mutations with manifestations of the syndrome. PATIENTS AND RESULTS: We identified 109 affected patients from 24 MEN 1 kindreds. The phenotypic expression of MEN 1 in affected individuals included hyperparathyroidism in 74%, pancreatic endocrine tumors in 51%, and pituitary tumors in 35%. Twelve of 14 insulinomas occurred in patients with pituitary tumors. Mutation analysis was completed in 14 of 24 kindreds (80 of the 109 patients). Mutations were most common in exons 2 (31%), 9 (15%), and 10 (23%). All 21 patients with frameshift mutations (and known pancreatic endocrine tumor status) had such tumors. Pituitary tumors were associated with frameshift mutations in exon 2. CONCLUSIONS: The type and location of MEN1 mutations may be associated with the phenotypic expression of specific tumors. Such information may assist in the genetic counseling and surveillance of at-risk patients. A specific genotype-phenotype correlation is unlikely because of the heterogeneity of the mutations in the MEN1 gene.
UI - 11702127
AU - Mortele KJ; Oei A; Bauters W; Timmermans F; Cuvelier C; Kunnen M; Ros PR
TI - Dynamic gadolinium-enhanced MR imaging of pancreatic VIPoma in a patient with Verner-Morrison syndrome.
SO - Eur Radiol 2001;11(10):1952-5
AD - Department of Radiology, University Hospital Gent, De Pintelaan 185, 9000 Gent, Belgium. email@example.com
We describe a rare case of a pancreatic VIPoma diagnosed in a patient presenting with watery diarrhea, hypokalemia, and achlorhydria, the so-called WDHA or Verner-Morrison syndrome. Emphasis is placed on the dynamic gadolinium-enhanced MR profile of the tumor, characteristics which have not been illustrated previously, to the best of our knowledge.
UI - 12057114
AU - Li ML; Norton JA
TI - Gastrinoma.
SO - Curr Treat Options Oncol 2001 Aug;2(4):337-46
AD - Department of Surgery, University of California, San Francisco, USA.
Gastrinoma treatment has evolved considerably in the last 20 years. In particular, the advent of effective acid-reducing pharmacologic agents has changed the primary morbidity of this disease entity from one of acid hypersecretion to one of tumor growth and spread. Thus, while symptoms can be temporized using histamine receptor antagonists, proton pump inhibitors, or somatostatin analogs, cure can be effected only by surgical means. Recent advances in operative techniques and pre- and intra-operative imaging studies, including routine duodenotomy, somatostatin-receptor scintigraphy, and intraoperative ultrasound, have allowed for identification and subsequent resection of more than 95% of gastrinoma tumors. Most experts agree that all sporadic cases of localized gastrinoma should be excised. In addition, debulking of metastatic tumor may improve symptoms and survival when cure cannot be ascertained. There is, however, some controversy as to the surgical approach for gastrinoma found in the setting of multiple endocrine neoplasia, type 1. Because of the usual multiplicity and particular indolence of these tumors, two primary strategies have emerged: aggressive approaches have been advocated in an effort to eradicate all present and potential tumor; and less aggressive, or nonoperative, approaches have been suggested because it is unclear whether intervention offers survival or disease-free benefit in this population. We advocate surgical intervention for patients with gastrinoma and multiple endocrine neoplasia, type 1 when tumors exceed 2.5 cm in size. This tumor size has been associated with a higher likelihood of hepatic metastases, which ultimately affects survival. The role of adjuvant therapies for gastrinoma remains limited.
UI - 12081066
AU - Hirshberg B; Libutti SK; Alexander HR; Bartlett DL; Cochran C; Livi A;
TI - Chang R; Shawker T; Skarulis MC; Gorden P Blind distal pancreatectomy for occult insulinoma, an inadvisable procedure.
SO - J Am Coll Surg 2002 Jun;194(6):761-4
AD - Division of Intramural Research, National Institute of Diabetes, Digestive and Kidney Diseases, National Institutes of Health, Bethesda, MD, USA.
BACKGROUND: Fasting hypoglycemia with neuroglycopenic symptoms corrected by administration of glucose are the hallmarks for the diagnosis of insulinoma. Surgical resection is the treatment of choice for insulinomas, but localization of these lesions can be challenging. Blind distal pancreatectomy has been advocated for occult insulinomas not detected on imaging studies or during abdominal exploration. With the advent of newer localization techniques, we challenge the wisdom of this approach. STUDY DESIGN: The records of patients (multiple endocrine neoplasia excluded) with pathologically proved insulinoma who were screened at our institution or referred to us after a failed blind distal pancreatectomy were reviewed. All records included patient history and results of physical examination and routine blood and urine tests. The diagnosis of insulinoma was confirmed during a supervised fast. Patients with biochemically proved insulinoma underwent localization studies and operation. Studies included CT scans, MRI, transabdominal ultrasound, intraoperative ultrasonography, angiography (more recently, Ca++-stimulated arteriography), and venous sampling. RESULTS: From 1970 to 2000, 99 patients (34 men, 65 women; mean age 43 years) underwent operation. All patients with benign tumors (92) were cured after operation. Seventeen patients were referred to the NIH after a failed blind distal pancreatectomy. Of these, 5 were diagnosed as having factitious hypoglycemia. In the other 12 patients a tumor was localized in the pancreatic head. Two patients incorrectly diagnosed with nesidioblastosis after initial surgery were subsequently cured by resection of an insulinoma. CONCLUSIONS: The use of preoperative imaging studies, most notably Ca++-stimulated arteriography, and intraoperative ultrasonography permits detection of virtually all insulinomas, including reopcrated cases. When a tumor is not detected, the procedure should be terminated and the patient referred to a center capable of performing advanced preoperative and intraoperative localization techniques. With the preoperative and intraoperative imaging strategies currently available, the use of blind distal pancreatectomy for occult insulinoma should be abolished.
UI - 11961480
AU - Takacs CA; Krivak TC; Napolitano PG
TI - Insulinoma in pregnancy: a case report and review of the literature.
SO - Obstet Gynecol Surv 2002 Apr;57(4):229-35
AD - Department of Obstetrics and Gynecology, Malcolm Grow Medical Center, Andrews Air Force Base, Maryland 20762-6600, USA. firstname.lastname@example.org
Insulinomas are rare tumors with an incidence of approximately four cases per million person-years. Nineteen cases of insulinoma during pregnancy have been reported. Hypoglycemic symptoms usually appear during the first trimester. A 28-year-old primigravida was admitted at 6 weeks of gestation after referral for uncontrolled seizures. Her previous seizure work-up included a normal EEG and a normal magnetic resonance imaging of the brain. Elevated fasting insulin and C-peptide levels accompanied severe hypoglycemia. The patient was managed with glucose monitoring, frequent small meals, and rare doses of glucagon. Postpartum testing was consistent with insulinoma, and magnetic resonance imaging indicated a mass in the tail of the pancreas. During surgical exploration with intraoperative ultrasound, two insulinomas were removed from the tail of the pancreas. The hypoglycemic episodes resolved and the fasting glucose levels normalized. Insulinomas are rare in pregnancy and can be difficult to diagnose. Symptoms may resolve during the second and third trimesters, possibly due to changes in glucose metabolism associated with pregnancy. Misdiagnosis has been fatal. Careful management during pregnancy and aggressive treatment after delivery are essential. TARGET AUDIENCE: Obstetricians and Gynecologists, Family Physicians. LEARNING OBJECTIVES: After completion of this article, the reader will be able to describe the pathophysiology of an insulinoma, to list the potential tests used to make the diagnosis of insulinoma, and to outline potential treatment options for a patient with an insulinoma.
UI - 11831609
AU - Levy MJ; Wiersema MJ
TI - Endoscopic ultrasound in the diagnosis and staging of pancreatic cancer.
SO - Oncology (Huntingt) 2002 Jan;16(1):29-38, 43; discussion 44, 47-9, 53-6
AD - Division of Gastroenterology and Hepatology, The Mayo Clinic Rochester, Minnesota 55905, USA. email@example.com
Patients with signs and symptoms suggestive of a pancreatic neoplasm typically undergo initial imaging with transabdominal ultrasound or computed tomography. This evaluation often reveals the presence of a pancreatic mass or fullness. At times, the nature of the lesion is poorly characterized, with uncertainty remaining as to whether the lesion is an inflammatory mass or a neoplasm, and if it is cystic or solid. In these circumstances, endoscopic procedures such as endoscopic retrograde cholangiopancreatography and/or endoscopic ultrasound may be required. These procedures offer other means of tissue sampling, disease staging, and an option for palliative therapy. In this article, we review the role of endoscopy for the diagnosis and staging of pancreatic tumors, with a particular focus on endoscopic ultrasound.
UI - 12078143
AU - Harjola VP; Farkkila M
TI - [Recurrent perforations of small intestines, gastroduodenal ulcers and diarrhea in a middle-aged woman]
SO - Duodecim 2000;116(7):755-60
AD - HYKS:n sisatautien klinikka Haartmaninkatu 4, 00290 Helsinki. firstname.lastname@example.org
UI - 12094386
AU - Fukunaga M
TI - Gastric carcinoma resembling pancreatic mixed acinar-endocrine carcinoma.
SO - Hum Pathol 2002 May;33(5):569-73
AD - Department of Pathology, Jikei University School of Medicine, Tokyo, Japan.
A case of gastric carcinoma resembling pancreatic mixed acinar-endocrine carcinoma of 77-year-old female is presented. This type of gastric tumor has not been previously reported. The endoscopic mucosal resection specimen of the fundus contained a 1.2 x 0.9 x 0.3 cm, well-circumscribed, tan, soft nodular tumor with protruded configuration with a central recess. Histologically, the tumor was confined to the mucosa and submucosa and was characterized by three growth patterns; acinar, solid, and glandular. The growth patterns were intermingled. The tumor cells in the acinar component had round nuclei with prominent nucleoli and diastase-resistant, periodic acid-Schiff-positive, eosinophilic cytoplasm. Immunohistochemically, the tumor cells were positive for CAM5.2, cytokeratin (CK) 7, CK 20, trypsin, lipase, alpha-1-antitrypsin, and alpha-1-antichymotrypsin. The tumor cells in the solid component were positive for Grimelius stain and chromogranin A. The findings indicated that the tumor showed acinar and endocrine differentiation. There was no heterotopic pancreas tissue in the specimen. The patient was well without tumor at the 7-month follow-up. It is important to know the existence of this type of gastric cancer and to not confuse it with a metastatic lesion of the pancreatic origin. Copyright 2002, Elsevier Science (USA). All rights reserved.
UI - 12095598
AU - McIntyre TP; Stahlfeld KR; Sell HW Jr
TI - Gastrinoma.
SO - Am J Surg 2002 Jun;183(6):666-7
AD - Department of Surgery, Mercy Hospital of Pittsburgh, 1400 Locust St., Pittsburgh, PA 15232-4738, USA.
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