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NCI CANCERLIT^® Search: Islet Cell Carcinoma - June 2002

National Cancer Institute^®
Last Modified: June 1, 2002

Insulinoma: diagnosis with fat-suppressed MR imaging.
Telomerase activity in pancreatic endocrine tumors.
Insulinoma with fibrillar inclusions and acinar cell elements.
[Fluid-fluid level in a non functioning and hemorragic neuro-endocrine islet-cell tumor of the pancreas: MRI features]
Insulinoma with subsequent association of Zollinger-Ellison syndrome.
[Diagnostic criteria of insulinoma]
Is glucagonoma of the pancreas a curable disease?

Table of Contents

1
UI - 8273692
AU - Kraus BB; Ros PR
TI - Insulinoma: diagnosis with fat-suppressed MR imaging.
SO - AJR Am J Roentgenol 1994 Jan;162(1):69-70
AD - Department of Radiology, University of Florida, Gainesville 32610.

2
UI - 12003383
AU - Tang SJ; Dumot JA; Wang L; Memmesheimer C; Conwell DL; Zuccaro G;
TI - Goormastic M; Ormsby AH; Cowell J Telomerase activity in pancreatic endocrine tumors.
SO - Am J Gastroenterol 2002 Apr;97(4):1022-30
AD - Department of Medicine, Cleveland Clinic Foundation, Ohio 44195, USA.
OBJECTIVES: Pancreatic endocrine tumors (PETs) have variable prognoses, and predictors of survival are lacking. PETs can be difficult to distinguish histologically from aggressive pancreatic neoplasms such as acinar cell carcinoma. Telomerase is a ribonuclear protein that maintains the length of the telomere and induces cell immortality. Telomerase is present in 95% of pancreatic adenocarcinoma and is associated with aggressive tumor behavior. Our aim is to determine telomerase activity in PETs and investigate its potential role as a prognostic indicator. METHODS: Telomerase detection using the telomeric repeat amplification protocol was performed on frozen surgical archived pancreatic endocrine tissue from 30 patients with PETs identified by light microscopy (hematoxylin-eosin stain). All results were confirmed with internal controls. A patient's survival was measured from the time of surgery. Acinar cell differentiation (presence of zymogen granules) was determined by electron microscopy. Follow-up data were acquired via telephone interview, medical record review, and death certificates. RESULTS: Three of 30 PETs diagnosed by light microscopy were telomerase positive: three were considered nonfunctional, and two of these three patients had extrapancreatic disease. All three telomerase-positive cases were reclassified as either acinar cell carcinoma (two cases) or mixed acinar-endocrine cell carcinoma (one case). All three patients (mean age = 63 yr) died from tumor progression within 2 yr of surgery (mean = 1.6 yr +/- 0.5 SD). The remaining PETs were telomerase negative: 13 insulinomas, four nonfunctional, two sporadic glucagonomas, one gastrinoma, one vipoma, one carcinoidlike PET, and five PETs from three patients with multiple endocrine neoplasm syndrome type I and two patients with von Hippel-Lindau syndrome. Excluding insulinomas, 12 of 14 patients with telomerase-negative PETs had extrapancreatic disease. Nevertheless, Kaplan-Meier survival estimates for these 12 patients were significantly longer than for patients with telomerase-positive acinar cell carcinoma (92% vs 0% at 2 yr, p = 0.001, log rank test). The survival of all telomerase-negative PETs (n = 27) was significantly longer than that of the patients with telomerase-positive acinar cell carcinoma (93% vs 0% at 2 yr, p = 0.0001). CONCLUSIONS: Telomerase activity helps to identify acinar cell carcinomas that histologically resemble PETs, which accounts for the poor prognosis demonstrated in these patients. The absence of telomerase activity in most PETs may be responsible for their indolent clinical course. Telomerase may identify potentially progressive tumors, such as acinar cell carcinoma, and may be useful in selecting patients for more aggressive treatment.

3
UI - 11783913
AU - Ordonez NG
TI - Insulinoma with fibrillar inclusions and acinar cell elements.
SO - Ultrastruct Pathol 2001 Nov-Dec;25(6):485-95
AD - The University of Texas M.D. Anderson Cancer Center, Houston 77030, USA.
Islet cell tumors associated with exocrine elements are rare. An insulinoma was removed from the head of the pancreas of a 33-year-old woman. Ultrastructural and immunohistochemical studies demonstrated that, in addition to the endocrine cells, the tumor had a small population of cells with an acinar cell morphology. Rare cells exhibiting both endocrine and exocrine features (amphicrine cells) were also identified. Another unusual finding in this case was the presence of a large number of intracytoplasmic filamentous inclusions that, even though they have been observed in other neoplasms, have not previously been reported in endocrine tumors of the pancreas. The demonstration of cells with mixed endocrine features supports the concept that both the endocrine and exocrine portions of the components of the pancreas have a common embryologic origin.

4
UI - 11591931
AU - Chevallier P; Pellegrino C; Bernard JL; Chevallier A; Souci J; Padovani
TI - B [Fluid-fluid level in a non functioning and hemorragic neuro-endocrine islet-cell tumor of the pancreas: MRI features]
SO - J Radiol 2001 Sep;82(9 Pt 1):1009-11
AD - Service d'Imagerie medicale, CHRU de Nice, Hopital Archet II, Nice, France. chevallier.p@chu-nice.fr
The authors report the MR features of a non functioning and hemorragic islet-cell tumor of the pancreas. This tumor was composed of a central cystic component with a fluid-fluid level seen on T1- and T2-weighted images and a peripheral hypervascular soft tissue component which showed hyperintensity on T2-weighted images with fat saturation.

5
UI - 11393407
AU - Mizuno N; Naruse S; Kitagawa M; Ishiguro H; Ito O; Ko SB; Yoshikawa T;
TI - Tanahashi C; Ito M; Hayakawa T Insulinoma with subsequent association of Zollinger-Ellison syndrome.
SO - Intern Med 2001 May;40(5):386-90
AD - Department of Internal Medicine II, Nagoya University School of Medicine.
We report a patient with insulinoma associated with Zollinger-Ellison syndrome. A 67-year-old woman was first admitted to our hospital for an abdominal mass. Abdominal computed tomography (CT) revealed a large pancreatic tumor, which was then diagnosed as an unresectable pancreatic adenocarcinoma. At the age of 71, she presented symptoms of hypoglycemia. Fasting blood glucose was 21 mg/dl and plasma immunoreactive insulin level was 846 microU/ ml. Plasma gastrin, glucagon, vasoactive intestinal polypeptide and somatostatin levels were all normal. At the age of 73, hypoglycemic attacks occurred more frequently and she was admitted to our hospital. Abdominal CT scan showed multiple liver metastases. Chemotherapy with 5-fluorouracil and doxorubicin was performed. Three months later, she had an emergency laparotomy because of a perforated duodenal ulcer. Plasma gastrin level was 1,960 pg/ml at that time. Gastric hypersecretion was well controlled with a proton pump inhibitor (lansoprazole) but she died of widespread cancer dissemination 8 years after her first admission. On autopsy, histologic examination revealed a mixed acinar-endocrine carcinoma of the pancreas. Immunohistochemical stains were positive for insulin, gastrin, and alpha1-antitrypsin.

6
UI - 11692406
AU - Flores Meneses L; Esmatjes Mompo E
TI - [Diagnostic criteria of insulinoma]
SO - Rev Clin Esp 2001 Sep;201(9):513-4
AD - Servicio de Endocrinologia y Diabetes, Instituto de Investigaciones Medicas Agusti Pi i Sunyer (IDIBAPS), Hospital Clinic Universitario, Barcelona.

7
UI - 12067219
AU - Chu QD; Al-kasspooles MF; Smith JL; Nava HR; Douglass HO Jr; Driscoll D;
TI - Gibbs JF Is glucagonoma of the pancreas a curable disease?
SO - Int J Pancreatol 2001;29(3):155-62
AD - Department of Surgical Oncology, Roswell Park Cancer Institute, State University of New York at Buffalo, 14263-0001, USA.
BACKGROUND: Glucagonomas are rare neuroendocrine tumors of the pancreas. Because of its rarity, its natural history is not well understood. AIM: We evaluated the natural history of glucagonomas treated at a tertiary care cancer center. METHODS: A retrospective analysis of 12 patients during 1970 to 2000 was performed. Six patients (50%) had a tumor located in the head of the pancreas. RESULTS: Abdominal pain (83%) and weight loss (75%) were the most common symptoms. Median tumor size was 6 cm (range 0.04-10). Seven patients (58%) had liver metastases. Five patients (42%) underwent curative resection. Overall median survival was 66 mo, and 5-yr overall survival was 66%. Five-yr overall survival was 83% for patients who had resection versus 50% for the non-resected patients (p = 0.04). Patients who were disease-free had a complete resection of the primary tumor and no liver involvement. CONCLUSIONS: Glucagonomas generally present with liver metastases at the time of diagnosis. Cure is only possible if the disease is localized and completely resected.

The above citations and abstracts reflect those newly added to CANCERLIT for the month and topic listed in the title. The citations have been retrieved from CANCERLIT using a predefined search strategy of indexed subject terms. Although the search strategy has been refined as best as possible, citations may appear that are not directly related to the topic, and occasionally relevant references may be omitted.

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