Endoscopy 2008; 40(7): 602-605
DOI: 10.1055/s-2007-995740
Case report

© Georg Thieme Verlag KG Stuttgart · New York

EUS and clinical characteristics of cystic pancreatic neuroendocrine tumors

P.  Kongkam1 , M.  Al-Haddad1 , S.  Attasaranya1 , J.  O’Neil1 , S.  Pais1 , S.  Sherman1 , J.  DeWitt1
  • 1Division of Gastroenterology and Hepatology, Indiana University Medical Center, Indianapolis, Indiana, USA
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Publikationsverlauf

submitted 7 November 2007

accepted after revision 10 March 2008

Publikationsdatum:
08. Juli 2008 (online)

Pancreatic neuroendocrine tumors (PNETs) may rarely appear as cystic or mixed solid-cystic masses. The endoscopic ultrasound (EUS) morphology and cyst fluid characteristics of these tumors are not well clarified. We retrospectively identified nine adult patients with nine single cystic pancreatic neuroendocrine tumors (CNETs). These nine included 0.67 % of the 1344 patients with pancreatic cystic lesions and 9.5 % of the 95 confirmed PNETs evaluated over the 12-year study period. At presentation, four patients were asymptomatic and five had known acute pancreatitis (n = 2), MEN-1 syndrome with hypoglycemia (n = 1), and abdominal pain (n = 2). Median maximal tumor diameter was 26 mm (range 20 – 64 mm). EUS morphology was mixed solid and cystic (n = 4) or cystic alone (n = 5). Cytology from EUS-fine-needle aspiration (FNA) (median 2 passes; range 1 – 6) demonstrated a PNET, and immunocytochemistry was confirmatory in all patients. Cyst fluid carcinoembryonic antigen (CEA) (n = 4) and amylase (n = 5) ranged from 0.1 to 1.8 ng/ml (normal 0 – 2.5 ng/ml) and 72 to 1838 U/L (normal 25 – 161 U/L), respectively. Six patients underwent surgery, and the preoperative diagnosis was confirmed in all.

References

  • 1 Gumbs A A, Moore P S, Falconi M. et al . Review of the clinical, histological, and molecular aspects of pancreatic endocrine neoplasms.  J Surg Oncol. 2002;  81 45-53; discussion 54
  • 2 Wamsteker E J, Gauger P G, Thompson N W, Scheiman J M. EUS detection of pancreatic endocrine tumors in asymptomatic patients with type 1 multiple endocrine neoplasia.  Gastrointest Endosc. 2003;  58 531-535
  • 3 Buetow P C, Parrino T V, Buck J L. et al . Islet cell tumors of the pancreas: pathologic-imaging correlation among size, necrosis and cysts, calcification, malignant behavior, and functional status.  AJR. 1995;  165 1175-1179
  • 4 Gines A, Vazquez-Sequeiros E, Soria M T. et al . Usefulness of EUS-guided fine needle aspiration (EUS-FNA) in the diagnosis of functioning neuroendocrine tumors.  Gastrointest Endosc. 2002;  56 291-296
  • 5 Anderson M A, Carpenter S, Thompson N W. et al . Endoscopic ultrasound is highly accurate and directs management in patients with neuroendocrine tumors of the pancreas.  Am J Gastroenterol. 2000;  95 2271-2277
  • 6 Rosch T, Lightdale C J, Botet J F. et al . Localization of pancreatic endocrine tumors by endoscopic ultrasonography.  New Engl J Med. 1992;  326 1721-1726
  • 7 Palazzo L, Roseau G, Chaussade S. et al . Pancreatic endocrine tumors: contribution of ultrasound endoscopy in the diagnosis of localization.  Ann Chir. 1993;  47 419-424
  • 8 Zimmer T, Stolzel U, Bader M. et al . Endoscopic ultrasonography and somatostatin receptor scintigraphy in the preoperative localisation of insulinomas and gastrinomas.  Gut. 1996;  39 562-568
  • 9 De Angelis C, Carucci P, Repici A, Rizzetto M. Endosonography in decision making and management of gastrointestinal endocrine tumors.  Eur J Ultrasound. 1999;  10 139-150
  • 10 Gouya H, Vignaux O, Augui J. et al . CT, endoscopic sonography, and a combined protocol for preoperative evaluation of pancreatic insulinomas.  AJR. 2003;  181 987-992
  • 11 Thompson N W, Czako P F, Fritts L L. et al . Role of endoscopic ultrasonography in the localization of insulinomas and gastrinomas.  Surgery. 1994;  116 1131-1138
  • 12 Ardengh J C, de Paulo G A, Ferrari A P. EUS-guided FNA in the diagnosis of pancreatic neuroendocrine tumors before surgery.  Gastrointest Endosc. 2004;  60 378-384
  • 13 Brugge W R, Lauwers G Y, Sahani D. et al . Cystic neoplasms of the pancreas.  New Engl J Med. 2004;  351 1218-1226
  • 14 Goh B K, Ooi L L, Tan Y M. et al . Clinico-pathological features of cystic pancreatic endocrine neoplasms and a comparison with their solid counterparts.  Eur J Surg Oncol. 2006;  32 553-556
  • 15 Ahrendt S A, Komorowski R A, Demeure M J. et al . Cystic pancreatic neuroendocrine tumors: is preoperative diagnosis possible?.  J Gastrointest Surg. 2002;  6 66-74
  • 16 van der Waaij L A, van Dullemen H M, Porte R J. Cyst fluid analysis in the differential diagnosis of pancreatic cystic lesions: a pooled analysis.  Gastrointest Endosc. 2005;  62 383-389
  • 17 Brugge W R, Lewandrowski K, Lee-Lewandrowski E. et al . Diagnosis of pancreatic cystic neoplasms: a report of the cooperative pancreatic cyst study.  Gastroenterology. 2004;  126 1330-1336
  • 18 Kotoulas C, Panayiotides J, Antiochos C. et al . Huge non-functioning pancreatic cystic neuroendocrine tumour: a case report.  Eur J Surg Oncol. 1998;  24 74-76
  • 19 Sohaib S A, Reznek R H, Healy J C, Besser G M. Cystic islet cell tumors of the pancreas.  AJR. 1998;  170 217
  • 20 Brown K, Kristopaitis T, Yong S. et al . Cystic glucagonoma: a rare variant of an uncommon neuroendocrine pancreas tumor.  J Gastrointest Surg. 1998;  2 533-536
  • 21 Tandan V R, Gallinger S. Management of cystic lesions of the tail of the pancreas.  Can J Surg. 1995;  38 347-350
  • 22 Taniguchi K, Tomioka T, Komuta K. et al . Pleomorphic nonfunctioning islet cell tumor of the pancreas.  Int J Pancreatol. 1995;  17 83-89
  • 23 Sarui H, Yoshimoto K, Okumura S. et al . Cystic glucagonoma with loss of heterozygosity on chromosome 11 in multiple endocrine neoplasia type 1.  Clin Endocrinol. 1997;  46 511-516
  • 24 Schwartz R W, Munfakh N A, Zweng T N. et al . Nonfunctioning cystic neuroendocrine neoplasms of the pancreas.  Surgery. 1994;  115 645-649
  • 25 Weissmann D, Lewandrowski K, Godine J. et al . Pancreatic cystic islet-cell tumors. Clinical and pathologic features in two cases with cyst fluid analysis.  Int J Pancreatol. 1994;  15 75-79
  • 26 Marrano D, Campione O, Santini D. et al . Cystic insulinoma: a rare islet cell tumour of the pancreas.  Eur J Surg. 1994;  160 519-522
  • 27 Goto M, Nakano I, Sumi K. et al . Cystic insulinoma and nonfunctioning islet cell tumor in multiple endocrine neoplasia type 1.  Pancreas. 1994;  9 393-395
  • 28 Iacono C, Serio G, Fugazzola C. et al . Cystic islet cell tumors of the pancreas. A clinico-pathological report of two nonfunctioning cases and review of the literature.  Int J Pancreatol. 1992;  11 199-208
  • 29 Collins B T, Cramer H M. Fine-needle aspiration cytology of islet cell tumors.  Diagnostic Cytopathol. 1996;  15 37-45
  • 30 Modlin I M, Tang L H. Approaches to the diagnosis of gut neuroendocrine tumors: the last word (today).  Gastroenterology. 1997;  112 583-590
  • 31 Pereira P L, Wiskirchen J. Morphological and functional investigations of neuroendocrine tumors of the pancreas.  Eur Radiol. 2003;  13 2133-2146
  • 32 Hernandez L V, Mishra G, Forsmark C. et al . Role of endoscopic ultrasound (EUS) and EUS-guided fine needle aspiration in the diagnosis and treatment of cystic lesions of the pancreas.  Pancreas. 2002;  25 222-228

J. M. DeWitt, MD 

Department of Medicine
Division of Gastroenterology and Hepatology
Indiana University Medical Center

550 North University Blvd.UH 4100
Indianapolis
IN 46202-5121
USA

Fax: +1-317-278-8144

eMail: jodewitt@iupui.edu