Eur J Pediatr Surg 2004; 14(2): 126-129
DOI: 10.1055/s-2004-815861
Case Report

Georg Thieme Verlag KG Stuttart, New York · Masson Editeur Paris

Large Supraumbilical Pseudocystic Tumour due to Ectopic Pancreatic Tissue Located in a Rest of the Omphaloenteric Duct

B. Tillig1 , V. Gerein2 , W. Coerdt2 , R. Vahdad1 , J. Engert1
  • 1Department of Paediatric Surgery, Ruhr University of Bochum, Herne, Germany
  • 2Department of Paediatric Pathology, Institute of Pathology, Johannes Gutenberg University Hospital, University of Mainz, Mainz, Germany
Further Information

Publication History

Received: March 16, 2003

Accepted after Revision: July 12, 2003

Publication Date:
07 June 2004 (online)

Abstract

Ectopic umbilical pancreatic tissue is extremely rare. We report on a case of a two-year-old boy who suffered from a large recurrent supraumbilical tumour with central cystic degeneration. Ectopic pancreatic tissue was located within the submucosal layer of an umbilical rest of the omphaloenteric duct. Peptic erosion and inflammatory alteration of tissue surrounding the umbilical vein caused recurrent bleeding and formation of a pseudocyst as well as chronical inflammatory granulations within the abdominal wall.

Résumé

Le tissu ombilical pancréatique ectopique est extrêmement rare. Nous faisons un rapport sur un cas d'un garçon de deux ans qui souffrait d'une grosse tumeur supra-ombilicale récurrente kystique. Le tissu pancréatique ectopique était placé dans la sous-muqueuse d'un reste ombilical du conduit omphalo-entérique formant une tumeur pseudo-kystique avec un saignement récurrent secondaire à l'érosion de la veine ombilicale et à l'inflammation.

Resumen

El tejido pancreático umbilical ectópico es extremadamente raro. Presentamos el caso de un niño de 2 años con un gran tumor recurrente supraumbilical con degeneración quística central. El tejido pancreático ectópico estaba localizado en la submucosa de un resto umbilical del conducto onfaloénterico. La erosión péptica e inflamatoria del tejido que rodeaba la vena umbilical causó hemorragia recidivante y pseuodoquiste con inflamación crónica de la pared abdominal.

Zusammenfassung

Umbilikal gelegenes ektopes Pankreasgewebe ist äußerst selten. Wir berichten über einen Fall einer umbilikalen Ektopie von Pankreasgewebe bei einem 2 Jahre alten Jungen mit einem rezidivierenden großen supraumbilikalen Tumor mit zentraler zystischer Degeneration. Das ektope Pankreasgewebe war in der Submukosa eines Restes des Ductus omphaloentericus am Nabel lokalisiert. Offensichtlich aufgrund einer peptischen Erosion und entzündlicher Reaktionen der Umgebung der V. umbilicalis kam es zu rezidivierenden Blutungen mit Bildung einer Pseudozyste und chronisch entzündlicher Granulationen in der umgebenden Bauchwand.

References

  • 1 Adda G, Hannoun L, Laygue J. Development of the human pancreas: variations and pathology. A tentative classification.  Anal Clin. 1984;  5 275-283
  • 2 Barbosa J JD, Dockerty M B, Waugh J M. Pancreatic heterotopia. Review of the literature and report of 41 authentical cases, of which 25 were clinically significant.  Surg Gynecol Obstet. 1946;  82 527-542
  • 3 Caberwal D, Kogan S J, Levitt S B. Ectopic pancreas presenting as an umbilical mass.  J Pediatr Surg. 1977;  12 593-599
  • 4 Guillou L, Nordback P, Gerber C, Schneider R P. Ductal adenocarcinoma arising in a heterotopic pancreas situated in a hiatal hernia.  Arch Pathol Lab Med. 1994;  118 568-571
  • 5 Hara M, Tsutsumi Y. Immunohistochemical studies of endocrine cells in heterotopic pancreas.  Virchows Arch A. 1986;  408 383-394
  • 6 Harold K L, Sturdevant M, Metthews B D, Mishra G, Heniford T. Ectopic pancreatic tissue presenting as submucosal gastric mass.  J Laparoendosc Adv Surg Tech. 2002;  12 333-337
  • 7 Hazzan D, Peer G, Shiloni E. Symptomatic heterotopic pancreas of stomach.  IMAJ. 2002;  4 388-389
  • 8 Hlavaty T, Lukac L, Vyskocil M, Galbavy S. Heterotopic pancreas in gastric antrum with macroscopic appearance of gastric polyp.  Bratisl Lek Listy. 2002;  103 117-120
  • 9 Kaneda M, Yano T, Yamamoto T, Suzuki T, Fujimori K, Itoh H, Mizumoto R. Ectopic pancreas in the stomach presenting as an inflammatory abdominal mass.  Am J Gastroenterol. 1989;  84 663-666
  • 10 Lai Edward C S, Tomkins R K. Heterotopic pancreas: review of a 26-year experience.  Am J Surg. 1986;  151 697-700
  • 11 Lee H Y, Choi Y H, Song I S, Lee J B, Yoo S M, Yang S J. Lithiasis in a heterotopic pancreas of the stomach.  J Comput Assist Tomogr. 2003;  27 85-87
  • 12 Melmed C, Ezrin C, Kovacs K, Goodman R S, Frohman L A. Acromegaly due to secretion of growth hormone by an ectopic pancreatic islet cell tumor.  N Engl J Med. 1985;  312 9-17
  • 13 Pang L C. Pancreatic heterotopia. A reappraisal and clinicopathologic analysis of 32 cases.  South Med J. 1988;  81 1264-1275
  • 14 Paolucci P, Brasesco O E, Rosin D, Saber A A, Avital S, Berho M, Rosenthal R J. Laparoscopic resection of ectopic pancreas in the gastric antrum: case report and literature review.  J Laparoendosc Adv Surg Tech. 2002;  12 139-141
  • 15 Prasad T RS, Gupta S D, Bhatnagar V. Ectopic pancreas associated with a choledochal cyst and extrahepatic biliary atresia.  Pediatr Surg Int. 2001;  17 552-554
  • 16 Sharma L K, Jaiswal T S. Isolated ectopic pancreatic tissue at umbilicus.  Indian J Pediatr. 1980;  47 347
  • 17 Sloots C EJ, de Brauw L M, Bot F J, Greve J WM. False-positive cytology in diagnostic laparoscopy due to ectopic pancreas.  Dig Surg. 1999;  16 434-436
  • 18 Tan H L, Yoong A, Yu C CW. Ectopic pancreatic rests: a rare cause of persistent umbilical discharge.  Pediatr Surg Int. 2000;  16 116-117
  • 19 Tilson M D, Touloukian R J. Mediastinal enteric sequestration with aberrant pancreas: A formes frustes of the intralobar sequestration.  Ann Surg. 1972;  176 669-671
  • 20 Tornòczky T, Kàlmàn E, Jàksò P, Mèhes G, Pajor L, Kajtàr G G, Battyàny I, Davidovics S, Sohail M, Krausz T. Solid and papillary epithelial neoplasm arising in heterotopic pancreatic tissue of the mesocolon.  J Clin Pathol. 2001;  54 241-246
  • 21 Ura H, Denno R, Hirata K, Saeki A, Hirata K, Natori H. Carcinoma arising from ectopic pancreas in the stomach: endosonographic detection of malignant change.  J Clin Ultrasound. 1998;  26 265-268
  • 22 v. Schweinitz D, Wittekind Ch, Freihorst J. Mediastinaler Sequester mit ektopem Pankreasgewebe.  Z Kinderchir. 1990;  45 249-250
  • 23 Willis R A. Some unusual developmental heterotopias.  Brit Med J. 1968;  3 267-272

Priv.-Doz. Dr. med. Bernd Tillig

Kinderchirurgische Klinik der Ruhr-Universität Bochum am Marienhospital Herne

Widumer Strasse 8

44267 Herne

Germany

Email: bernd.tillig@ruhr-uni-bochum.de

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