Endoscopy 2003; 35(10): 854-857
DOI: 10.1055/s-2003-42613
Case Report
© Georg Thieme Verlag Stuttgart · New York

Ampullary Carcinoid Tumor Presenting as Acute Pancreatitis in a Patient with von Recklinghausen’s Disease: Case Report and Review of the Literature

W.  Mayoral1 , J.  Salcedo1 , F.  Al-Kawas1
  • 1Division of Gastroenterology. Georgetown University Hospital, Washington, DC, USA
Further Information

Publication History

Submitted 16 November 2001

Accepted after Revision 21 March 2003

Publication Date:
10 October 2003 (online)

A patient with von Recklinghausen syndrome presented with multiple attacks of pancreatitis. Evaluation using computed tomography, endoscopic retrograde cholangiopancreatography, and endosonography revealed an ampullary carcinoid.

The patient underwent a pylorus-sparing Whipple resection, and is doing well after 30 months of follow-up. This is the second reported case of ampullary carcinoid presenting with pancreatitis in a patient with von Recklinghausen syndrome.

References

  • 1 Warren W K, McDonald M W, Humelogan J C. Periampullary and duodenal carcinoid tumors.  Gut. 1964;  5 448-449
  • 2 Walton G F, Gibbs E R, Spencer G O, Laws H L. Carcinoid tumors of the ampulla of Vater.  Am Surg. 1997;  63 302-304
  • 3 Hochberg F H, DaSilva A BNN, Galdabini J, Richardson E P. Gastrointestinal involvement in von Recklinghausen’s neurofibromatosis.  Neurology. 1974;  24 224-251
  • 4 Juan I K, Sono F, Okada T. et al . Neurogenic tumor of small intestine: report of a case with review of the literature.  Gastroenterol Jpn. 1980;  15 112-119
  • 5 Ghrist T D. Gastrointestinal involvement in neurofibromatosis.  Arch Intern Med. 1963;  112 111-116
  • 6 Levy D, Khatib R. Intestinal neurofibromatosis with malignant degeneration.  Dis Colon Rectum. 1960;  3 140-144
  • 7 Davis G B, Berk R N. Intestinal neurofibromas in von Recklinghausen’s disease.  Am J Gastroenterol. 1973;  60 410-414
  • 8 Gossett A, Masson P. Tumeurs endocrines de l’appendice.  Presse Méd. 1914;  22 237-240
  • 9 Lynch T, Gutman D H. Neurofibromatosis I.  Neurol Clin. 2002;  20 841-865
  • 10 Hansen O P, Hansen M, Hansen H H, Rose B. Multiple endocrine adenomatoses of mixed type.  Acta Med Scand. 1976;  200 327-331
  • 11 Basson M D, Ahlman H, Wangberg B. et al . Biology and management of the midgut carcinoid.  Am J Surg. 1993;  165 288-297
  • 12 Cantor A M, Rigby C C, Beck P R, Mangion D. Neurofibromatosis, phaeochromocytoma and somatostatinoma.  Br Med J. 1982;  285 1618-1619
  • 13 Perrone T. Duodenal gangliocytic paraganglioma and carcinoid.  Am J Pathol. 1986;  10 147-148
  • 14 Hardt P D, Doppl W E, Klor H U, Hinrichs B. A rare combination of pheochromocytoma and somatostatin-rich neuroendocrine tumor of Vater’s papilla (carcinoid) in a patient with von Recklinghausen neurofibromatosis.  Z Gastroenterol. 1998;  36 233-238
  • 15 Klein A, Clemens J, Cameron J. Periampullary neoplasms in von Recklinghausen’s disease.  Surgery. 1989;  106 815-819
  • 16 Moertel C G, Sauer W G, Dockerty M B, Baggenstoss A H. Life history of the carcinoid tumor of the small intestine.  Cancer. 1961;  14 901-912
  • 17 Kuiper D H, Gracie W A, Pollard H M. Twenty years of gastrointestinal carcinoids.  Cancer. 1970;  25 1424-1430
  • 18 Kahrilas P J, Hogan W J, Geenen J E. et al . Chronic recurrent pancreatitis secondary to a submucosal ampullary tumor in a patient with neurofibromatosis.  Dig Dis Sci. 1987;  32 102-107
  • 19 Lee H Y, Garber P E. Von Recklinghausen’s disease associated with pheochromocytoma and carcinoid tumor.  Ohio State Med J. 1970;  66 583-586
  • 20 Weichert L, Roth L, Krementz E. et al . Carcinoid-islet cell tumors of the duodenum.  Am J Surg. 1971;  121 195-205
  • 21 Barber P V. Carcinoid tumor of the ampulla of Vater associated with cutaneous neurofibromatosis.  Postgrad Med J. 1976;  52 514-517
  • 22 Johnson L, Weaver M. Von Recklinghausen’s disease and gastrointestinal carcinoids.  JAMA. 1981;  245 2496
  • 23 Alamowitch C, Larousse C. Association maladie de Recklinghausen et carcinoide de l’ampoule de Vater: une nouvelle entité nosologique: les apudomes.  Nouv Presse Méd. 1982;  11 3255-3257
  • 24 Hough D R, Chan A, Davidson H. Von Recklinghausen’s disease associated with gastrointestinal carcinoid tumors.  Cancer. 1983;  51 2206-2208
  • 25 Kapur B, Sarin S, Annad C. Carcinoid tumor of the ampulla of Vater associated with viscero-cutaneous neurofibromatosis.  Postgrad Med J. 1983;  59 734-735
  • 26 Marcial A M, Pinkus S G, Skarin A. et al . Ampullary somatostatinoma, psammomatous variant of gastrointestinal carcinoid tumor - an immunohistochemical and ultrastructural study: report of a case and review of the literature.  Am J Clin Pathol. 1983;  80 755-761
  • 27 Weder W, Saremaslani P, Maurer R. [Calcitonin-producing duodenal carcinoid in Recklinghausen’s neurofibromatosis: clinical case report and review of the literature; in German].  Schweiz Med Wochenschr. 1983;  113 885-892
  • 28 Dawson B V, Kazama R, Papplanus S H. Association of carcinoid with neurofibromatosis.  South Med J. 1984;  77 511-513
  • 29 Boissonnas A, Buttin S, Khalifa P. et al . [Carcinoid tumor of the ampulla of Vater and Recklinghausen’s neurofibromatosis, in French.  Ann Méd Interne (Paris). 1985;  136 46-48
  • 30 Hibi M, Kato Y, Nimura Y. An operative case of primary malignant somatostatinoma of the papilla of Vater: report of a case.  Hokkaido J Surg. 1985;  30 227
  • 31 Lumsden A, Williams A, Dixon J. Carcinoid tumor of the ampulla of Vater.  Eur J Surg Oncol. 1985;  11 55-56
  • 32 Stamm B, Hedinger C E, Sremaslani P. Duodenal and ampullary carcinoid tumors.  Virchows Arch Pathol Anat. 1986;  408 475-489
  • 33 Mulder C J, Festen H P, Mertens J C. et al . Carcinoid tumor of the ampulla of Vater presenting with biliary obstruction: report of four cases.  Gastrointest Endosc. 1987;  33 385-387
  • 34 Turri L, Fangaresi E. Multiple duodenal and ampullary carcinoids and von Recklinghausen’s neurofibromatosis.  Chir Ital. 1988;  4 285-294
  • 35 Scully R E, Mark E J, McNeely W F, McNeely B U. Case records of the Massachusetts General Hospital. Weekly clinicopathological exercises. Case 15 - 1989. A 52-year old man with neurofibromatosis and jaundice.  N Engl J Med. 1989;  320 996-1004
  • 36 Burke A P, Sobin L H, Shekitka K M. et al . Somatostatin-producing duodenal carcinoids in patients with von Recklinghausen’s neurofibromatosis.  Cancer. 1990;  65 1591-1595
  • 37 de Mol van Otterloo J C, Ooms E C, Lips C J. et al . [Neurofibromatosis and a tumor of Vater’s papilla; in Dutch].  Ned Tijdschr Geneeskd. 1991;  135 1646-1649
  • 38 Chen C H, Lin J T, Lee W Y. et al . Somatostatin-containing carcinoid tumor of the duodenum in neurofibromatosis: report of a case.  J Formos Med Assoc. 1993;  92 900-903
  • 39 Van Basten J P, van Hoek B, de Bruine A. et al . Ampullary carcinoid and neurofibromatosis: case report and review of the literature.  Neth J Med. 1994;  44 202-206
  • 40 Simon L, Kiss J, Kovacs H. et al . Neurofibromatosis (von Recklinghausen’s disease) and carcinoid tumor in Vater’s ampulla.  Orv Hetil. 1995;  136 2287-2292
  • 41 Tan C C, Hall R I, Semeraro D. et al . Ampullary somatostatinoma associated with von Recklinghausen’s neurofibromatosis presenting as obstructive jaundice.  Eur J Surg Oncol. 1996;  3 298-301
  • 42 Ramo O J, Ristkari S KK, Miettinen M. et al . Duodenal carcinoid tumor with psammoma bodies: a rare cause of hematemesis.  Eur J Sur. 1997;  163 227-230
  • 43 Hardt P D, Doppl W E, Klor H U, Hinrichs B. A rare combination of pheochromocytoma and somatostatin-rich neuroendocrine tumor of Vater’s papilla (carcinoid) in a patient with von Recklinghausen neurofibromatosis.  Z Gastroenterol. 1998;  36 233-238

F. H. Al-Kawas, M.D.

Division of Gastroenterology, Georgetown University Hospital

3800 Reservoir Road, NW · Washington · DC 20007 · USA

Fax: +1-2027843957

Email: alkawasf@gunet.georgetown.edu

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