Subscribe to RSS
Please copy the URL and add it into your RSS Feed Reader.
https://www.thieme-connect.de/rss/thieme/en/10.1055-s-00000012.xml
Download PDF
Endoscopy 2003; 35(10): 854-857
DOI: 10.1055/s-2003-42613
DOI: 10.1055/s-2003-42613
Case Report
© Georg Thieme Verlag Stuttgart · New YorkAmpullary Carcinoid Tumor Presenting as Acute Pancreatitis in a Patient with von Recklinghausen’s Disease: Case Report and Review of the Literature
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