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Revista Española de Enfermedades Digestivas

versión impresa ISSN 1130-0108

Rev. esp. enferm. dig. vol.104 no.6  jun. 2012 



Ampullary carcinoid tumor. An atypical location

Tumor carcinoide primario de conductos biliares extrahepáticos, una localización atípica


Key words: Carcinoid tumor. Ampulloma. Extrahepatic bile ducts. Ampulla of Vater.

Palabras clave: Tumor carcinoide. Ampuloma. Conductos biliares extrahepáticos. Ampolla de Vater.


Dear Editor,

A 41-year-old male patient came to emergency because of conjunctival jaundice after suffering an infection of upper respiratory tract. His medical history included amputation of right leg after occupational accident and neurofibromatosis type I or Von Recklinghausen disease. On examination, patient had typical "café au lait" spots and subconjunctival jaundice, soft and depressible abdomen with one finger-breadth hepatomegaly. Analytically, he showed moderate macrocytic anemia (hemoglobin 12.7 g/dL) and elevated liver enzymes (total bilirubin 1.75 mg/dL, direct bilirubin 1.12 mg/dL, AST 186 U/l, ALT 331 U/l, GGT 1185 U/l and alkaline phosphatase 563 U/l). After classified as obstructive jaundice, imaging tests were requested (ultrasound and CT scan) to assess the cause, which showed a distal obstruction of bile duct without demonstrable cause. The study was supplemented by oral endoscopy where was appreciated a protrusion of duodenal mucosa in second portion, suggestive of ampuloma or pancreatic head cancer. An endoscopic retrograde cholangio-pancreatography (ERCP) demonstrated a friable tumor of 1 cm in diameter. Biopsy was highly suggestive of neoplastic proliferation, which had immunohistochemical characteristics of neuroendocrine carcinoid tumor (CR-, chromogranin A + + +, Vimentin +/-, CEA -, EMA + + +, S100 -, CK7 -, CK20-). With the diagnosis of ampullary carcinoid tumor, it was decided performing elective surgery, realizing a pancreaticoduodenectomy (pylorus-preserving modification of classic Whipple procedure). Postoperative course was favorable and uneventful; at present, after more than 8 years after surgery, patient is asymptomatic and without signs of local or distant recurrence.

Carcinoid tumors are the most common neuroendocrine tumors. Incidence has increased considerably in recent years, but can not be ruled to be due to an improvement in diagnostic methods (1).

The first ampullary carcinoid tumor was described by Oberndorfer in 1907 (2), being published to date approximately 120 cases in the literature (3). This tumors are very rare (2% of all ampullary tumors and 0.05% of all gastrointestinal carcinoids), with an incidence even lower than duodenal carcinoids (2%) (4). Most common symptoms are, in order of frequency, jaundice (53.1%), abdominal pain (24.6%), pancreatitis (6%) and weight loss (3.6%) (5).

Diagnosis is established by histological and immunohistochemical study of the lesion, being generally postoperative, because biopsy during endoscopy or ERCP has a high failure rate, due to is often submucosal location (6).

It has been demonstrated that tumor size has no correlation with metastatic potential, unlike duodenal carcinoids where size, invasion of muscularis propria and mitotic activity rather correlated, so that duodenal tumors smaller than 2 cm can be resected locally (7). So, given the propensity of ampullary carcinoid tumors to develop lymph node metastases, we recommend performing a Whipple procedure, regardless of tumor size, although there is no concrete evidence that this technique improves survival (8).


Luis Tallón-Aguilar, Remedios Jurado-Marchena, Antonio Tejada-Gómez,
Rafael Balongo-García and Pedro Luis Naranjo-Rodríguez

Department of General Surgery and Digestive Diseases. Hospital Infanta Elena. Huelva, Spain



1. Varas-Lorenzo MJ, Muñoz-Agel F, Espinós-Pérez JC, Bardají-Bofill M. Tumores carcinoides gastrointestinales. Rev Esp Enferm Dig 2010;102:533-7.         [ Links ]

2. Oberndorfer S. Karzinoide Tumoren des Dunndarms. Frankf Zschr Pathol 1907;1:426-32.         [ Links ]

3. Ozsoy M, Ozsoy Y, Canda AE, Nalbant OA, Haskaraca F. The rare malignancy of the hepatobiliary system: ampullary carcinoid tumor. Case Report Med 2011;17:30-6.         [ Links ]

4. Hartel M, Wente MN, Sido B, Friess H, Buchler MW. Carcinoid of the ampulla of Vater. J Gastroenterol Hepatol 2005;20:676-81.         [ Links ]

5. Senda E, Fujimoto K, Ohnishi K, Higashida A, Ashida C, Okutani T, et al. Minute ampullary carcinoid tumor with lymph node metastases: a case report and review of literature. World J Surg Oncol 2009;22:7-9.         [ Links ]

6. Selvakumar E, Rajendran S, Balachandar TG, Kannan DG, Jeswanth S, Ravichandran P, et al. Neuroendocrine carcinoma of the ampulla of Vater: a clinicopathologic evaluation. Hepatobiliary Pancreat Dis Int 2008;7:422-5.         [ Links ]

7. Rohan VS, Narendra H, Patel JJ, Tankshali RA. Carcinoid of ampulla of Vater. Indian J Cancer 2007;44:90-2.         [ Links ]

8. Carter JT, Grenert JP, Rubenstein L, Stewart L, Way LW. Neuroendocrine tumors of the ampulla of Vater: biological behavior and surgical management. Arch Surg 2009;144(6):527-31.         [ Links ]

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