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Revista Española de Enfermedades Digestivas

versión impresa ISSN 1130-0108

Rev. esp. enferm. dig. vol.104 no.9 Madrid sep. 2012 



Signet ring cell adenocarcinoma of the ampulla of Vater: A rare pathology

Adenocarcinoma con células en anillo de sello de la ampolla de Vater: una patología infrecuente



Key words: Ampulla of Vater. Signet ring cell. Adenocarcinoma.

Palabras clave: Ampolla de Vater. Células en anillo de sello. Adenocarcinoma.


Dear Editor,

Tumors of the ampulla of Vater are uncommon, representing 0.2% of malignant tumors of the gastrointestinal tract and 8% of malignant tumors of the biliopancreatic area. They arise from the intestinal mucosa of the ampulla of Vater, being adenocarcinoma the most frequent (99% of cases). However, the signet-ring cells subtype at this location is a very rare variant, with only 20 cases reported in the literature (1-9).


Case report

We report the case of a 78-year-old woman with painless jaundice of recent onset. The abdominal CT showed bile duct dilatation and, after the performance of EUS and ERCP with biopsy, an adenocarcinoma of the ampulla of Vater was diagnosed.

A cephalic pancreaticoduodenectomy (CPD) was then performed with Whipple reconstruction, with pancreatico-jejunal anastomosis made using polypropylene mesh following the technique described by Wang (10). The postoperative followup was uneventful. The resected specimen confirmed the presence of a mucosecretor adenocarcinoma with areas of signet-ring cells at the ampulla of Vater, with diffuse growth pattern of 1.1 cm in diameter infiltrating the wall to the muscle layer without pancreatic involvement. Only 1 of 21 lymph nodes had neoplastic infiltration. The patient received adjuvant chemotherapy with gemcitabine and after 14 months follow-up remains free of disease.



Signet ring cell adenocarcinoma is a rare variant of gastrointestinal adenocarcinoma, in which the intracellular mucin displaces the nucleus of tumor cells to the periphery. The stomach is the most frequent localization (90%), with generally more aggressive behavior and worse prognosis associated. Other locations of this disease include the breast, gallbladder, pancreas or colon. The development of signet ring cells carcinoma at the level of the ampulla of Vater is truly exceptional. Since the first description made in 1979 by Sekoguchi (1), the cases reported in the literature are limited to 20 (Table I).

Surgical resection is the only curative treatment, being the pancreatoduodenectomy or the ampullectomy the most common options. There are several factors to consider before the choice of treatment, mainly the tumor stage and the surgical and anesthetic risk. Some authors advocate in all cases for the performance of a CPD because of the risk of lymph node spread that even in T1 stage can reach up to 10%.

Because of the few cases described of this tumor it can not be defined if it is associated with more aggressive behavior, which would be a factor to take into account to perform a CPD resection. In the 20 cases reported to date, only 1 of them was treated by ampullectomy with good results, although with a short follow-up period of only 12 months (3).

Several prospective studies have shown an improved survival in patients resected with curative criteria, which are treated with adjuvant chemotherapy, usually employing gemcitabine and 5-fluorouracil.


Lucía Lesquereux-Martínez, Aquilino Fernández-Pérez and Manuel Bustamante-Montalvo
Department of Gereral Surgery and Digestive Diseases.
Hospital Clínico Universitario de Santiago de Compostela, A Coruña. Spain



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2. Arnal Monreal FM, Lorenzo Patiño MJ, Sacristán F, Ghanimé Saide G. Carcinoma de celulas en anillo de sello de ampolla de Vater. Rev Esp Enferm Dig 1994;85:391-3.         [ Links ]

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9. Burgos García A, Martín Arranz E, Rey Sanz R, Marín Serrano E, Martín Arranz MD, González Sanz-Agero P, et al. Carcinoma de células en anillo de sello de la ampolla de Vater. Gastroenterol Hepatol 2011; 34:141-6.         [ Links ]

10. Wang X, Zhou W, Xin Y, Huang D, Mou Y, Cai X. A new technique of polypropylene mesh-reinforced pancreaticojejunostomy. Am J Surg 2007;194:413-5.         [ Links ]

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