Signet-ring cell carcinoma of the ampulla of Vater: report of a case.

Signet-ring cell carcinoma (SRCC) of the ampulla of Vater is uncommon, and only 13 cases have been previously described. We herein report a rare case of a 43-year-old woman with SRCC in the ampulla of Vater. Although this histologic type of cancer generally predicts a poor prognosis elsewhere in the gastrointestinal tract, the present patient has survived with no evidence of recurrence for 7.5 years. The patient was hospitalized for pruritus and jaundice. She underwent a pancreaticoduodenectomy with an extended lymphadenectomy for ampullary carcinoma. This neoplasm was classified as AcdbBi, 2.0 x 1.8 cm, ulcerative type, Panc(0), Du(2), T(2)N(0)M(-), Stage II according to the Japanese Classification on Cancer of the Biliary Tract. Unfortunately, previous reports of ampullary SRCC have been limited to short-term follow-ups with a median period of only 12 months (range, 6-134 months), and long-term survival (more than 5 years) was only documented in two cases without lymph node metastasis (including the present case). In conclusion, the experience gained in the present and previous cases suggests that long-term survival is possible following a curative resection in selected patients with ampullary SRCC without nodal involvement. Due to the lack of sufficient evidence, additional reports are warranted to determine whether SRCC also portends a poor prognosis in patients with ampullary cancer.