Signet-Ring Cell Carcinoma of the Ampulla of Vater: Report of a Case

Abstract

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.

Signet Ring Cell Carcinoma of the Ampulla of Vater with Early Development of Bone Metastasis: Case Report and Review of the Rare Malignancy

“…In case of recurrence, survival appears to be limited to median time of 12 months [4,18,19]. Our case, 35th in number, adds to the very limited number of case reports of the ampullary signet ring cells carcinomas.…”

“…Overall survival among all reported patients with pure signet ring carcinoma was 24.9 months (range 6-132 months). About 30 % of reported cases had no signs of recurrence when followed up at 1 year [4]. Pattern of recurrence appears to be variable, with distant metastasis scarcely reported in the literature as a first presentation.…”

“…Distant metastases appear to be associated with a short life expectancy after surgery. Acharya [3] 78/F T3N0M0 Pancreatoduodenectomy -Alive at 6 months Akatsu [4] 43/F T2N0M0 Pancreatoduodenectomy -Alive at 7.5 years Bloomston [5] 58/F T2N0M0 Pancreatoduodenectomy -Alive at 11 years Dauodi [6] 55/M T3N0M0 Pancreatoduodenectomy Chemotherapy Alive at 8 months Erquchi [7] 83/M T3N0M0 Pancreatoduodenectomy -Alive at 18 months Fang [8] 53/M T2N0M0 Pancreatoduodenectomy -Alive at 2 years Gao [9] 38/F T3N0M0 Pancreatoduodenectomy -Alive at 6 months Garcia [10] 73/M T2N1M0 Pancreatoduodenectomy -Alive at 14 months Garcia [10] 74/M T3N0MX Total pancreatectomy -Alive at 3 months Gardner [2] 69/F T3N0M0 Pancreatoduodenectomy -Unknown Gheza [11] 66/M Unknown Pancreatoduodenectomy -Alive at 8 months Hara [12] 68/M T2N0M0 Pancreatoduodenectomy -Alive at 10 months Ishibashi [13] 59/M T3N0M0 Pancreatoduodenectomy -Alive at 1.5 years Kim [14] 4 7 / M T X N 1 M X -Chemotherapy Died at 15 months Lesquereux-Martinez [15] 78/F TXN1M0 Pancreatoduodenectomy Chemotherapy Alive at 14 months Li [16] 56/F T2N1M0 Pancreatoduodenectomy -Alive at 12 months Maekawa [17] 75/M T3N0M0 Pancreatoduodenectomy -Died at 6 months Nabeshima [18] 49/M T3NXM1 -Chemotherapy Died at 12 months Paplomata [19] 45/F T4N1MX Pancreatoduodenectomy Chemotherapy Died at 12 months Purohit [20] 32/F TXNXM1 Declined Declined Unknown Ramia [21] 67/F T2N0M0 Pancreatoduodenectomy -Alive at 12 months Sekoguchi [22] 47/M T3N0M0 --Unknown Tas [23] 4 0 / M T X N 1 M 1 --Unknown Terada [24] 74/F TXNXM0 Biliary stent Chemotherapy Died at 14 months Tseng [25] 47/M T3N0M0 Pancreatoduodenectomy -Alive at 6 months Valeri [26] 66/M Unknown Pancreatoduodenectomy -Alive at 6 months Wen [27] 40/F T3N0M0 Pancreatoduodenectomy Chemotherapy Alive at 8 months Wen [27] 64/F T4NXM0 Pancreatoduodenectomy -Alive at 6 years Wen [27] 75/F T4NXM0 Pancreatoduodenectomy Chemoradiation Died at 16 months Wen [...…”

Signet-ring cell carcinoma of ampulla of Vater: Contrast-enhanced ultrasound findings

“…Less than 15 cases have been described in the literature. Akatsu [4] has summarized the previous 14 cases (eight men and six women) and concluded that the median age at diagnosis was 57 years (range, 32-83 years), approximately 15 years older than SRCC of the stomach, but similar to the median age for SRCC of the large bowel. Our case was a 38-year-old woman.…”

“…However, it is unclear whether the prognosis of a patient with SRCC is worse than that of patients with ordinary carcinoma occurring in the ampulla of Vater, because of the small number of cases so far reported. A patient has been reported to survive for 7.5 years after radical resection, and the author has suggested that long-term survival is possible in ampullary SRCC without nodal involvement [4] . A 58-year-old patient lived for 134 mo after resection and had no evidence of recurrence [7] .…”