Painless Jaundice Leading to Rare Entity of Signet Ring Cell Carcinoma in Ampulla of Vater

Abstract

65 year old female comes in with painless jaundice and weight loss of greater than 40 lbs in the past year, Patient found to have signet ring carcinoma of the stomach with metastasis to the ampulla of vater. SRCC in the ampulla of vater is a rare entity.1 While the incidence of gastric cancer is decreasing, the incidence of signet ring cell carcinoma (SRCC) is only increasing with about a 10-fold increase from 1970 to 2000.2 65 yo F with PMH of HTN, DM, non-ischemic cardiomyopathy s/p AICD 2013 (EF 45% 9/2015), CVA with expressive aphasia, cervical cancer s/p hysterectomy admitted for painless jaundice (total bili of 21) and unintentional weight loss (about 40lbs within the past year). CT abd/pelvis w/out contrast which showed intrahepatic and extrahepatic biliary ductal dilatation. EUS was significant for wall thickening in the entire body and antrum of the stomach with wall thickness measuring 14mm in the antrum and 19mm in the body; impression consistent with Linitis plastica or infiltrating gastric cancer. EUS guided FNA showed signet ring carcinoma of stomach with local metastasis to ampulla of vater. ERCP unable to be performed due to edematous mucosa; had percutaneous biliary drain placed through IR. Bili trended downward after placement of drain. She had surveillance CT chest and abdomen/pelvis w/ contrast for further staging (negative for any metastasis or lymphadenopathy). Patient was not a candidate for chemotherapy/RT/surgery. Only 26 cases previously have been described with SRCC in the ampulla of vater.1 Prognosis in early gastric cancer is better in SRCC compared to non SRCC gastric carcinomas while prognosis is less clear between SRCC and non-SRCC gastric cancers in advanced gastric cancer.2 Prognosis with SRCC in the ampulla is even less clear, with >5 year survival reported in only 4/26 cases.1 Our patient was advanced gastric cancer of SRCC and given metastasis and poor functional status was not a candidate for further therapeutic measures.2