Signet Ring Cell Carcinoma of the Extrahepatic Bile Duct Diagnosed by Preoperative Biopsy: A Case Report

Abstract

A 73-year-old woman was admitted because of obstructive jaundice. Computed tomography revealed a stricture in the lower bile duct with enhanced bile duct wall. Endoscopic retrograde cholangiopancreatography (ERCP) revealed a tapering stenosis at the lower bile duct. Transpapillary histological biopsy using biopsy forceps through ERCP was performed; the diagnosis of signet ring cell carcinoma (SRCC) of the bile duct was established. Regional lymph node enlargement and distant metastases were not detected on diagnostic imaging. Pancreaticoduodenectomy with pylorus preservation was performed. Histological examination of the resected specimen confirmed SRCC of the extrahepatic bile duct coexisting with adenocarcinoma (ADC) of the extrahepatic bile duct with negative resection margins. However, tumor cells directly invaded the pancreatic parenchyma and the muscle layer of the duodenum, prompting us to administer adjuvant chemotherapy to the patient, with no sign of tumor recurrence at 1-year follow-up. Almost all tumors originating from the extrahepatic bile duct are ADC and other histological variants are rare. Of these, SRCC is extremely rare and only four cases have been reported. Furthermore, to the best of our knowledge, this is the first case report regarding the preoperative diagnosis of SRCC of the bile duct. Current reports indicate that younger age and Asian ethnicity are the clinical features of SRCC of the extrahepatic bile duct. Immunohistochemical staining of CK7, CK20 and MUC2 may be useful for predicting prognosis. Chemotherapy has not resulted in increased survival rates and only surgical resection currently serves as a curative treatment.

Primary signet-ring cell carcinoma of the extrahepatic bile duct: A case report

“…In the current study, we included 3 males[ 7 , 10 , 12 ] and 8 females[ 8 , 9 , 11 , 13 - 17 ] with an average age of 58.5 years (range: 32-73 years). This phenomenon suggested that primary SRCC of the extrahepatic bile duct occurs in elderly patients, which was similar to previous reports[ 9 ].…”

“…However, due to the small number of patients, the correlation between the incidence of primary SRCC of the extrahepatic bile duct and gender needs to be investigated further. The analysis of 11 patients revealed that primary SRCC of the extrahepatic bile duct occurred in the distal bile duct in 8 cases[ 7 , 8 , 10 - 12 , 14 - 16 ] and the perihilar bile duct in only 3 cases[ 9 , 13 , 17 ]. This showed that primary SRCC of the extrahepatic bile duct occurs in the distal bile duct compared to the perihilar bile duct.…”

“…Also, integrative treatments that combine two or three have been applied[ 7 - 17 ]. 7/11 cases[ 8 - 10 , 12 - 14 , 16 ] received only surgical resection, 1/11 case[ 17 ] received chemotherapy alone, 1/11 case[ 11 ] received surgical resection followed by chemotherapy, 1/11 case[ 15 ] received chemoradiotherapy, and 1/11 case[ 7 ] received combined treatment of surgical resection, radiotherapy, and chemotherapy. Currently, surgical treatment is the gold standard for patients with cancer without distant metastasis.…”

Rare histotypes of epithelial biliary tract tumors: A literature review

“…Otherwise, it is named "adenocarcinomas with a signet ring cell component" (Benesch and Mathieson, 2020). Based on immunohistochemical characteristics, there are two types of SRCCs: a) intestinal type (CK7 negative and CK20/MUC2 positive) with better prognosis, and b) pancreatic-biliary type (CK7 positive and CK20/MUC2 negative) with worse prognosis (Kita et al, 2014;Roh et al, 2007).…”

Untitled

“…Signet-ring-cell (SRC) Cholangiocarcinoma is an extremely rare variant, with only 11 cases reported to date [3][4][5][6][7][8][9][10][11][12][13]. All cases describe a localized mass, usually in the extrahepatic biliary tract.…”