Jornal de Citologia e Histologia

Carcinosarcoma of the Gallbladder Arising in a Patient with Pancreaticobiliary Maljunction: A Case Report and Review of the Literature

Abstract

Kirstin Coetzee, Jones Omoshoro-Jones and Pam Michelow

Background: Carcinosarcoma of the gallbladder is a very uncommon malignancy. We present a case of gallbladder carcinosarcoma arising in a 38-year-old female patient with pancreaticobiliary maljunction where FNA documented recurrence of this very unusual tumor. Case: A 38-year old woman presented with signs and symptoms of acute pancreatitis due to gallstones (biliary pancreatitis) Further investigations, including abdominal ultrasonography and endoscopic retrograde cholangiopancreatography (ERCP), revealed an ill-defined mass in the gallbladder, in addition to pancreaticobiliary maljunction. At laparoscopic cholecystectomy, a mass was found within the gallbladder lumen itself. A biphasic malignant neoplasm was diagnosed in a free lying, polypoid mass within the gallbladder lumen. This carcinosarcoma was considered to have been locally confined to the gallbladder, equivalent to a stage T1a, and further surgery was not undertaken. The patient re-presented under a year later with dyspnoea; and a large subdiaphragmatic mass was noted that underwent ultrasound-guided fine needle aspiration (FNA) biopsy. The cytomorphologic findings showed a tumor comprising malignant glandular structures and pleomorphic stromal cells in a metachromatic background, compatible with the previous diagnosis of carcinosarcoma. Conclusion: Carcinosarcomas are uncommon malignancies and can arise in almost any site where carcinomas arise. Careful attention to morphology will allow the biphasic nature of this lesion to be determined but ancillary investigations may be required. Carcinosarcoma is a biphasic malignant neoplasm with both malignant epithelial (carcinomatous) and stromal (sarcomatous) elements. Carcinosarcoma occurring in the gallbladder is a rare malignancy of which there have been fewer than seventy reports in the worldwide literature to date, with thirty two of these in the English literature [1-14]. We present a case of gallbladder carcinosarcoma in a young patient with pancreaticobiliary maljunction, treated with cholecystectomy, with subsequent local recurrence diagnosed on image-guided fine needle aspirate (FNA). There appears to be only one other reported case of FNA of a carcinosarcoma of the gallbladder in which an initial diagnosis of a high-grade spindle cell sarcoma was made cytologically.