P1107 - Diagnostic Challenges of Cholangiocarcinoma Presenting as an Ovarian Mass

Vihitha Thota, MD¹, Sudheer Konduru, MD¹, Sana Mulla, MD², O'Neil Chaudhry, MD³, Manaswitha Thota, BS⁴, Rajesh Thirumaran, MD, MRCP^2
1Mercy Catholic Medical Center, Lansdowne, PA; ²Mercy Catholic Medical Center, Darby, PA; ³Mercy Fitzgerald Hospital, Darby, PA; ⁴Virginia Commonwealth University School of Medicine, Ashburn, VA

Introduction: Cholangiocarcinoma (CCA) is a rare, highly lethal malignancy arising from intra or extrahepatic bile ducts. Metastatic disease to the ovary can be a diagnostic challenge as it mimics primary mucinous ovarian neoplasm and can be clinically apparent before diagnosis of the primary tumor. We present one such case of an ovarian tumor initially thought to be a primary ovarian carcinoma with workup later revealing CCA.

Case Description/Methods: A 64-year-old female with a history of hypertension presented with a several-week history of intermittent right upper quadrant abdominal pain associated with subjective weight loss and anorexia. Examination revealed mild right-sided abdominal tenderness with blood work showing transaminitis, direct hyperbilirubinemia, and elevated alkaline phosphatase. Computed tomography (CT) of abdomen/pelvis showed a large (76 x 73 x 59 mm) heterogeneous left adnexal lesion suspicious for ovarian carcinoma with metastasis to liver infiltrating >70% of liver volume and metastatic deposits in the peritoneum and gallbladder fossa. CA-125 was elevated with normal CA 19-9, CEA, and AFP. Liver biopsy showed poorly differentiated metastatic adenocarcinoma with tumor cells positive for CK7, CK20, CA-125 and negative for estrogen receptor, suggestive of ovarian primary. However, a few days later, further staining revealed tumor cells positive for CDX2 and CK-19, negative for WT-1, p63, and CA 19-9, favoring pancreaticobiliary origin. Given a poor prognosis due to extensive metastatic disease, the patient was transitioned to hospice care after a thorough multidisciplinary evaluation.

Discussion: Cholangiocarcinomas comprise < 3% of all malignancies, however are very aggressive, often presenting with distant metastasis at the time of diagnosis, as seen in our case, with a 5-year survival rate of 5-10%. While the most common areas of spread are confined to the abdomen, extra-abdominal spread can be seen in rare instances, including to the ovaries. This poses significant diagnostic challenges as both metastatic CCA and primary mucinous ovarian neoplasm present with vague abdominal symptoms and share many immunohistochemical markers, such as CK7 and CK20. Furthermore, the biomarkers for cholangiocarcinoma, including CA 19-9 and CEA, are non-specific and can be normal even in advanced disease. Therefore, clinicians need to consider CCA as part of the differential for ovarian tumors and further studies should be done to establish improved screening for CCA.


Disclosures:


Vihitha Thota, MD¹, Sudheer Konduru, MD¹, Sana Mulla, MD², O'Neil Chaudhry, MD³, Manaswitha Thota, BS⁴, Rajesh Thirumaran, MD, MRCP². P1107 - Diagnostic Challenges of Cholangiocarcinoma Presenting as an Ovarian Mass, ACG 2021 Annual Scientific Meeting Abstracts. Las Vegas, Nevada: American College of Gastroenterology.