Case Presentation: A 35-year-old female dog groomer with a history of an ovarian cyst presented as a direct admission from the Gastroenterology clinic due to worsening upper abdominal pain and weight loss for 2 months. An abdominal CT obtained two weeks prior showed a large left liver lobe mass measuring 6.3 x 4.6 cm. On admission, further history obtained was notable for ovarian cancer in her mother, uterine cancer in her grandmother, and colon cancer in her grandfather. Initial labs were significant for an alkaline phosphatase of 208, carbohydrate antigen 19-9 (CA 19-9) of 4862, and a normal white blood cell count. An MRI Liver was obtained that showed multiple cystic-appearing masses in the left lobe with the largest measuring 3.8 x 5.0 x 3.4 cm. A subsequent percutaneous aspiration and biopsy was performed with the final pathology consistent with intrahepatic cholangiocarcinoma (IHC). CT chest, abdomen, and pelvis showed no metastatic disease. EGD and colonoscopy were also negative. The patient remained afebrile during the admission with improved control of her symptoms. She was discharged with plans for follow-up in the Oncology clinic in another state for permanent care due to having more family support in that area.

Discussion: Based on our patient’s initial presentation, family history, and imaging findings, pyogenic liver abscess and malignancy were highest on our differential. Typically, a bacterial pyogenic abscess presents with right upper quadrant pain, fever, hyperbilirubinemia, and transaminitis (1,2). Moreover, her occupation as a dog groomer made us suspicious of cystic echinococcosis as in the early stages of disease patients are more asymptomatic (3). Malignancy was also considered, given her extensive family history. Final pathology of the liver mass was diagnostic for IHC, which has an incidence 0.001% in the US (4). Early IHC is often asymptomatic, so they often present later in advanced stages with obstructive symptoms including jaundice, pale stools, and dark-colored urine (5). The CA 19-9 tumor marker can be supportive of a diagnosis, but tissue biopsy is definitive (6). Of particular interest in this case was the patient’s young age and extensive family history of cancer. Although it cannot be confirmed due to lack of records, the patient’s family history is concerning for Lynch syndrome via the revised Bethesda guidelines. IHC is rare among the extracolorectal cancers for Lynch syndrome (0.02 – 4% risk) with a mean age of diagnosis of 54 – 57 years (7).

Conclusions: Our case is noteworthy for the patient’s young age, employment history, family history, and relatively acute presentation for IHC in the setting of possible Lynch syndrome. These factors combined to form an interesting differential diagnosis and work-up of pyogenic liver abscess, cystic echinococcus, and malignancy.

IMAGE 1: MR liver