Case Presentation:
A 69‐year‐old white female presented with a 5‐week history of fatigue, myalgias, low‐grade fevers, increasing abdominal girth, concurrent hearing loss with bilateral serous otitis media diagnosed 3 days prior by otolaryngology, and 10 days of increasingly blurry vision. Hospital internal medicine admitted her for further evaluation of symptoms and abnormal laboratory values. Physical exam showed normal vital signs with the patient in no acute distress, no jaundice, and slight abdominal discomfort. Further analysis revealed AST 409, AST 637, alkaline phosphatase 691, total bilirubin 1.5, direct bilirubin 0.9. low‐grade disseminated intravascular coagulation, fibrinogen 402 (220–400) and ferritin 1026. CT of the abdomen revealed an infiltrating mass causing intrahepatic ductal dilation. Further investigations including a vasculitis panel, hepatitis serologies, CEA and alpha‐feto protein were within normal limits. Abdominal MR confirmed an infiltrative lobuleted mass near the biliary confluence extending along the intrahepatic bile ducts suspicious for cholangiocarcinoma. An endoscopic retrograde cholangiogram (ERC) followed, and a stent was placed; however, cytology from biliary duct brushings was nondiagnostic. A retinal specialist was consulted because of her deterioration in vision, who found counting fingers vision in each eye, clear media with no evidence of anterior or posterior inflammation, and bilateral serous retinal detachments with shifting fluid. Of note, no intraocular tumors were noted in either eye. The concurrence of bilateral serous retinal detachments and serous otitis media pointed to a likely paraneoplastic process, and both the otolaryngology and the retina service suggested a course of high‐dose oral steroids Her vision improved with high‐dose steroids. A follow‐up ERC for worsening jaundice demonstrated a tight hilar stricture with obstruction of the right and left main ducts and splaying of central ducts suggesting an exophytic mass effect. Cytology revealed adenocarcinoma of an upper gastrointestinal Tract as the primary.
Discussion:
This is the first reported case of cholangiocarcinoma presenting with a paraneoplastic process of bilateral retinal detachments. Cholangiocarcinoma is not commonly associated with a specific paraneoplastic syndrome, but case reports linking it to vasculitis, rare skin disorders, and hypercalcemia exist. This case highlights an uncommon presentation of this primary liver tumor in addition to the classic findings of painless jaundice, fevers, pruritis, abdominal pain, and weight loss.
Conclusions:
We report the only case of paraneoplastic bilateral retinal detachments and bilateral serous otitis effusions in a patient with cholangiocarcinoma.
Author Disclosure:
W. Tong, none; M. Kidd, none; E. Carey, none; K. Jamai, none; J. Charles, none.