WEO E-Newsletter Issue 21, 2012: Brainteaser/image of the month
The image shown in Figure 1 is an MRI scan of the liver performed on a 23 year old woman with no medical or surgical history who developed jaundice and itching over a weekend. The biliary abnormality seen in the image, and her jaundice and itching, completely resolved after a course of oral Prednisone, starting at 40mg daily and de-escalating by 10mg weekly until it was stopped. The patient was then asymptomatic for 2 months, when her jaundice and itching returned. A further MRI scan showed that the previous abnormality had returned. The jaundice and itching responded promptly to re-introduction of steroids, but started to relapse on 2.5 mg daily. She has remained asymptomatic on a daily dose of 5mg Prednisone.
What is the likeliest diagnosis of this young woman's biliary abnormality?
- Hilar cholangiocarcinoma (Klatskin tumor)
- Primary sclerosing cholangitis (PSC)
- Inflammatory pseudotumor of the liver
- Post-surgical ischemic stricture
The correct answer is C – Inflammatory pseudotumor of the liver. The MRI shows a mass in the vicinity of the hilum of liver, causing stricturing of the central bile ducts with some peripheral dilatation. This is a rare condition characterized by a dense strands of fibrosis within the liver, infiltrated by mainly plasma cells. The bile duct abnormality can be indistinguishable from hilar cholangiocarcinoma (Klatsin tumor). However, cholangiocarcinoma would be exceeding rare in a 23 year old. As this patient had no prior medical or surgical history, she was not a candidate for post-surgical ischemic injury involving the bile ducts. Primary sclerosing cholangitis (PSC) would be unlikely to respond so spectacularly to steroid therapy, and the patient had no history of inflammatory bowel disease.
There is no clear consensus regarding management of inflammatory pseudotumor of the liver: resolution with steroids has been reported, as has spontaneous regression, and some surgeons have reported "cure" by major hepatic resection. As evidenced by our patient's remission on steroids and relapse without, there is likely an inflammatory - and possibly an autoimmune - basis to this disorder. In this case, we resisted the considerable temptation to perform ERCP and place a biliary stent to improve drainage. In retrospect, the patient's prompt response to steroid therapy rendered this unnecessary.
Deng FT, Li YX, Ye L Hilar inflammatory pseudotumor mimicking hilar cholangiocarcinoma. Hepatobiliary Pancreat Dis Int 2010;9(2):219-212
Milias K. Madhavan KK, Bellamy C, et al. Inflammatory pseuodtumor of the liver:: experience of a specialist surgical unit. J Gastroenterol Hepatol 2009;24(9):1562-1566
Yan FH, Zhou KR, Jiang YP, et al. Inflammatory pseudotumor of the liver: 13 cases of MRI findings. World J Gastroenterol 2001;7(3):422-424.