Clinical Challenges

Clinical Challenges - November 2017 What's your diagnosis?

Publish date: October 27, 2017

By Ryan Law, MD, Thomas C. Smyrk, and Stephen C. Hauser. Published previously in Gastroenterology (2013;144[3]:508, 658).

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A 43-year-old woman presented with progressively worsening right upper-quadrant abdominal pain. The episodic pain occurred after high-fat meals and lasted from minutes to hours with accompanying nausea. Her previous medical history was notable for endometriosis. She denied other constitutional symptoms. Physical examination revealed no hepatosplenomegaly, jaundice, right upper-quadrant mass, or stigmata of chronic liver disease.

Initial laboratory evaluation yielded normal white blood cell count and liver chemistries. Ultrasonography, computed tomography, and magnetic resonance imaging of the abdomen all demonstrated a 2.0 × 4.1 × 3.9-cm, nonenhancing, elongated, cystic mass located superior to the gallbladder within the porta hepatis, with possible communication at the bile duct confluence and abutment of the right portal vein (Figure A). No definitive findings of acute cholecystitis were present.

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Endoscopic retrograde cholangiopancreatography with endoscopic ultrasonography was performed to further delineate the anatomy of the lesion. On endoscopic ultrasonography, the structure in question seemed to be embedded in the hepatic parenchyma with partial extension beyond the liver edge. Adherent debris was noted within the cystic structure. No lymphadenopathy was present. Cholangiography demonstrated filling of the lesion from a central right intrahepatic duct (Figure B). Attempts at cannulation of the cyst were unsuccessful.

The patient subsequently developed abnormal liver chemistries with continued right upper-quadrant pain. She was referred to an experienced hepatobiliary surgeon and underwent operative intervention. What is the diagnosis and how would you treat this patient?


 

The diagnosis


Answer: Hepatic foregut duplication cyst and concurrent acute gangrenous cholecystitis


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Our patient underwent operative intervention for cholecystitis; the surgical specimen showed acute cholecystitis with necrosis. Surgical enucleation of the unilocular cyst was also performed. No communication to the gallbladder was identified. Histologically, three distinct layers were noted (mucosa, submucosa, and muscularis propria), consistent with a foregut duplication cyst (Figure C). The muscularis propria contained a rudimentary myenteric plexus, including ganglion cells, identified on S100 staining (Figure D, upper right; higher power, Figure E). The cyst was lined by multilayered cuboidal epithelium with cilia, beneath which there were scattered, nondescript glands.


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Two examples of foregut duplication cyst within the liver have been described in the literature, one representing duplicated duodenum1 and one ileum.2 The classic histologic features of foregut duplications include 1) well-developed smooth muscle layers, including muscularis mucosa and muscularis propria, 2) an epithelial lining that may be gastric, intestinal, or respiratory type, and 3) contiguity to the foregut segment that is duplicated.


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Our differential diagnosis also included ciliated hepatic foregut cyst; however, that entity should have ciliated epithelium surrounded by disorganized bundles of smooth muscle and a dense fibrous outer capsule.3 The imaging findings also raised consideration of other possible etiologies, including intrahepatic biliary mucinous cystadenoma, gallbladder duplication, and type II choledochal cyst. Typical findings of these lesions were not identified. Malignancy has reportedly arisen from all of these various lesions; therefore, surgical excision was indicated given the concern over etiology.

References


1. Imamoglu K.H., Walt, A.J. Duplication of the duodenum extending into liver. Am J Surg. 1977;133:628-32.
2. Seidman J.D., Yale-Loehr A.J., Beaver B., et al. Alimentary duplication presenting as an hepatic cyst in a neonate. Am J Surg Pathol. 1991;15:695-8.
3. Vick D.J., Goodman Z.D., Deavers M.T., et al. Ciliated hepatic foregut cyst: A study of six cases and review of the literature. Am J Surg Pathol. 1999;23:671-7.

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