Multicystic biliary hamartoma: A report of a rare entity and a review of the literature

Abstract

INTRODUCTIONMulticystic biliary hamartoma is a rare liver tumor that was first described in 2005. Only nine cases are reported in the literature and all of them originate from Eastern patient populations, specifically Japan and Korea.PRESENTATION OF CASEHerein we report the occurrence of the tenth multicystic biliary hamartoma reported to date, arising in a Caucasian American woman initially presenting with abdominal pain. At 4.7 cm this is the second largest tumor reported to date and the only one arising in a Western patient population.DISCUSSIONThe patient underwent multimodality imaging and the tumor was biopsied preoperatively, but the diagnosis remained unclear. An extended right hepatectomy was performed for resection of her tumor, and the tumor was definitively diagnosed based on the surgically resected specimen. As all nine of the previously reported cases also underwent resection, the natural history of this lesion remains unknown. The lack of both recurrence and tumor spread in the previously reported cases indicates that this may be a benign lesion not requiring surgical resection unless symptomatic.CONCLUSIONMulticystic biliary hamartoma is an extremely rare tumor. Increased awareness of the radiologic and pathologic features will likely lead to the diagnoses of further cases in both Western and Eastern populations and could potentially assist with preoperative diagnosis. The natural history and optimal management of this tumor remain uncertain.

A case of multicystic biliary hamartoma with a marked peribiliary gland component successfully treated by purely laparoscopic anatomical liver resection

“…The clinical diagnosis of MCBH is challenging and differential diagnosis from MCN, IPNB, BAF are often a matter of serious debate [3,5,11]. In the present case, IPNB could be ruled out by the ndings of ERC.…”

“…Multicystic biliary hamartoma (MCBH) is an extremely rare benign liver lesion which was initially de ned by Zen et al in 2006. [1] Since then, only fteen cases have been reported as MCBH in the English literature [1][2][3][4][5][6][7][8][9][10][11]. The typical characteristics of MCBH are a gross, well-circumscribed, multicystic honeycomb appearance and histological ndings of cystic or ductal structures composed of ductal epithelium, with various amounts of peribiliary (periductal) glands and brous connective tissue within the lesion [1.2].…”

Bile Duct Hamartoma Mimicking Liver Metastasis in Suspected Porcelain Gallbladder: a Case Report

“…[2,3] Solitary multicystic bile duct hamartoma are extremely rare and till 2014 only ten cases have been reported in the English literature. [4] We report a case of incidental solitary multicystic biliary hamartoma in a patient undergoing laparoscopic cholecystectomy for suspected porcelain gallbladder.…”

A Liver Full of Stars: Hepatostellular!

“…As in our case, these lesions are typically discovered incidentally. 1,2 Further histopathologic classification of biliary hamartomas exists as follows: class 1, predominately solid lesions with narrowing of bile channels; class 2, mixed solid and cystic lesions; and class 3, predominately cystic lesions with dilation of bile ducts. 1 The etiology of Von Meyenburg complexes is believed to be a congenital malformation owing to deficient remodeling of primitive ductal plate configuration.…”

“…The ductal plate malformation also causes other congenital disease, including congenital hepatic fibrosis, polycystic liver disease, and Caroli's disease. 1,2 Biliary hamartomas are many times mistaken as metastatic lesions. 3 The distinguishing features of metastatic liver disease include the presence of enhancing component, larger sizes of cysts/nodules, and more varied pattern of distribution.…”