Intrahepatic sarcomatoid cholangiocarcinoma: A case report of the youngest patient on record and a review of the condition's characteristics

Abstract

As a rare disease, intrahepatic sarcomatoid cholangiocarcinoma (s-CCC) represents less than 1% of malignancies of the hepatobiliary system and its main clinical symptoms include abdominal pain and fever. Results of pathological examinations, despite being the “gold standard”, can easily be confused with hepatocellular carcinoma (HCC). This report is about a 32-year-old male patient who was hospitalized due to occupancy of segment V of the liver for three days and had a history of chronic hepatitis B (CHB) over a 20-year span. Magnetic resonance imaging (MRI) showed a 43 mm × 52 mm-sized liver mass in the V segment, with patchy peripheral enhancement during the arterial phase and rapid wash-out during the portal and late phases. A laparoscopic hepatectomy of segment V, along with cholecystectomy, was performed. Histopathological and immunohistochemical examinations indicated a malignant neoplasm that was positive for vimentin and cytokeratin, with these features providing a positive diagnosis for intrahepatic sarcomatoid cholangiocarcinoma. After surgery, an adjuvant therapy of albumin-paclitaxel combined with gemcitabine regimen was given. No recurrence was found six months after the surgery, with follow-up still ongoing. This report aims to improve the awareness, diagnosis, and treatment of s-CCC.

Sarcomatoid Intrahepatic Cholangiocarcinoma: A Rare and Aggressive Primary Liver Cancer

“…The median survival time of patients with S-iCCA with and without surgery is 11 months and three months, respectively [10]. While adjuvant chemotherapy, especially gemcitabine, and cisplatin, may prolong survival, its effectiveness remains unclear [1,2,[4][5][6]. Due to the rarity of the condition, there is not enough literature on immunohistochemical or histopathological factors affecting survival time.…”

“…Sarcomatous change of primary liver tumors is mostly found in hepatocellular carcinoma, particularly following chemotherapy, radiofrequency ablation, transarterial chemoembolization, and percutaneous ethanol injection. However, sarcomatoid changes in cholangiocarcinoma, referred to as S-iCCA, are an extremely rare variant with unclear pathophysiology [ 1 ]. Per the World Health Organization (WHO) classification, S-iCCA is a cholangiocarcinoma with spindle cells that either resembles spindle cell sarcomas/fibrosarcomas or has features of a malignant fibrous histiocytoma [ 5 ].…”

“…Risk factors are not well-established due to the rarity of the condition and limited research but are potentially age above 40 years, chronic hepatitis B, chronic hepatitis C, and liver cirrhosis [ 1 , 3 ]. While our patient was older than 40 years, he did not exhibit the latter risk factors.…”

“…Sarcomatoid intrahepatic cholangiocarcinoma (S-iCCA) is one of the rarest variants of all primary liver cancers, accounting for less than 1% of cases [ 1 ]. The cells of the sarcomatoid component grow rapidly and frequently metastasize, contributing to the characteristic poor prognosis of the disease.…”

Системна хiмiотерапiя при нерезектабельнiй формi внутрiшньопечiнкової саркоматоїдної холангiокарциноми: клiнiчний випадок та огляд лiтератури

“…У випадку сВХК серологiчнi маркери, характернi для ВХК, втрачають значення. Так, у вивчених нами джерелах описано вiдсутнiсть елевацiї АФП, СА 19-9, РЕА та PIVKA-II у бiльшостi пацiєнтiв із сВХК [2,6,10,11], що збігається з результатами обстеження нашої пацiєнтки. Бiльше того, данi радiологiчних методiв візуалізації також не є специфiчними при сВХК.…”