Primary carcinosarcoma of the liver: imaging features and clinical findings in six cases and a review of the literature

Abstract

BackgroundCarcinosarcoma of the liver is a very rare tumor composed of a mixture of carcinomatous and sarcomatous elements. Less than 25 adequately documented cases have been reported, with inadequate description of imaging features. In order to improve the awareness of this rare tumor, this study aimed to analyze the clinicopathologic and imaging features of six cases of hepatic carcinosarcoma (HCS) confirmed by surgical pathologic evaluation.MethodsWe retrospectively studied the clinicopathologic and imaging features of six cases of HCS (matching the World Health Organization definition) and discussed the differential diagnosis on the basis of imaging findings. The patients, including five men and one woman, were 38 to 69 years of age. Five patients underwent CT scans, one underwent MRI scans.ResultsWhile 3 patients were positive for hepatitis-B surface antigen, 2 had cirrhosis. The largest tumor diameter ranged from 5.0 to 21.0 cm. Satellite nodules, venous thrombi, and organ invasion (gastric wall, gallbladder, and right adrenal gland) were identified. Pathologically, the carcinomatous components corresponded to hepatocellular carcinoma in three cases, cholangiocellular carcinoma in one case, and adenocarcinoma in two cases. The sarcomatous components exhibited complex features, with undifferentiated spindle cells in five cases and a leiomyosarcoma in one. All tumors showed heterogeneous density/intensity with extensive cystic change and necrosis; spot calcification was observed in one case. Capsule was not identified. While four tumors showed heterogeneous hypervascular enhancement, two showed hypovascular enhancement. All patients underwent surgical resection. The follow-up period ranged from 2 to 18 months. Four patients died from recurrence and metastasis.ConclusionsThe clinical and imaging features of HCS are heterogeneous. Due to the heterogenous nature and very low morbidity of HCS, combination of careful analysis of imaging findings and clinical features might be useful for a more accurate diagnosis of HCS.

Clinical Manifestations and Outcomes of Patients with Sarcomatoid Hepatocellular Carcinoma

“…Although sarcomatoid carcinoma and carcinosarcoma are synonymously used, sarcomatoid carcinoma is preferred for spindle cells with remaining epithelial differentiation confirmed through immunohistochemical staining‐positive epithelial markers, such as CK, and no specific type of mesenchymal lineage, whereas carcinosarcoma is preferred for spindle cells with specific mesenchymal differentiation . On the basis of the fourth edition of the WHO classification of tumors, carcinosarcoma is currently understood as a carcinoma that has undergone sarcoma‐like differentiation . Similar to sarcomatoid carcinoma, the sarcomatoid component of carcinosarcoma is considered to be a clonal evolution of the differentiated component (HCC or cholangiocarcinoma) .…”

“…(27) On the basis of the fourth edition of the WHO classification of tumors, carcinosarcoma is currently understood as a carcinoma that has undergone sarcoma-like differentiation. (28,29) Similar to sarcomatoid carcinoma, the sarcomatoid component of carcinosarcoma is considered to be a clonal evolution of the differentiated component (HCC or cholangiocarcinoma). (10,30) Additionally, in a comparative study between sarcomatoid carcinoma and carcinosarcoma, no significant difference was noted between these two types of biphasic tumors for several clinicopathological characteristics.…”

Management and outcomes among patients with sarcomatoid hepatocellular carcinoma: A population‐based analysis

“…3,7,8 Due to its rarity, there have been limited data regarding the outcomes of patients with sarcomatoid HCC, with those few studies focusing on the radiologic or histopathologic features of the disease. [9][10][11][12] For example, although Jernigan et al reported on 20 cases of patients with sarcomatoid HCC, the sample size was too small to assess the long-term outcomes of these patients compared with individuals with conventional HCC. 13 As such, the objective of the current study was to provide a comprehensive assessment of the incidence, treatment, and prognosis of patients with sarcomatoid HCC identified in the National Cancer Data Base (NCDB).…”

Imaging features of ultrasound and contrast-enhanced ultrasound in primary hepatic carcinosarcoma: three cases report

“…Previous published papers mostly reviewed the pathogenesis and clinical characteristics [2][3][4][5] or described the imaging features in computed tomography (CT), magnetic resonance imaging (MRI) or 18 F-fluorodeoxyglucose positron emission tomography (PET) of PHCS [6][7][8]. Little is known about ultrasound (US) and contrast-enhanced ultrasound (CEUS) [9].…”