Carcinosarcoma of the liver, malignant mixed tumour of the liver

Definition

Carcinoma of the liver is a rare malignant neoplasm comprising both carcinomatous and sarcomatous components. The carcinoma may be hepatocellular carcinoma or cholangiocarcinoma. The WHO definition does not require heterogeneous components within the sarcomatous tissue.

Epidemiology

This is a tumour of older adults, with a male predominance. It is not associated with alcohol abuse.

Macroscopic appearances

The cut surface of the tumour commonly shows necrosis or haemorrhage.

Histopathology

Both carcinomatous and sarcomatous components often account for over 30% of the tumour. There may be areas of transition between the two components. The carcinomatous component is either hepatocellular or cholangiocarcinomatous. The sarcomatous component is predominantly spindled or pleomorphic, negative for epithelial markers. There may be differentiation into cartilaginous, osseous or rhabdomyosarcomatous tissue.

Immunohistochemistry

	Carcinomatous component	Sarcomatous component
AE1/3	3/41	0/41
HepPar-1	3/41	0/41
AFP	1/31	1/41
CK7	1/11	0/11
CK8	1/11	0/11
CK18	2/21	0/21
Vimentin	0/51	5/51
Desmin	0/21	2/31
Myoglobin	0/11	0/21
CD31		0/11
CD34	0/11	0/21
CD68	0/21	2/21
CD117		0/11
HHF35	0/31	0/41
S-100	0/21	0/31
Factor VIIIRA	0/11	0/11
CEA	0/21	0/21
MAC387	0/11	0/11
alpha-1-antitrypsin	0/21	2/21

Differential diagnosis

Poorly differentiated spindle cell carcinoma: the spindle cells are positive for epithelial markers.

Prognosis

The prognosis is poor, with a high rate of extrahepatic metastases.

References

1 Lao XM, Chen DY, Zhang YQ, et al. Primary Carcinosarcoma of the Liver: Clinicopathologic Features of 5 Cases and a Review of the Literature. Am J Surg Pathol 2007; 31:817-826

This page last revised 22.6.2007.