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Acute liver failure due to primary angiosarcoma: A case report and review of literature

Bhati CS, Bhatt AN, Starkey G, Hubscher SG, Bramhall SR - World J Surg Oncol (2008)

Bottom Line: Acute liver failure is an extremely rare presentation of a primary liver tumour.We report a case of a seventy year-old man who presented with a very short period of jaundice leading to fulminant hepatic failure (FHF).On further investigation he was found to have primary angiosarcoma of liver.

Affiliation: Liver Unit, Queen Elizabeth Hospital, Birmingham, UK. c.s.bhati@bham.ac.uk

ABSTRACT

Background: Hepatic angiosarcoma is a primary sarcoma of the liver, accounting for only 2% of all primary hepatic malignancies. Acute liver failure is an extremely rare presentation of a primary liver tumour.

Case presentation: We report a case of a seventy year-old man who presented with a very short period of jaundice leading to fulminant hepatic failure (FHF). On further investigation he was found to have primary angiosarcoma of liver.

Conclusion: The treatment outcomes for hepatic angiosarcoma are poor, we discuss the options available and the need for prompt investigation and establishment of a diagnosis.

(A) Liver biopsy showing sinusoidal infiltration by pleomorphic spindle cells typical of hepatic angiosarcoma. There is disruption of the normal trabecular architecture with hepatocyes forming glandular structures containing bile plugs ("cholestatic rosettes"). (B) Spindle cells are strongly immunoreactive for the vascular endothelial marker CD34. (A = Haematoxylin and eosin, B = immunoperoxidase).
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Figure 2: (A) Liver biopsy showing sinusoidal infiltration by pleomorphic spindle cells typical of hepatic angiosarcoma. There is disruption of the normal trabecular architecture with hepatocyes forming glandular structures containing bile plugs ("cholestatic rosettes"). (B) Spindle cells are strongly immunoreactive for the vascular endothelial marker CD34. (A = Haematoxylin and eosin, B = immunoperoxidase).

Mentions: A post mortem liver biopsy was carried out confirming initial suspicions that this was a primary angiosarcoma of the liver. Microscopically, tumour was composed of poorly cohesive cells, oval to spindle shaped with high grade cytological atypia. The tumour had a sinusoidal growth pattern surrounding clusters of hepatocytes forming cholestatic rosettes (Figure 2a). Immunohistochemistry staining was strongly and diffusely positive for vascular endothelial markers (CD31, CD34) (Figure 2b) and for vimentin. Stains for the cytokeratins and hepatocyte specific antigen highlighted the presence of entrapped non neoplastic hepatocyte and bile ducts. Staining for smooth muscle actin appeared to be confined to areas of fibrotic tissue.

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Acute liver failure due to primary angiosarcoma: A case report and review of literature

Bhati CS, Bhatt AN, Starkey G, Hubscher SG, Bramhall SR - World J Surg Oncol (2008)

(A) Liver biopsy showing sinusoidal infiltration by pleomorphic spindle cells typical of hepatic angiosarcoma. There is disruption of the normal trabecular architecture with hepatocyes forming glandular structures containing bile plugs ("cholestatic rosettes"). (B) Spindle cells are strongly immunoreactive for the vascular endothelial marker CD34. (A = Haematoxylin and eosin, B = immunoperoxidase).
© Copyright Policy - open-access
Figure 2: (A) Liver biopsy showing sinusoidal infiltration by pleomorphic spindle cells typical of hepatic angiosarcoma. There is disruption of the normal trabecular architecture with hepatocyes forming glandular structures containing bile plugs ("cholestatic rosettes"). (B) Spindle cells are strongly immunoreactive for the vascular endothelial marker CD34. (A = Haematoxylin and eosin, B = immunoperoxidase).
Mentions: A post mortem liver biopsy was carried out confirming initial suspicions that this was a primary angiosarcoma of the liver. Microscopically, tumour was composed of poorly cohesive cells, oval to spindle shaped with high grade cytological atypia. The tumour had a sinusoidal growth pattern surrounding clusters of hepatocytes forming cholestatic rosettes (Figure 2a). Immunohistochemistry staining was strongly and diffusely positive for vascular endothelial markers (CD31, CD34) (Figure 2b) and for vimentin. Stains for the cytokeratins and hepatocyte specific antigen highlighted the presence of entrapped non neoplastic hepatocyte and bile ducts. Staining for smooth muscle actin appeared to be confined to areas of fibrotic tissue.

Bottom Line: Acute liver failure is an extremely rare presentation of a primary liver tumour.We report a case of a seventy year-old man who presented with a very short period of jaundice leading to fulminant hepatic failure (FHF).On further investigation he was found to have primary angiosarcoma of liver.

Affiliation: Liver Unit, Queen Elizabeth Hospital, Birmingham, UK. c.s.bhati@bham.ac.uk

ABSTRACT

Background: Hepatic angiosarcoma is a primary sarcoma of the liver, accounting for only 2% of all primary hepatic malignancies. Acute liver failure is an extremely rare presentation of a primary liver tumour.

Case presentation: We report a case of a seventy year-old man who presented with a very short period of jaundice leading to fulminant hepatic failure (FHF). On further investigation he was found to have primary angiosarcoma of liver.

Conclusion: The treatment outcomes for hepatic angiosarcoma are poor, we discuss the options available and the need for prompt investigation and establishment of a diagnosis.

View Similar Images In: Results  - Collection
View Article: PubMed Central - HTML -  PubMed
Show All Figures - Show MeSH
getmorefigures.php?pmc=2567320&rFormat=json&query=null&req=5