Herald bleeding from a ruptured primary hepatic angiosarcoma: A case report

Abstract

Abstract. Primary hepatic angiosarcomas (PHAs) are rare tumours with an estimated annual incidence of 0.05 per million in the Caribbean, which is similar to that reported in Western countries. Although a number of cases are detected post-mortem, the most common clinical presentation is with tumour rupture and herald bleeding. This is the case report of a 60-year old female patient who presented with vague upper abdominal pain, found via imaging examinations to be due to a ruptured hepatic tumour in segment III of the liver. The tumour was removed via laparoscopic left lateral sectionectomy, with clear resection margins. The histopathological and immunohistochemical examination established the diagnosis of PHA. Therefore, an index of suspicion should be maintained in selected patients and, when detected ante-mortem, PHAs should be treated aggressively with complete surgical resection to achieve microscopically clear margins, as the response of these tumours to other forms of adjuvant therapy may be unpredictable.

Progression of an Unusual Primary Liver Tumor

“…The spontaneous rupture of PHA was reported in 15% to 27% of patients in a previous study. 2 As in our case, no obvious symptoms were noted in the early stage, and spontaneous rupture was observed in the later stage.…”

“…Complete resection with clear margins is the choice of treatment; however, the prognosis is poor even after complete resection. 2 In our case, operation after diagnosis was declined because of the patient's poor prognosis and body performance. She lived for 18 months after the diagnosis.…”

Long-term 14-year survival with primary hepatic angiosarcoma

“…Unlike other malignant tumours of the liver, markers including AFP, CEA and CA19-9 are often not elevated, and only 41% of patients showed elevated transaminases 3. While CT, MRI and US are used for initial assessment, features of PHA tend to be non-specific with no known pathognomonic features 1 11…”

A case of spontaneously ruptured hepatic angiosarcoma resected after transcatheter arterial embolization

“…The etiology of 75% of primary hepatic angiosarcomas is unknown, and although the remaining 25% are believed to be related to hemochromatosis and exposure to vinyl chloride monomer, thorium dioxide, arsenic compounds and radiation [ 3 , 4 ], in our case there was no history of exposure to these substances. Because exposure to these agents has become uncommon in modern practice [ 9 ], it is not surprising that none of these environmental risk factors existed in this case.…”