Background : Primary hepatic angiosarcoma (PHA) is an extremely rare malignant neoplasm that accounts for only 0.1 ~ 2% of primary malignant liver tumors. The nonspecific symptoms of PHA and rapid progression result in a poor prognosis. Although there are various treatments for PHA, no standard treatment is suitable for diffuse and multiple PHAs. We herein report a case of ruptured diffuse PHA with poor prognosis after surgery.

Methods : A 36-year-old female without any medical problem visited the emergency center due to abdominal pain for 3 days. Initial laboratory test showed anemia (Hb 6.2 g/dl), thrombocytopenia (36,000 /uL), and slightly increased liver function tests. Diffuse variable-sized high-enhancing mass lesions on the whole liver and extravasation with hemoperitoneum were detected on the CT scan. We tried an angiographic embolization, but there was no definite bleeding focus. The interventional radiologist performed selective embolization at feeding vessels of the largest mass on S4. After angiography, the patient was observed in ICU with stable vital signs until 5 days. On day 6, re-bleeding with hypovolemic shock was identified abruptly. We performed an emergency tumorectomy of the ruptured S4 mass.

Results : Although there was no eventful bleeding after surgery, the patient suffered lethal liver failure and expired at postoperative 38 days.

Conclusions : Due to intra-abdominal bleeding from tumor rupture appearing to be a common manifestation, short-term outcomes after surgery for diffuse PHAs are extremely poor. Surgical resection of diffuse and multiple PHAs can result in aggravating liver failure.