Primary Hepatic Angiosarcoma: A Rare Liver Malignancy – Varying Manifestations but Grave Prognosis

Rujeerapaiboon, Natthapat; Wetwittayakhlang, Panu

doi:10.1159/000506928

Cited by 14 publications

(7 citation statements)

References 15 publications

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“…Acute intra-abdominal bleeding may occur secondary to the spontaneous rupture of PHA, as previously described by a few case reports [ 9 , [14] , [15] , [16] ]. Haemoperitoneum due to spontaneous tumour rupture occurs in 15–27% of PHA cases and DIC may develop subsequently [ 2 ].…”

Section: Clinical Discussionmentioning

confidence: 96%

Case report: Haemoperitoneum secondary to acute rupture of primary hepatic angiosarcoma

Kim

Reardon

Cross

et al. 2021

International Journal of Surgery Case Reports

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Introduction and importance Primary hepatic angiosarcoma (PHA) is a rare and aggressive liver malignancy of endothelial cell origin and is associated with poor outcome. Pre-operative confirmation of the diagnosis is challenging, as clinical and radiological findings are generally non-specific. Very rarely, spontaneous haemoperitoneum may occur due to the spontaneous rupture of previously undiagnosed PHA. Case presentation We describe a case of a 28-year-old male with haemoperitoneum due to the rupture of previously undiagnosed PHA. After failing to respond to the non-operative measures, the patient underwent emergency partial liver resection and recovered without any post-operative complications. Histopathological examination of the specimen confirmed the diagnosis of PHA. Two months after the operation, the patient represented with advanced metastatic disease and disseminated intravascular coagulation (DIC). The patient died one month after discharge. Clinical discussion A patient with PHA presents a diagnostic challenge due to its rare incidence and non-specific clinical findings. Spontaneous intra-abdominal haemorrhage can occur due to PHA rupture and carries a dismal prognosis. In addition to emergency haemorrhage control, complete surgical resection with clear margins is the definitive treatment to date, however, most cases of PHA are unresectable at diagnosis and recurrence is common even after complete resection. Conclusion PHA is associated with very poor outcomes, due to its rapid progression, early recurrence, and metastatic nature. The median survival is approximately 5 months. Haemoperitoneum secondary to rupture of previously undiagnosed PHA is uncommon and is a poor prognostic indicator. Complete surgical resection of the disease is challenging and there is no established treatment.

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Section: Clinical Discussionmentioning

confidence: 96%

Case report: Haemoperitoneum secondary to acute rupture of primary hepatic angiosarcoma

Kim

Reardon

Cross

et al. 2021

International Journal of Surgery Case Reports

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“…In 2020, Rujeerapaiboon et al reported that, out of 3 female cases with liver angiosarcoma, one presented with abdominal pain and weight loss. 8 The abdominal CT scan detected diffused multiple arterial enhancing infiltrating nodules varying in size, scattered throughout the entire liver. Laparoscopic liver biopsy confirmed liver angiosarcoma.…”

Section: Discussionmentioning

confidence: 99%

Liver Angiosarcoma with Poor Prognosis in a 61-Year-Old Woman: A Case Report and Literature Review

Hung¹,

Tran²

2022

IJWH

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Hepatic angiosarcoma is very rare malignancy and more common in men than in women. To date, only a few female cases of liver angiosarcoma have been reported. Here, we report a female case of liver angiosarcoma, first detected in Vietnam, with a high malignancy stage, rapid progression, and poor prognosis. Case Presentation: A 61-year-old woman was admitted to the Bach Mai Hospital with fatigue, anorexia, weight loss, and severe pain in the right upper quadrant for 2 weeks prior. Clinical examination detected a firm 4-cm hepatomegaly below the right costal margin and grade I splenomegaly. Abdominal ultrasonography and CT revealed diffuse lesions in the entire liver parenchyma, spreading to the spleen, while MRI showed signs of bone metastasis. Blood tests showed elevated transaminase enzymes, especially Gamma Glutamyl Transferase 501 U/L; thrombocytopenia; no anemia; and other tumor markers such as AFP, CEA, and CA19-9 were within normal limits. On CT images, the dots and nodules in the liver and spleen appeared hyperenhanced in the arterial phase and washout in the venous phase. The results of both histopathology and immunohistochemistry showed liver angiosarcoma. Surgery and radiation were not indicated due to the suspicion of bone metastasis. Chemotherapy with doxorubicin at a dose of 60 mg/m2 and intravenous infusion once every 21 days was administered. Unfortunately, during the first dose of chemotherapy with doxorubicin, side effects appeared. Since the disease developed continuously and uncontrollably, the patient was subsequently exhausted, anemic, presented peritoneal fluid, and eventually died of intra-abdominal bleeding. Conclusion:For the diagnosis of liver angiosarcoma, ultrasound-guided liver biopsy could be applied for safe and effective histopathology, and selective embolization of the hepatic artery is necessary to prevent bleeding complications. The disease has a very poor prognosis, and if chemotherapy does not respond, the patient can die within six months of diagnosis.

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“…Early diagnosis is difficult due to the non‐specific nature of symptoms. It is associated with a poor survival due to its rapid progression, high recurrence rate and resistance to many traditional chemotherapy and radiotherapy options (1).…”

Section: Figmentioning

confidence: 99%

A ruptured 200‐mm primary hepatic angiosarcoma: A case report

Alderuccio

Harisis

et al. 2021

ANZ Journal of Surgery

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Primary Hepatic Angiosarcoma: A Rare Liver Malignancy – Varying Manifestations but Grave Prognosis

Cited by 14 publications

References 15 publications

Case report: Haemoperitoneum secondary to acute rupture of primary hepatic angiosarcoma

Case report: Haemoperitoneum secondary to acute rupture of primary hepatic angiosarcoma

Liver Angiosarcoma with Poor Prognosis in a 61-Year-Old Woman: A Case Report and Literature Review

A ruptured 200‐mm primary hepatic angiosarcoma: A case report

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