Surgically Diagnosed Primary Hepatic Angiosarcoma

Tsunematsu, Seiji; Muto, Shuichi; Oi, Hiroki; Naka, Tomoaki; Kitagataya, Takashi; Sasaki, Rui; Taya, Yoko; Baba, Urara; Tsukamoto, Yuki; Uemura, Kazuhito; Kimura, Takeshi; Ohara, Yukio

doi:10.2169/internalmedicine.9318-17

Cited by 10 publications

(16 citation statements)

References 20 publications

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“…Angiosarcomas are highly aggressive lesions, and are particularly rare in the liver. Diagnosis of PHA remains challenging since clinical presentation of PHA and radiological findings are usually unspecific [ 6 , 7 , 17 ]. Furthermore, no specific tumor markers are available, so far.…”

Section: Discussionmentioning

confidence: 99%

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity

Katou

Martinelli

Silveira

et al. 2022

JCM

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Primary hepatic angiosarcoma (PHA) is a rare malignant tumor of the liver, and data on patient outcome after surgical treatment are scarce. The aim of this study was to evaluate postoperative morbidity and overall survival (OS) of patients who underwent hepatectomy for PHA. This is a bicentric retrospective analysis of all consecutive patients who underwent liver resection in curative intent for PHA between 2012 and 2019 at the University Hospital of Muenster and the University Hospital of Bern. Nine patients (five female, four male) were included from both centers. Median age was 72 years (44–82). Most lesions (77.8%) were larger than 5 cm, and mean size of the biggest lesion was 9.4 ± 4.5 cm. Major hepatectomy was performed in four (44.4%), and radical resection (R0) was achieved in six (66.7%) patients. Postoperative complication rate was 88.8%, including 44.4% higher than 3a in the Clavien–Dindo classification. OS survival rates at 1, 2, and 3 years were 44.4%, 22.2%, and 12.5%, respectively, and median OS was 5 months. OS was significantly better after radical resection (R0: 15 months vs. R1: 0 months, p = 0.04), whereas presentation with tumor rupture at diagnosis was associated with the worst OS (0 months vs. 15 months, p = 0.005). Disease recurrence occurred in three patients (33.3%) between three and seven months after surgery. Radical resection remains the only potentially curative treatment option for PHA. However, postoperative morbidity is high, and the overall prognosis remains poor. Multimodal therapy options and management strategies are urgently needed and could improve the prognosis of patients suffering from PHA in the future.

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Section: Discussionmentioning

confidence: 99%

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity

Katou

Martinelli

Silveira

et al. 2022

JCM

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“…This is consistent with previous reports in the literature. 9,10 The diagnosis of liver angiosarcoma still faces many obstacles and challenges owing to non-specific clinical symptoms, laboratory tests, indecision imaging, and high bleeding risk due to liver biopsy. According to Tsunematsu's report, a case was not performed a biopsy of a liver tumor that was suspected to be malignant based on pre-operative imaging, but liver angiosarcoma was diagnosed only after surgical resection of the liver tumor.…”

Section: Discussionmentioning

confidence: 99%

“…According to Tsunematsu’s report, a case was not performed a biopsy of a liver tumor that was suspected to be malignant based on pre-operative imaging, but liver angiosarcoma was diagnosed only after surgical resection of the liver tumor. 10 Averbukh et al performed the first ultrasound-guided liver biopsy and detected no evidence of malignancy, but a second biopsy after two months revealed a liver angiosarcoma at an advanced stage. 4 Laparoscopic liver biopsy has certain advantages, including direct tumor detection, selection of specimen collection sites, and post-biopsy bleeding control.…”

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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“…The prognosis for patients with liver angiosarcoma is extremely grim with mean survival around 6 months due to early distant metastasis to other organs such as the lungs, spleen, and colon [ [9] , [10] , [11] ]. There are some environmental risk factors linked to liver angiosarcomas such as the use of an oral contraceptive pill or anabolic steroids, radiation exposure, arsenic, vinyl chloride, and Thorotrast (a suspension of radioactive compound thorium dioxide, that was used as a radiocontrast agent until 1950s) which contribute to increasing the incidence of hepatic angiosarcoma [ 12 , 13 ]. Diagnosis of hepatic angiosarcoma is challenging since patients usually present with nonspecific symptoms including right upper quadrant abdominal pain, weight loss, fever, and fatigue.…”

Section: Discussionmentioning

confidence: 99%

Hepatic angiosarcoma in an adult who had Wilms tumor treated in childhood: A case report

Shayesteh

Fouladi

Blanco

et al. 2021

Radiology Case Reports

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Hepatic angiosarcoma is a rare, highly aggressive mesenchymal liver malignancy with poor prognosis that stems from the endothelial cells that line the walls of blood or lymphatic vessels. It is the third most common primary liver malignancy and is most prevalent among older males. It is difficult to diagnose due to various clinical presentations from asymptomatic to abdominal pain, pleural effusion, and liver failure. The diagnosis of liver angiosarcoma is suspected on imaging features and confirmed by histopathological assessment. Primary management is determined based on the stage of tumor from surgery to palliative care such as chemotherapy or tumor transarterial embolization. We report a 51-year-old female who presented with stage 4 liver angiosarcoma and a history of childhood Wilms tumor. We focus on tumor management using radiological modalities and pathological analysis and discuss secondary liver tumors in survivors of childhood Wilms tumor.

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Surgically Diagnosed Primary Hepatic Angiosarcoma

Cited by 10 publications

References 20 publications

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity

Liver Resection for Primary Hepatic Angiosarcoma: Bicentric Analysis of a Challenging Entity

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

Hepatic angiosarcoma in an adult who had Wilms tumor treated in childhood: A case report

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