Paraneoplastic Phenomena of Disseminated Intravascular Coagulopathy in Hepatic Angiosarcoma – Rare, Challenging and Fatal. Case Report and Literature Review
Case studies
Sandra Strainienė
Vilnius University, Clinic of Gastroenterology, Nephrourology and Surgery,  Institute of Clinical Medicine, Vilnius University, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania Vilnius University Hospital Santaros Klinikos, Centre of Hepatology, Gastroenterology and Dietetics
https://orcid.org/0000-0003-1884-1353
Kipras Jauniškis
Vilnius University, Faculty of Medicine, 21 M.K. Ciurlionio Street, 03104 Vilnius, Lithuania
https://orcid.org/0000-0002-4318-4431
Ilona Savlan
Vilnius University, Clinic of Gastroenterology, Nephrourology and Surgery,  Institute of Clinical Medicine, Vilnius University, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania Vilnius University Hospital Santaros Klinikos, Centre of Hepatology, Gastroenterology and Dietetics
https://orcid.org/0000-0002-3689-5040
Justinas Pamedys
National Centre of Pathology, Affiliate of Vilnius University Hospital Santaros Clinics, 5 P. Baublio Street, Vilnius, 08406, Lithuania
Ieva Stundienė
Vilnius University, Clinic of Gastroenterology, Nephrourology and Surgery,  Institute of Clinical Medicine, Vilnius University, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania Vilnius University Hospital Santaros Klinikos, Centre of Hepatology, Gastroenterology and Dietetics
https://orcid.org/0000-0002-2569-3638
Valentina Liakina
Clinic of Gastroenterology, Nephrourology and Surgery, Centre of Hepatology, Gastroenterology and Dietetics, Institute of Clinical Medicine, Vilnius University, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania
https://orcid.org/0000-0001-8685-1292
Jonas Valantinas
Vilnius University, Clinic of Gastroenterology, Nephrourology and Surgery,  Institute of Clinical Medicine, Vilnius University, Vilnius University, 3 Universiteto Street, Vilnius 01513, Lithuania Vilnius University Hospital Santaros Klinikos, Centre of Hepatology, Gastroenterology and Dietetics
https://orcid.org/0000-0003-4534-2293
Published 2021-07-29
https://doi.org/10.15388/Amed.2021.28.2.1
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Keywords

Hepatic angiosarcoma
Paraneoplastic syndrome
Disseminative intravascular coagulopathy
Case report
Literature review

How to Cite

Strainienė S., Jauniškis K., Savlan I., Pamedys J., Stundienė I., Liakina V. and Valantinas J. (2021) “Paraneoplastic Phenomena of Disseminated Intravascular Coagulopathy in Hepatic Angiosarcoma – Rare, Challenging and Fatal. Case Report and Literature Review”, Acta medica Lituanica, 28(2), p. 1. doi: 10.15388/Amed.2021.28.2.1.

Abstract

Background. Hepatic angiosarcoma is an uncommon, malignant, primary liver tumor, comprising 2% of liver cancers and accounting for < 1% of all sarcomas. Patients usually present with nonspecific symptoms, such as fatigue, weight loss, right upper quadrant pain, anemia, which leads to late diagnosis of an advanced stage tumor. The median life expectancy after the diagnosis of hepatic angiosarcoma is about 6 months, with only 3% of patients surviving more than 2 years. Liver failure and hemoperitoneum are the leading causes of death in patients with liver angiosarcoma. In rarer cases, it might cause paraneoplastic syndromes such as disseminated intravascular coagulopathy. The treatment of angiosarcomas is complicated as there are no established and effective treatment guidelines due to the tumor’s low frequency and aggressive nature.
Case summary. We present the case of a 68-year old woman who was admitted to the hospital due to fatigue
and severe anemia (hemoglobin 65 g/l). Laboratory results also revealed high-grade thrombocytopenia
(8 × 109/l). The abdominal ultrasound and computed tomography scan showed multiple lesions throughout with hepatic angiosarcoma. The treatment with first-line chemotherapy (doxorubicin) was initiated despite
ongoing paraneoplastic syndrome – disseminative intravascular coagulopathy. However, the disease was terminal, and the patient died 2 months since diagnosed.
Conclusions. Hepatic angiosarcoma is a rare and terminal tumor. Therefore, knowledge about its manifestations and effective treatment methods is lacking. Disseminative intravascular coagulopathy is a unique clinical characteristic of angiosarcoma seen in a subset of patients.

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