Primary hepatic angiosarcoma

Chaudhary, Poras; Bhadana, Utsav; Singh, Rajnish; Ahuja, Arvind

doi:10.1016/j.ejso.2015.04.022

Cited by 94 publications

(123 citation statements)

References 30 publications

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“…Survival after PHA is very poor, with 5-year survival estimated at 6.4% [10]. To our knowledge, there is no known association between a history of gastrointestinal carcinoid tumors and subsequent development of angiosarcoma, as occurred in this patient, and there is no mechanistic explanation to suspect such an association.…”

Section: Discussionmentioning

confidence: 94%

“…PHA is a rare and aggressive malignancy, estimated to occur at a rate of 0.5–2.5 cases per 10,000,000 persons, and accounts for 2% of all primary hepatic malignancies [1, 10]. It is known to present a significant diagnostic challenge given its rare occurrence, non-specific symptomatology, and challenging radiographic diagnosis.…”

Section: Discussionmentioning

confidence: 99%

“…Diagnosis hinges on imaging, which is non-specific, and biopsy which carries a significant risk of inducing hemorrhage. Microscopic examination shows spindle-shaped cells with both sinusoidal and solid patterns, and immunohistochemical staining positive for ERG, factor VIII, CD 31, and CD 34 [7, 10]. While CD31 has traditionally been a reliable marker, one study found ERG to be positive in 100% of samples, while CD34 was positive in 87.5% [10].…”

Section: Discussionmentioning

confidence: 99%

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Primary Hepatic Angiosarcoma Mimicking Multifocal Liver Abscess with Disseminated Intravascular Coagulation and Hemoperitoneum

Rowe

Nehme

Wallace

et al. 2017

Cureus

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Primary hepatic angiosarcoma (PHA), a rare and aggressive malignancy, has rarely been reported to present with disseminated intravascular coagulation with liver hemorrhage. Incidence is estimated at 0.5–2.5 cases per 10,000,000. To our knowledge, it has not been reported to mimic liver abscess with a septic presentation. Advanced imaging techniques may aid in the diagnosis, though biopsy with microscopy and immunohistochemistry is the mainstay. Prognosis is very poor, with a 5-year survival rate estimated at 6.4%. We present the case of a 70-year-old man who presented with sepsis thought to be due to a multifocal liver abscess, who did not respond to drainage and antibiotics. This atypical course led to further workup which subsequently revealed a diagnosis of PHA.

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Section: Discussionmentioning

confidence: 94%

Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

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Primary Hepatic Angiosarcoma Mimicking Multifocal Liver Abscess with Disseminated Intravascular Coagulation and Hemoperitoneum

Rowe

Nehme

Wallace

et al. 2017

Cureus

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“…Cholangiocarcinoma is a heterogeneous malignancy that develops in the biliary tree of adults and is classified as intrahepatic, perihilar or distal based on the anatomical location 5 . Primary hepatic angiosarcoma is an extremely rare soft tissue sarcoma in which pleomorphic endothelial cells grow into vascular spaces, including sinusoids and terminal hepatic venules 6 . Hepatoblastoma is similarly a very rare paediatric primary liver cancer thought to arise from a hepatocyte precursor known as a hepatoblast, which is present during fetal liver development 7 …”

Section: Introductionmentioning

confidence: 99%

“…5 Primary hepatic angiosarcoma is an extremely rare soft tissue sarcoma in which pleomorphic endothelial cells grow into vascular spaces, including sinusoids and terminal hepatic venules. 6 Hepatoblastoma is similarly a very rare paediatric primary liver cancer thought to arise from a hepatocyte precursor known as a hepatoblast, which is present during fetal liver development. 7 The original six hallmark features of cancer focussed on tumour cell features that enabled survival, proliferation and dissemination.…”

Section: Introductionmentioning

confidence: 99%

Immune checkpoint inhibition: prospects for prevention and therapy of hepatocellular carcinoma

et al. 2017

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The global prevalence of liver cancer is rapidly rising, mostly as a result of the amplified incidence rates of viral hepatitis, alcohol abuse and obesity in recent decades. Treatment options for liver cancer are remarkably limited with sorafenib being the gold standard for advanced, unresectable hepatocellular carcinoma but offering extremely limited improvement of survival time. The immune system is now recognised as a key regulator of cancer development through its ability to protect against infection and chronic inflammation, which promote cancer development, and eliminate tumour cells when present. However, the tolerogenic nature of the liver means that the immune response to infection, chronic inflammation and tumour cells within the hepatic environment is usually ineffective. Here we review the roles that immune cells and cytokines have in the development of the most common primary liver cancer, hepatocellular carcinoma (HCC). We then examine how the immune system may be subverted throughout the stages of HCC development, particularly with respect to immune inhibitory molecules, also known as immune checkpoints, such as programmed cell death protein-1, programmed cell death 1 ligand 1 and cytotoxic T lymphocyte antigen 4, which have become therapeutic targets. Finally, we assess preclinical and clinical studies where immune checkpoint inhibitors have been used to modify disease during the carcinogenic process. In conclusion, inhibitory molecule-based immunotherapy for HCC is in its infancy and further detailed research in relevant in vivo models is required before its full potential can be realised.

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Clinical characteristics, imaging features, and outcomes of primary hepatic angiosarcoma: A single‐center study and literature review

Wu,

Kuo,

Sun

et al. 2024

Adv in Digestive Medicine

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Primary hepatic angiosarcoma (PHA) accounts for 0.5% to 2% of all primary hepatic malignancies, and histopathology findings are required to diagnose PHA accurately. This study investigated 12 patients with PHA at a single medical center in southern Taiwan. We analyzed the clinical characteristics, imaging features, histopathology findings, and survival outcomes of patients with PHA. Of the 12 patients, abdominal pain and fullness were the most common symptoms, and their liver biochemistry data and tumor markers were mostly within the normal limits. The liver tumors tended to present as multifocal or diffuse nodules with bilobar involvement. The median overall survival (OS) of the 12 patients after diagnosis was 9 months. Improved OS was observed in the surgery group compared with the nonsurgery group (15 vs. 2 months, p = .003). The median OS of patients in the surgery ± systemic therapy group was superior to that of patients in the systemic therapy group and in the no‐therapy groups (15 vs. 5 vs. 2 months, respectively; p = .012). Complete surgical resection remains the optimal treatment choice, and combined surgery and systemic therapy seem beneficial but require further investigation.

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Primary hepatic angiosarcoma

Cited by 94 publications

References 30 publications

Primary Hepatic Angiosarcoma Mimicking Multifocal Liver Abscess with Disseminated Intravascular Coagulation and Hemoperitoneum

Primary Hepatic Angiosarcoma Mimicking Multifocal Liver Abscess with Disseminated Intravascular Coagulation and Hemoperitoneum

Immune checkpoint inhibition: prospects for prevention and therapy of hepatocellular carcinoma

Clinical characteristics, imaging features, and outcomes of primary hepatic angiosarcoma: A single‐center study and literature review

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