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Primary hepatic epithelioid hemangioendothelioma: a case report.

J Int Med Res

December 2024

Department of Hepatobiliary Surgery, Affiliated Hangzhou First People's Hospital, Westlake University, School of Medicine, Hangzhou, China.

Epithelioid hemangioendothelioma is a low-grade malignant tumor of vascular origin. The rarity of hepatic epithelioid hemangioendothelioma (HEHE) makes the diagnosis and treatment of this entity challenging. We report a case of a 69-year-old female patient who suffered from HEHE and complained of abdominal distension pain with dizziness and appetite loss for more than half a month.

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Aims: The hepatic perivascular epithelioid cell tumour (PEComa), including angiomyolipoma, exhibits diverse morphology and clinical behaviour; however, its prognostic features remain undefined. This study aimed to investigate its histological features and prognostic factors.

Methods And Results: In total, 132 patients were included.

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BACKGROUND Hepatic lesion in a young woman can lead to multiple diagnostic hypotheses, mainly infection and tumor. Crohn's disease (CD) is hardly evoked by clinicians but is reportedly associated with liver damage, especially diffuse granulomas and aseptic abscess. IgA deficiency has been associated with celiac disease or inflammatory bowel disease, including CD.

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Article Synopsis
  • Hepatic epithelioid hemangioendothelioma (HEHE) is a rare malignant vascular tumor with different types that affect prognosis and treatment plans, posing unique clinical challenges for diagnosis and therapy.
  • The unclear causes of HEHE complicate its diagnosis, which often relies on tissue biopsy but encounters issues due to late identification and misdiagnosis, lacking specific treatment guidelines.
  • Surgical options are the primary recommendation for better survival outcomes, but limited patient eligibility and donor organ shortages for liver transplants create obstacles; non-surgical treatments show promise but have limited research backing.
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