AI Article Synopsis

  • Hepatic epithelioid hemangiendothelioma is a rare vascular tumor with an unpredictable clinical course, and treatment depends on the patient's condition, with liver transplantation being the preferred option for diffuse cases without metastases.
  • A 32-year-old woman presented with multiple liver lesions but normal physical and biochemical exams; imaging suggested the tumor, but a biopsy failed to identify it accurately.
  • The definitive diagnosis was achieved through laparotomy and immunohistochemistry, leading to successful treatment via orthotopic liver transplantation, with no tumor recurrence observed 18 months post-surgery.

Article Abstract

Hepatic epithelioid hemangiendothelioma is a rare vascular tumor. The clinical course is unpredictable and different treatment modalities are offered depending on the patients condition. Orthotopic liver tranplantation is the choice of treatment in diffuse cases without metastases. A 32 year old woman was admitted to hospital with multiple mass lesions diagnosed by ultrasonography of the liver. Physical examination was normal except for a painless hepatomegaly, and her biochemical tests were within the normal range. Computed tomographic scanning showed the presence of multiple lesions in both lobes, some of which were accompanied by a small degree of calcification. Although these findings were suggestive of hepatic epithelioid hemangioendothelioma, ultrasonographic guided fine needle aspiration biopsy failed to diagnose the exact nature of the lesions. The diagnosis of hepatic epithelioid hemangioendothelioma was confirmed by diagnostic laparotomy and immunohistochemical examination of the specimen by FVIII-RAg, CD34 and CD 31 markers. The patient was treated by orthotopic liver transplantation and had no evidence of tumor 18 months after transplantation. The problems in differential diagnosis and treatment options are discussed in this report of the first case of this rare tumor, treated by orthotopic liver transplantation in Turkey.

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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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Backgrounds: Primary hepatic epithelioid hemangioendothelioma (HEHE) is a rare neoplasm of vascular origin with varying biologic behavior, making it challenging to diagnose.

Case Presentation: We present a case of synchronous hepatocellular carcinoma (HCC) and HEHE in a 43-year-old Chinese male patient. Multiple hypoechoic liver lesions were depicted, but no specific imaging findings were detected on enhanced computed tomography (CT) or contrast-enhanced magnetic resonance imaging (MRI).

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Background: Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal neoplasm that predominantly affects the kidney and uterus. The occurrence of this tumor in the liver, particularly with simultaneous involvement of the liver and kidney, is exceedingly uncommon. Pathological diagnosis is the gold standard.

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Background/aims: Epithelioid hemangioendothelioma (EHE) is an uncommon vascular tumor that commonly affects the liver. Hepatic EHE (HEHE) presents with variable clinical and histopathological features. We describe detailed clinico-histopathological features, differential diagnosis, and treatment outcomes of the cases of HEHE diagnosed in our center.

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