Hepatic EHE (epithelioid hemangioendothelioma) is an uncommon entity of vascular origin and a low-grade malignant tumor. Primary hepatic EHE is rare. These tumors can be multifocal at presentation like in the soft tissues, bones, brain, liver, and small intestine. First described by Weiss and Enzinger in 1982 as a malignant vascular neoplasm with indolent behavior. We report the case of a 23-year-old male, known case of chronic liver disease, who presented with incisional hernia following exploratory laparotomy 8 months back, performed for intestinal obstruction. Contrast-enhanced computed tomography abdomen revealed an incisional hernia with a large defect along with multiple lesions in the liver (suspected metastases) and peritoneal deposits and a few discrete lung nodules. Some areas of interloop collections were also noted. Biopsies were taken from the liver which revealed benign cirrhotic lesion. Relevant to the findings, multiple biopsies were performed and fluid was sent for evaluation. The liver biopsy came out positive for borderline vascular malignancy (epithelioid hemangioendothelioma). This was confirmed with the immunohistochemistry report. Epithelioid hemangioendothelioma occurs mostly in soft tissues of extremity and lungs. The involvement of the liver may be seen as metastasis or rarely as a primary tumor. The incidence of primary malignant hepatic hemangioendothelioma is about 0.1/100,000; the mean age at the time of diagnosis is 41.7 years, and male:female ratio is 2:3. Liver transplantation, hepatectomy, chemoembolization, radiotherapy, and chemotherapeutic agents are reported treatment regimens. Malignant EHE of liver presents as multiple hepatic nodules. Being locally aggressive, it can invade the peritoneum, gut, and lungs. Orthoptic liver transplantation appears to be the only remedy because of the multifocal nature of the disease. Partial hepatectomy is possible for localized tumors.
Gastrointest Tumors

中文翻译：

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年轻男性转移性肝上皮样血管内皮瘤：罕见的表现

肝 EHE（上皮样血管内皮瘤）是一种罕见的血管起源实体，是一种低度恶性肿瘤。原发性肝 EHE 很少见。这些肿瘤在出现时可能是多灶性的，如软组织、骨骼、大脑、肝脏和小肠。Weiss 和 Enzinger 于 1982 年首次将其描述为具有惰性行为的恶性血管肿瘤。我们报告了一例 23 岁男性，已知患有慢性肝病，8 个月前因肠梗阻进行剖腹探查后出现切口疝。腹部对比增强计算机断层扫描显示切口疝有大缺损，肝脏多处病变（疑似转移）和腹膜沉积物以及一些离散的肺结节。还注意到了一些区域间循环收藏。肝脏活检显示良性肝硬化病变。与调查结果相关，进行了多次活检并送出液体进行评估。肝活检结果呈交界性血管恶性肿瘤（上皮样血管内皮瘤）阳性。免疫组化报告证实了这一点。上皮样血管内皮瘤主要发生在四肢和肺的软组织中。肝脏受累可能被视为转移或很少被视为原发肿瘤。原发性恶性肝血管内皮瘤的发病率约为0.1/100,000；诊断时的平均年龄为 41.7 岁，男女之比为 2:3。肝移植、肝切除术、化学栓塞、放射疗法和化学治疗剂是报道的治疗方案。肝脏恶性 EHE 表现为多个肝脏结节。由于具有局部侵袭性，它可以侵入腹膜、肠道和肺部。由于该疾病的多灶性，正视肝移植似乎是唯一的治疗方法。对于局部肿瘤，部分肝切除术是可能的。
胃肠道肿瘤