PDF(Pubmed)
Abstract：
UNASSIGNED: Primary hepatic epithelioid hemangioendothelioma (HEHE) is an extremely rare tumour of vascular origin with an incidence of <0.1 cases per 100,000 people worldwide.
UNASSIGNED: A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team\'s findings, liver transplantation was indicated as the only curative treatment.
UNASSIGNED: Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis.
CONCLUSIONS: Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis.
UNASSIGNED: A 29-year-old female with the history of epigastric pain and unintentional weight loss (3 kg over six months) was referred for upper endoscopy. The examination was without visual pathological findings, but a rapid urease test was positive. First-line treatment with clarithromycin-containing triple therapy for Helicobacter pylori infection was given. After completion of eradication therapy, diffuse abdominal pain developed. An abdominal computed tomography (CT) showed multiple liver nodules. Three consecutive core liver biopsies were performed and were inconclusive. A subsequent surgical liver nodule resection was performed. Histopathology of the specimen revealed grade 2 hepatocellular carcinoma; bone scintigraphy was negative for metastasis. A multidisciplinary team (MDT) recommended giving the patient sorafenib, which was poorly tolerated. The histology was reviewed using immunohistochemistry staining at the request of the oncologist, which showed expression of CD31 and CD34. Based on clinical, morphological and immunohistochemistry findings, a diagnosis of hepatic epithelioid hemangioendothelioma was made. Based on the multidisciplinary team\'s findings, liver transplantation was indicated as the only curative treatment.
UNASSIGNED: Because of the rarity of this disease, combining clinical, radiological and histopathological methods as well as an MDT approach can help to reach the correct final diagnosis. As demonstrated in this clinical case, it is crucial to perform immunohistochemistry of a liver biopsy to confirm a HEHE diagnosis.
CONCLUSIONS: Hepatic epithelioid hemangioendothelioma is a rare vascular tumour that is often misdiagnosed and mismanaged.This case emphasises the critical importance of interdisciplinary teamwork and the use of non-invasive and invasive techniques to achieve a definitive diagnosis.
摘要：
■原发性肝上皮样血管内皮瘤（HEHE）是一种极为罕见的血管源性肿瘤，全球每100,000人中有<0.1例。
■一名29岁女性，有上腹痛和意外体重减轻（6个月内3公斤）的病史，接受上消化道内窥镜检查。检查没有视觉病理发现，但快速尿素酶检测呈阳性.给予含克拉霉素三联疗法治疗幽门螺杆菌感染的一线治疗。完成根除治疗后，出现弥漫性腹痛。腹部计算机断层扫描（CT）显示多个肝结节。进行了三次连续的核心肝活检，但尚无定论。随后进行外科肝结节切除术。标本的组织病理学显示2级肝细胞癌；骨闪烁显像未显示转移。一个多学科小组（MDT）建议给患者索拉非尼，这是耐受性差。应肿瘤学家的要求，使用免疫组织化学染色检查组织学，显示CD31和CD34的表达。基于临床，形态学和免疫组织化学结果，诊断为肝上皮样血管内皮瘤。根据多学科小组的发现，肝移植被认为是唯一的治愈性治疗。
■由于这种疾病的罕见，结合临床，放射学和组织病理学方法以及MDT方法可以帮助达到正确的最终诊断。正如这个临床病例所证明的，进行肝活检的免疫组织化学对确认HEHE诊断至关重要。
结论：肝上皮样血管内皮瘤是一种罕见的血管肿瘤，常被误诊和误治。该案例强调了跨学科团队合作以及使用非侵入性和侵入性技术来实现明确诊断的至关重要性。
■一名29岁女性，有上腹痛和意外体重减轻（6个月内3公斤）的病史，接受上消化道内窥镜检查。检查没有视觉病理发现，但快速尿素酶检测呈阳性.给予含克拉霉素三联疗法治疗幽门螺杆菌感染的一线治疗。完成根除治疗后，出现弥漫性腹痛。腹部计算机断层扫描（CT）显示多个肝结节。进行了三次连续的核心肝活检，但尚无定论。随后进行外科肝结节切除术。标本的组织病理学显示2级肝细胞癌；骨闪烁显像未显示转移。一个多学科小组（MDT）建议给患者索拉非尼，这是耐受性差。应肿瘤学家的要求，使用免疫组织化学染色检查组织学，显示CD31和CD34的表达。基于临床，形态学和免疫组织化学结果，诊断为肝上皮样血管内皮瘤。根据多学科小组的发现，肝移植被认为是唯一的治愈性治疗。
■由于这种疾病的罕见，结合临床，放射学和组织病理学方法以及MDT方法可以帮助达到正确的最终诊断。正如这个临床病例所证明的，进行肝活检的免疫组织化学对确认HEHE诊断至关重要。
结论：肝上皮样血管内皮瘤是一种罕见的血管肿瘤，常被误诊和误治。该案例强调了跨学科团队合作以及使用非侵入性和侵入性技术来实现明确诊断的至关重要性。
