肝上皮样血管内皮瘤的临床、组织病理学和免疫表型谱：印度北部一家三级医疗中心的八年数据

IF 3.3 Q2 GASTROENTEROLOGY & HEPATOLOGY

Journal of Clinical and Experimental Hepatology Pub Date : 2024-10-15 DOI:10.1016/j.jceh.2024.102429

Gunjangeet Kaur , Suvradeep Mitra , Adarsh Barwad , Debajyoti Chatterjee , Treshita Dey , Divya Khosla , Uma N. Saikia , Lileshwar Kaman , Usha Dutta , Ajay Duseja , Ashim Das

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引用次数: 0

摘要

背景/目的上皮样血管内皮瘤（EHE）是一种不常见的血管肿瘤，常累及肝脏。肝EHE（HEHE）具有不同的临床和组织病理学特征。我们详细描述了本中心确诊的肝EHE病例的临床-组织病理学特征、鉴别诊断和治疗结果。方法回顾了过去八年（2016-2023年）本院确诊的所有肝EHE病例（n = 8；11个样本）（共36例EHE；22.2%）。对所有病例的临床特征、放射学、组织病理学、免疫表型、分子特征和治疗结果进行了评估。结果发病年龄中位数为 49.5 岁，男女比例为 7:1。腹痛是最常见的症状。约三分之二的患者有多灶性病变。组织病理学显示，纯上皮样、主要上皮样和主要纺锤形细胞形态的患者分别占50%、25%和25%。所有病例均表现为典型的肌层/纤维基质和门静脉/中央静脉有组织血栓。CD31 是最常用的免疫印迹，在所有病例中均呈阳性。75%的病例中CAMTA1断裂荧光原位杂交呈阳性，而没有病例显示TFE3免疫阳性。化疗是最常用的疗法（5 例），其次是手术（2 例）。中位随访时间为 26 个月。五名患者带病存活（两名患者≥3岁），一名患者死于心脏猝死，两名患者失去随访。结论我们描述了 HEHE 的临床组织病理学特征和鉴别诊断。这似乎是印度最大的 HEHE 病例系列。

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Clinical, Histopathological, and Immunophenotypic Spectrum of Hepatic Epithelioid Hemangioendothelioma: Eight Years’ Data of a Tertiary Care Center from North India

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.

Methods

All cases of HEHE diagnosed in our institute in the last eight years (2016–2023) were reviewed (n = 8; 11 samples) (total 36 cases of EHE; 22.2%). The clinical features, radiology, histopathology, immunophenotype, molecular features, and treatment outcomes of all cases were evaluated.

Results

The median age of presentation was 49.5 years with a female: male ratio of 7:1. Abdominal pain was the commonest presentation. Approximately two-thirds of the patients had multifocal lesions. Histopathology showed purely epithelioid, predominantly epithelioid, and predominantly spindle-cell morphology in 50%, 25%, and 25%, respectively. All cases showed typical myxohyaline/fibrous stroma and organized thrombi of the portal/central veins. CD31 was the most commonly used immunostain with positivity in all cases. CAMTA1 break-apart fluorescence in situ hybridization was positive in 75% of cases, while none showed TFE3 immunopositivity. Chemotherapy was the most commonly employed therapy (n = 5) followed by surgery (n = 2). The median duration of follow-up was 26 months. Five patients were alive with disease (two patients ≥3 years), one patient died of sudden cardiac death, and two patients were lost to follow-up. Two patients developed metastatic disease at follow-up.