Nguyen Cong Long, Nguyen Ham Hoi, Luong Tuan Hiep, Nguyen Nghe Tinh, Le Van Khang, Vu Truong Khanh
Department of Gastroenterology & Hepatology, Bach Mai Hospital, Ha Noi, Viet Nam; School of Medicine and Pharmacy, Vietnam National University, Hanoi, Viet Nam.
Department of Gastrointestinal and Hepato-Pancreato-Biliary Surgery, Bach Mai Hospital, Hanoi, Viet Nam.
Int J Surg Case Rep. 2022 May;94:107087. doi: 10.1016/j.ijscr.2022.107087. Epub 2022 Apr 18.
Angiomyolipoma (AML) is a solid benign neoplasm with mesenchymal features. The clinical signs and symptoms of hepatic angiomyolipoma are nonspecific, and treatment strategy is variable.
A 35-years-old male patient has admitted to the hospital with symptoms of severe anemia. Abdominal multi-slice computed tomography (MSCT) and Gadoxetic acid (GA)-enhanced magnetic resonance imaging (MRI) revealed a heterogeneous 23 × 17 cm-in-sized tumor with heterogeneous enhancement and increased angiogenesis. Percutaneous hepatic biopsy was proceeded and in immunohistochemistry, tumor cells responded positively to the HMB-45, SMA, and Glutamine stains, the CD-34 stain was positive for blood vessels as well as Ki-67 sporadically positive, but the Heppar1, S-100, CK stains reacted negative. The final pathologic result was consistent with the primary hepatic angiomyolipoma. The final surgical management was extended right hepatectomy with ligation of the right hepatic artery (RHA) and vein (RHV), as well as the middle hepatic vein (MHV), one month after portal embolization as well as to enlarge the remnant liver volume.
A hepatic angiomyolipoma (HAML) primary origin is relatively rare, the clinical manifestations are variable and non-specific. Histological examination and immunohistochemistry staining are considered as the gold standard for HAML diagnosis. HAML are commonly expressed benign behaviors, but HAML cases with malignant behaviors were reported in a cumulative incidence. Radical surgery must be still the most effective and major treatment approach.
The present case being the first case with initial syndrome of severe anemia accounted in the English literature. Giant angiomyolipoma is composed of blood vessels and could lead to extensive internal tumoral hemorrhage. We here present a report of this case with had primary hepatic angiomyolipoma with clinical picture of severe anemia.
血管平滑肌脂肪瘤（AML）是一种具有间叶组织特征的实体良性肿瘤。肝血管平滑肌脂肪瘤的临床体征和症状不具有特异性，治疗策略也各不相同。
一名35岁男性患者因严重贫血症状入院。腹部多层螺旋计算机断层扫描（MSCT）和钆塞酸二钠（GA）增强磁共振成像（MRI）显示，有一个大小为23×17厘米的异质性肿瘤，增强不均匀且血管生成增加。进行了经皮肝活检，免疫组织化学检查显示，肿瘤细胞对HMB-45、平滑肌肌动蛋白（SMA）和谷氨酰胺染色呈阳性反应，CD-34染色显示血管呈阳性，Ki-67也呈散在阳性，但肝细胞1（Heppar1）、S-100和细胞角蛋白（CK）染色呈阴性。最终病理结果与原发性肝血管平滑肌脂肪瘤一致。最终手术治疗为扩大性右肝切除术，结扎右肝动脉（RHA）和静脉（RHV）以及肝中静脉（MHV），在门静脉栓塞一个月后进行，以增大残余肝体积。
肝血管平滑肌脂肪瘤（HAML）原发性起源相对罕见，临床表现多样且不具有特异性。组织学检查和免疫组织化学染色被认为是HAML诊断的金标准。HAML通常表现为良性行为，但也有累积发病率报道的具有恶性行为的HAML病例。根治性手术仍然是最有效和主要的治疗方法。
本病例是英文文献中首例以严重贫血为初始综合征的病例。巨大血管平滑肌脂肪瘤由血管组成，可导致广泛的肿瘤内出血。我们在此报告了一例具有严重贫血临床表现的原发性肝血管平滑肌脂肪瘤病例。
