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Abstract：
BACKGROUND: Hepatic angiomyolipoma (HAML) is a rare mesenchymal liver tumour which belongs to the family of perivascular epithelioid cell tumours (PEComas). It is typically composed of blood vessels, smooth muscle, and adipose cells, and shows strong immunoreactivity for HMB-45.
UNASSIGNED: A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained.
CONCLUSIONS: Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells.
CONCLUSIONS: HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.
UNASSIGNED: A 57-year-old woman was referred to our hospital with an unclear liver lesion. A fine needle biopsy revealed a suspicion of hepatic angiomyolipoma with extramedullary haematopoiesis. Preoperative imaging revealed a tumour 17 cm in diameter in the left liver lobe segments II and III. A lobectomy of the left lobe segments II and III was performed. The pathological diagnosis of hepatic angiomyolipoma was obtained.
CONCLUSIONS: Variations in the predominance of the tissue components in HAML impedes diagnosis based on imaging alone. The most promising evidence of HAML is the histological identification of lipomatous, myomatous, and angiomatous tissue combined with immunohistochemical positivity for HMB-45. Although the tumour is considered benign, some cases have been described with malignant behaviour. Surgical resection should be considered in case of symptoms, inconclusive biopsy, or growth in follow-up. Other surgical indications may include aggressive patterns such as vascular invasion, p53 immunoreactivity, or rapidly proliferating tumour cells.
CONCLUSIONS: HAML is a rare liver tumour. In patients with symptoms, uncertain diagnosis, or tumour growth, surgical resection should be performed according to oncological criteria.
摘要：
背景：肝血管平滑肌脂肪瘤（HAML）是一种罕见的间叶性肝肿瘤，属于血管周围上皮样细胞肿瘤（PEComas）家族。它通常由血管组成，平滑肌,脂肪细胞，并且显示对HMB-45的强免疫反应性。
■一名57岁的妇女因肝脏病变不清被转诊到我们医院。细针活检显示怀疑有髓外造血的肝血管平滑肌脂肪瘤。术前成像显示，左肝叶II和III段的肿瘤直径为17厘米。对左叶II和III段进行了肺叶切除术。获得肝血管平滑肌脂肪瘤的病理诊断。
结论：HAML中组织成分优势的变化阻碍了仅基于影像学的诊断。HAML最有希望的证据是脂肪瘤的组织学鉴定，肌瘤，血管瘤组织联合HMB-45免疫组化阳性。虽然肿瘤被认为是良性的，一些病例被描述为恶性行为。如果出现症状，应考虑手术切除，不确定的活检，或后续增长。其他手术适应症可能包括侵袭性模式，如血管侵犯，p53免疫反应性，或快速增殖的肿瘤细胞。
结论：HAML是一种罕见的肝脏肿瘤。有症状的患者,不确定的诊断，或肿瘤生长，应根据肿瘤标准进行手术切除。
■一名57岁的妇女因肝脏病变不清被转诊到我们医院。细针活检显示怀疑有髓外造血的肝血管平滑肌脂肪瘤。术前成像显示，左肝叶II和III段的肿瘤直径为17厘米。对左叶II和III段进行了肺叶切除术。获得肝血管平滑肌脂肪瘤的病理诊断。
结论：HAML中组织成分优势的变化阻碍了仅基于影像学的诊断。HAML最有希望的证据是脂肪瘤的组织学鉴定，肌瘤，血管瘤组织联合HMB-45免疫组化阳性。虽然肿瘤被认为是良性的，一些病例被描述为恶性行为。如果出现症状，应考虑手术切除，不确定的活检，或后续增长。其他手术适应症可能包括侵袭性模式，如血管侵犯，p53免疫反应性，或快速增殖的肿瘤细胞。
结论：HAML是一种罕见的肝脏肿瘤。有症状的患者,不确定的诊断，或肿瘤生长，应根据肿瘤标准进行手术切除。
