Ortiz Santiago, Tortosa Francisco
Anatomía Patológica, Centro Hospitalario Lisboa Norte, Portugal.
Anatomía Patológica, CHLN - Hospital de Santa Maria, Portugal.
Rev Esp Enferm Dig. 2016 Jan;108(1):27-30. doi: 10.17235/reed.2015.3947/2015.
Hepatic angiomyolipoma is a rare neoplasm that can be difficult to diagnose in cases of ecoguide biopsy. We studied 4 cases of female patients with a mean age of 51 years. None of the patients presented specific abdominal symptoms, or other tumour masses detected by chance. One of them had relevant personal history: Renal cell carcinoma. This same patient was diagnosed with a contralateral renal angiomyolipoma. None of the patients showed evidence of tuberous sclerosis. Three tumours have been diagnosed by biopsy and only in one patient was decided to completely remove the tumour surgically. None of the patients had evidence of recurrence of disease or an increase in tumour size over an average period of 45 months. Histologically, the tumours have been sub classified into angiomyolipomas epithelioid. In 50% of the cases, extramedullary haematopoiesis was observed. The first diagnostic impression using imaging methods included: Focal nodular hyperplasia, hepatocellular adenoma, hepatocellular carcinoma and metastasis. By immunohistochemistry, it has been demonstrated that all tumours expressing melanocytic markers (HMB45 and Melan A) and markers of smooth muscle cells (smooth muscle actin). The diagnosis of these tumours is increasing due to programmes for early detection of hepatocellular carcinoma in patients with liver cirrhosis. It must be taken into account their broad spectrum of morphological diversity to avoid incorrect diagnosis of a malignant neoplasm.
肝血管平滑肌脂肪瘤是一种罕见的肿瘤,在超声引导活检的情况下可能难以诊断。我们研究了4例平均年龄为51岁的女性患者。所有患者均无特异性腹部症状,也未偶然发现其他肿瘤肿块。其中1例有相关个人病史:肾细胞癌。该患者还被诊断出对侧肾血管平滑肌脂肪瘤。所有患者均无结节性硬化症的证据。3例肿瘤通过活检确诊,仅1例患者决定手术完全切除肿瘤。所有患者在平均45个月的时间里均无疾病复发或肿瘤大小增加的迹象。组织学上,肿瘤已被亚分类为上皮样血管平滑肌脂肪瘤。50%的病例中观察到髓外造血。使用影像学方法的初步诊断印象包括:局灶性结节性增生、肝细胞腺瘤、肝细胞癌和转移瘤。通过免疫组织化学证实,所有肿瘤均表达黑素细胞标志物(HMB45和Melan A)和平滑肌细胞标志物(平滑肌肌动蛋白)。由于肝硬化患者肝细胞癌早期检测计划的实施,这些肿瘤的诊断率正在上升。必须考虑到它们广泛的形态学多样性,以避免对恶性肿瘤的误诊。