肝脏血管平滑肌脂肪瘤182例临床病理特征及预后分析

Hepatic angiomyolipoma： a clinicopathologic features and prognosis analysis of 182 cases

目的了解肝脏血管平滑肌脂肪瘤（HAML）患者的临床病理特征及部分临床病理参数与预后的相关性。 方法回顾性分析182例HAML的临床病理学特征，根据文献复习分为上皮样型、经典型和脂肪为主型，并通过变量分析比较各参数与肿瘤分型的相关性。对165例临床病理资料完整的病例进行随访，并对病理学参数与预后关系进行分析。 结果HAML好发于女性（男女比为1∶4），发病年龄17～77岁（中位年龄46岁），肿瘤最大径0.3～32.0 cm，平均5.0 cm；多为单发（168/182，92.3%），≥2个灶者14例（14/182，7.7%），均为女性。组织学分型提示HAML以上皮样型为主（112/165，67.9%）。肿瘤浸润性生长方式、含铁血黄素沉积、肿瘤渗血/出血、瘤巨细胞、细胞核异型、核内包涵体和出现核仁7项形态学特征多出现在上皮样型中，这些形态学特征在各亚型中差异有统计学意义（P〈0.05）。共有97例（58.8%，97/165）获得完整的随访资料，随访时间4至130个月，平均41个月。5例术后发生复发或转移，无1例死于肿瘤。统计学分析发现肿瘤直径≥10 cm、多发病灶、肿瘤侵犯汇管区、肿瘤性坏死、脉管内瘤栓、肿瘤内出现核分裂象和瘤巨细胞7项病理参数与肿瘤复发或转移存在明显相关性（P〈0.05）。 结论肿瘤≥10 cm、多发病灶、肿瘤侵犯汇管区、肿瘤性坏死、脉管内瘤栓、肿瘤内出现核分裂象和瘤巨细胞可能与肿瘤的复发或转移有密切关系。肿瘤浸润性生长和细胞核异型性在肿瘤复发或转移中无显著影响。

ObjectiveTo study the clinicopathological characteristics of hepatic angiomyolipoma （HAML） and to evaluate the correlation between clinicopathological parameters and tumor subtypes. MethodsRetrospective analysis of clinicopathological features was conducted in 182 cases of HAML. ResultsHAML patients were predominantly female（M∶F=1∶4） and most commonly presented with non-specific symptoms. The median age at diagnosis was 46 years, ranged from 17 to 77 years. Tumor diameter was ranged from 0.3 to 32.0 cm with an average of 5.0 cm. Majority of the tumor was epithelioid type （112/165, 67.9%）. Extramedullary hematopoiesis, multinucleated giant cells, intranuclear inclusions, nucleolus, cellular atypia, invasive growth pattern, multiple masses, hyperpigmentation and purpura-like changes mostly occurred in the epithelioid type （P〈0.05）. Extramedullary hematopoiesis was commonly seen in HAML, the significance of which was still uncertain. ConclusionsMost of HAML are epithelioid type, characterized by a proliferation of predominantly epithelioid cells, in which extramedullary hematopoiesis is commonly seen. Some morphologic features that may predict malignant such as necrosis, mitotic figures, and tumor emboli are only found in the epithelioid HAML. Mitotic activity, tumor necrosis, tumor thrombus, giant cells, periportal invasion, multiple lesions and tumors size over 10 cm are closely related with tumor recurrence and metastasis.