[Primary hepatic angiosarcoma: a clinicopathological analysis of nine cases].

Objective: To investigate the clinical manifestations, histomorphology, and differential diagnosis of primary hepatic angiosarcoma. Methods: Nine cases of primary hepatic angiosarcoma diagnosed in the Department of Pathology, the First Affiliated Hospital of Nanjing Medical University from January 2014 to December 2021 were collected, including biopsy and surgical specimens. The histomorphology, clinical, and radiologic findings were analyzed. The relevant literature was also reviewed. Results: There were six males and three females, aged 30 to 73 years (mean 57 years). Grossly, the growth pattern of the tumor was classified as either mass formation or non-mass formation (sinusoidal). Microscopically, the mass-forming primary hepatic angiosarcoma were further subdivided into vasoformative or non-vasoformative growth patterns; and those non-vasoformative tumors had either epithelioid, spindled, or undifferentiated sarcomatoid features. Sinusoidal primary hepatic angiosarcoma on the other hand presented with markedly dilated and congested blood vessels of varying sizes, with mild to moderately atypical endothelial cells. Follow-up in all nine cases revealed 8 mortality ranging from 1 to 18 months (mean 5 months) from initial diagnosis. One patient was alive with disease within a period of 48 months. Conclusions: Primary hepatic angiosarcoma is a rare entity with a wide spectrum of histomorphology, and often misdiagnosed. It should be considered when there are dilated and congested sinusoids, with overt nuclear atypia. The overall biological behavior is aggressive, and the prognosis is worse.目的： 探讨原发性肝脏血管肉瘤的临床病理学特征、诊断及鉴别诊断。 方法： 收集南京医科大学第一附属医院2014年1月至2021年12月经穿刺及手术标本诊断的原发性肝脏血管肉瘤9例，对其病理特征及镜下组织学形态进行回顾性阅片，结合临床及影像学特点进行分析总结，并复习相关文献。 结果： 9例患者中男性6例，女性3例；发病年龄30~73岁（平均年龄57岁）。大体检查，肿瘤的生长方式主要有肿块型和无肿块型（弥漫血窦型）。肿块型镜下形态可表现为有管腔形成的经典的血管肉瘤或无管腔形成的高级别肉瘤形态，后者依据细胞形态又分为上皮样、梭形、未分化肉瘤样形态，而无肿块型镜下表现为弥漫、扩张、充血的血窦，衬覆一定异型性的肿瘤性内皮细胞。9例患者获随访结果，随访时间为1~48个月，8例患者死亡，从诊断到死亡为1~18个月，中位生存时间约5个月，1例患者随访48个月，带病生存。 结论： 原发性肝脏血管肉瘤罕见，镜下形态多样，较易误诊及漏诊。尤其在穿刺活检中出现大小不等的明显扩张、充血的血窦，且窦内皮细胞伴一定异型时，应想到血管肉瘤的诊断。肿瘤整体生物学行为恶性，预后差。.