Chen Shaojie, Huang Wei, Yuan Yuhong, Li Xiaofeng, Zhang Shineng, Wang Lingyun
Department of Gastroenterology, Sun Yat-sen Memorial Hospital, Sun Yat-sen University, Guangzhou, China.
Guangdong Provincial Key Laboratory of Malignant Tumor Epigenetics and Gene Regulation, Sun Yat-Sen Memorial Hospital, Sun Yat-Sen University, Guangzhou, China.
Transl Gastroenterol Hepatol. 2024 Sep 12;9:76. doi: 10.21037/tgh-24-19. eCollection 2024.
Primary hepatic angiosarcoma (PHA) is a rare hepatic malignancy primarily observed in the elderly. It carries a poor prognosis as a result of the characteristics of rapid progression, high aggressiveness, and resistance to traditional chemo- and radiotherapies. Its nonspecific clinical manifestations, along with the lack of laboratory features and various imaging findings, make it hard to recognize in clinic, especially among non-high-risk populations. Hence, pathological diagnosis is essential to establish an accurate diagnosis.
In the present report, a young male presented with right upper quadrant abdominal pain and hemoperitoneum was eventually diagnosed as hepatic angiosarcoma based on the immunohistochemical staining results of a liver needle biopsy. Contrast-enhanced ultrasound (CEUS) effectively improved the accuracy of the liver needle biopsy in this case. This report describes the epidemiology, presentation, laboratory tests, imaging findings, and pathological features of a rare hepatic angiosarcoma seen in this young male. The patient rapidly developed severe abdominal hemorrhage. Transcatheter hepatic artery embolization, blood transfusions, and symptomatic treatments were administered. The family members abandoned treatment because the patient was in critical condition and could not receive antitumor therapy.
This case emphasizes the significance of pathological findings in the diagnosis of PHA especially in non-high-risk individuals, and the supportive role of CEUS in guiding the liver needle biopsy. Abdominal hemorrhage is one of the serious complications of PHA and transarterial embolization (TAE) should be considered for controlling life-threatening bleeding from ruptured tumor. Further investigation is required to early diagnosis and to improve the prognosis of patients with PHA.
原发性肝血管肉瘤（PHA）是一种罕见的肝脏恶性肿瘤，主要见于老年人。由于其进展迅速、侵袭性高以及对传统化疗和放疗耐药的特点，其预后较差。其非特异性临床表现，加上缺乏实验室特征和各种影像学表现，使得在临床上难以识别，尤其是在非高危人群中。因此，病理诊断对于准确诊断至关重要。
在本报告中，一名年轻男性因右上腹疼痛和腹腔积血就诊，最终根据肝脏穿刺活检的免疫组化染色结果被诊断为肝血管肉瘤。对比增强超声（CEUS）有效提高了该病例肝脏穿刺活检的准确性。本报告描述了在这名年轻男性中发现的罕见肝血管肉瘤的流行病学、临床表现、实验室检查、影像学表现和病理特征。患者迅速出现严重的腹腔出血。给予了经导管肝动脉栓塞、输血及对症治疗。由于患者病情危急且无法接受抗肿瘤治疗，家属放弃了治疗。
本病例强调了病理结果在PHA诊断中的重要性，尤其是在非高危个体中，以及CEUS在指导肝脏穿刺活检方面的支持作用。腹腔出血是PHA的严重并发症之一，对于控制肿瘤破裂引起的危及生命的出血应考虑行经动脉栓塞（TAE）治疗。需要进一步研究以早期诊断并改善PHA患者的预后。
