血管肉瘤（AS）是一种罕见且高度侵袭性的软组织疾病，最常发生在深部软组织中。有极少数报道的卵巢AS病例，甚至更少有关分子异常的报道。在这里，我们简要回顾了两例原发性卵巢AS（oAS）病例的特定分子事件和免疫检查点。基于文献回顾，讨论了该疾病的临床特征和预后、诊断、鉴别诊断以及新的治疗方法。病例1：一名51岁女性患者因右下肢疼痛5个月，下腹疼痛伴血尿1个月入院。进行肌腹直肌及筋膜部分切除、部分子宫切除、双侧输卵管卵巢切除术、腹股沟及盆腔淋巴结清扫术。病理学显示为原发性oAS。荧光原位杂交显示c-MYC基因扩增。施行MESNA + ADM + IFO + DTIC方案，但病情稳定。患者1个月后死亡。病例2：一名41岁女性患者出现疲劳、恶心、食欲下降和弥漫性腹痛。体格检查发现腹部膨隆，右侧盆腔可触及复杂囊性肿块。病理学显示为原发性oAS。施行MAID化疗，并对肿瘤样本进行PD-L1染色。患者从抗PD-1免疫治疗中受益，在随访期间停药27个月，仍然没有任何疾病证据。通过使用病理学指标来指导治疗，可以实现原发性oAS的长期生存益处。©作者2023。百世登出版集团拥有所有权利。保留所有权利。

Angiosarcoma (AS) is a rare and highly aggressive soft tissue disease that most commonly arises in deep soft tissues. There are only a few reported cases of AS involving the ovary and even fewer reports of the underlying molecular abnormalities. Here, we briefly review two cases of primary ovarian AS (oAS) with specific molecular events and immune checkpoints. The clinical features and prognosis of the disease, diagnosis, differential diagnosis, and new treatment approaches are discussed based on a literature review.Case 1: A 51-year-old female patient was admitted with right lower limb pain for 5 mo, and lower abdominal pain with hematuria for 1 mo. Partial removal of rectus abdominis muscle and fascia, partial hysterectomy, bilateral salpingo-oophorectomy, and inguinal and pelvic lymphadenectomy were performed. Pathology revealed primary oAS. Fluorescence in situ hybridization revealed c-MYC gene amplification. MESNA + ADM + IFO + DTIC (MAID) regimen was administered, but stable disease was achieved. The patient died 1 mo later. Case 2: A 41-year-old female patient presented with fatigue, nausea, decreased appetite, and diffuse abdominal pain. On physical examination, the abdomen was distended and a complex cystic mass was palpable in the right pelvic cavity. Pathology revealed primary oAS. MAID chemotherapy was administered and programmed death ligand 1 (PD-L1) staining was performed on the tumor samples. The patient benefited from anti-PD-1 immunotherapy and is alive without any evidence of disease 27 mo off therapy in follow-up.Long-term survival benefit for primary oAS can be achieved by alternative therapeutic strategies using pathological indicators to inform treatment.©The Author(s) 2023. Published by Baishideng Publishing Group Inc. All rights reserved.