Tsuchiya Naoki, Imai Yuichi, Cho Tamaki, Ogawara Yuki, Mizushima Taichi, Takase Hiroki, Fujii Satoshi, Miyagi Etsuko
Obstetrics and Gynecology, Yokohama City University Graduate School of Medicine, Yokohama, JPN.
Molecular Pathology, Yokohama City University Graduate School of Medicine, Yokohama, JPN.
Cureus. 2025 Jan 3;17(1):e76849. doi: 10.7759/cureus.76849. eCollection 2025 Jan.
Primary ovarian angiosarcoma is an extremely rare malignant soft tissue tumor that arises from the vascular endothelium, with a limited number of reports. It has no specific symptoms, and it is rarely diagnosed in the early stages because of rapid progression. Furthermore, no standard drug therapy is available, and the prognosis is poor. The patient was a 68-year-old woman suspected of having a ruptured ovarian tumor on plain abdominal computed tomography and anemia was observed. An emergency laparotomy was performed to stop the bleeding. Thereafter, one transcatheter arterial embolization, three laparotomies, and local radiotherapy were performed in an attempt to achieve hemostasis. However, the intraperitoneal bleeding remained intractable, and the transfusion was terminated after thorough consultation with the patient. The patient died 44 days after admission. Autopsy findings confirmed the diagnosis of primary ovarian angiosarcoma and pulmonary metastasis. Intractable intraperitoneal hemorrhage similar to that observed in this case has been reported, and because the prognosis is extremely poor, establishing an appropriate treatment regimen is desirable. Previously, the most commonly used drug regimens included anthracyclines and ifosfamide. However, recently, the possibility of treatment with molecular targeted agents and immune checkpoint inhibitors has been reported. In the future, case accumulation and genetic analyses will be required to establish appropriate treatment methods.
原发性卵巢血管肉瘤是一种极其罕见的恶性软组织肿瘤，起源于血管内皮，相关报道数量有限。它没有特定症状，由于进展迅速，很少在早期被诊断出来。此外，目前尚无标准的药物治疗方法，预后较差。该患者为一名68岁女性，腹部平扫计算机断层扫描怀疑有卵巢肿瘤破裂，并观察到贫血。进行了急诊剖腹手术以止血。此后，进行了一次经导管动脉栓塞、三次剖腹手术和局部放疗，试图实现止血。然而，腹腔内出血仍然难以控制，在与患者充分协商后停止了输血。患者入院44天后死亡。尸检结果证实为原发性卵巢血管肉瘤并伴有肺转移。已有报道称存在与该病例中观察到的类似的难治性腹腔内出血，并且由于预后极差，因此需要制定合适的治疗方案。以前，最常用的药物方案包括蒽环类药物和异环磷酰胺。然而，最近有报道称使用分子靶向药物和免疫检查点抑制剂进行治疗的可能性。未来，需要积累病例并进行基因分析以建立合适的治疗方法。
