Li Hui, Wang Li, Wu Ying-Hong, Chen Gang, Li Hong-Xia, Fan Li-Fen, Gu Min, Jiang Cai-Hong
Department of Medical Oncology, Ordos Central Hospital, Ordos 017000, Inner Mongolia Autonomous Region, China.
Department of Pathology, Ordos Central Hospital, Ordos 017000, Inner Mongolia Autonomous Region, China.
World J Clin Cases. 2023 Nov 6;11(31):7673-7679. doi: 10.12998/wjcc.v11.i31.7673.
Malignant schwannoma is a rare tumor in the peripheral nervous system, accounting for approximately 5% to 10% of systemic soft tissue sarcomas. Especially, malignant schwannoma occurring in the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis is extremely rare in clinical practice. Here, we report the first case of an patient diagnosed with malignant peripheral nerve sheath tumor (MPNST) of the broad ligament of the uterus with hemophilic syndrome and bone marrow fibrosis, and share our reference clinical diagnosis and treatment experience.
A patient was diagnosed with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis. She received combination, and repeated imaging revealed further encountered rare complications (hemophilia syndrome and bone marrow fibrosis) after two cycles of chemotherapy. Thereafter, combined treatment with pazopanib, gemcitabine, and dacarbazine was initiated. Unfortunately, the patient succumbed to death at hospital after two weeks.
This report firstly provided reference clinical practice for a patient with MPNST of the uterus harboring hemophilic syndrome and bone marrow fibrosis. Our case raises a reminder about the tolerance and safety of combination therapy, especially in young women.
恶性神经鞘瘤是一种罕见的周围神经系统肿瘤，约占全身软组织肉瘤的5%至10%。特别是，发生于子宫阔韧带并伴有血友病综合征和骨髓纤维化的恶性神经鞘瘤在临床实践中极为罕见。在此，我们报告首例被诊断为子宫阔韧带恶性周围神经鞘膜瘤（MPNST）并伴有血友病综合征和骨髓纤维化的患者，并分享我们的临床诊断和治疗参考经验。
一名患者被诊断为患有伴有血友病综合征和骨髓纤维化的子宫MPNST。她接受了联合治疗，重复影像学检查显示在两个周期化疗后出现了罕见的并发症（血友病综合征和骨髓纤维化）。此后，开始使用帕唑帕尼、吉西他滨和达卡巴嗪进行联合治疗。不幸的是，患者在两周后于医院死亡。
本报告首次为患有伴有血友病综合征和骨髓纤维化的子宫MPNST患者提供了临床实践参考。我们的病例提醒人们注意联合治疗的耐受性和安全性，尤其是在年轻女性中。
