Department of Orthopedic Surgery, Keio University School of Medicine, 35 Shinanomachi, Shinjuku-ku, Tokyo, 160-8582, Japan.
Division of Thoracic Surgery, Department of Surgery, Keio University School of Medicine, Tokyo, Japan.
World J Surg Oncol. 2022 Jan 7;20(1):8. doi: 10.1186/s12957-021-02473-2.
Malignant peripheral nerve sheath tumor (MPNST) is a rare soft tissue sarcoma mainly treated via surgical resection. Herein, we report a case of MPNST wherein a massive tumor thrombus extended to the major veins and heart.
A 39-year-old female with a history of neurofibromatosis type 1 developed MPNST from the right radial nerve. In addition to adjuvant chemotherapy, she underwent wide tumor resection and concomitant radial nerve resection, followed by postoperative radiotherapy. Histological evaluation revealed marked venous invasion. The 2-year follow-up CT revealed an asymptomatic recurrent tumor thrombus extending from the right subclavian vein to the heart. An urgent life-saving operation was performed to ligate the base of the right subclavian vein and remove the entire intravenous thrombus that extended to the right ventricle. The remaining tumor in the right subclavian vein increased in size 3 months after thrombectomy. After confirming the absence of any metastatic lesions, the patient underwent extended forequarter amputation to achieve surgical remission. One year later, a new metastasis to the right diaphragm was safely resected. The patient remains alive without any evidence of disease 2 years after the extended forequarter amputation.
In cases of a previous history of microscopic venous invasion, recurrence can occur as a massive tumor thrombus that extends to the great vessels.
恶性外周神经鞘瘤（MPNST）是一种罕见的软组织肉瘤，主要通过手术切除进行治疗。在此，我们报告一例 MPNST 病例，该肿瘤内有大量肿瘤血栓延伸至大静脉和心脏。
一名 39 岁女性，患有 1 型神经纤维瘤病，源自右侧桡神经的 MPNST。除了辅助化疗外，她还接受了广泛的肿瘤切除术和桡神经切除术，随后进行了术后放疗。组织学评估显示明显的静脉侵犯。2 年的随访 CT 显示无症状的复发性肿瘤血栓从右锁骨下静脉延伸至心脏。为了进行紧急的救生手术，结扎了右锁骨下静脉的根部，并切除了延伸至右心室的整个静脉内血栓。血栓切除术后 3 个月，右锁骨下静脉内剩余肿瘤增大。在确认无任何转移病灶后，患者接受了前胸部扩大切除术以实现手术缓解。1 年后，安全地切除了右侧膈肌的新转移灶。在前胸部扩大切除术后 2 年，患者仍然存活且没有疾病迹象。
对于既往有镜下静脉侵犯史的患者，复发时可能会出现延伸至大血管的大量肿瘤血栓。
