Department of Traditional Chinese Medicine, Shanghai Changzheng Hospital, Second Military Medical University, 415 Fengyang Road, Shanghai, 200003, China.
J Neurooncol. 2012 Aug;109(1):205-11. doi: 10.1007/s11060-012-0865-9. Epub 2012 Apr 19.
Malignant peripheral nerve sheath tumors (MPNSTs) are usually located in the trunk, extremities, head, or neck, and most occur with neurofibromatosis type 1 (NF1; von Recklinghausen's disease). No biomarkers have previously been found to be associated with their progression. Retroperitoneal NF1-independent MPNSTs are rare; they are considered to be less aggressive and to have better prognoses compared to NF1-related tumors. Currently, en bloc excision is the only consensus treatment approach. In a 27-year-old male with a giant retroperitoneal MPNST and no stigmata or family history of neurofibromatosis type-1 (NF1), a remarkable elevation of serum CA125 was detected. The high-grade tumor displayed a striking progression: the primary lesion, 25 cm in diameter, recurred in its previous site as a 17-cm MPNST less than 50 days after total excision. Subsequent treatment with microwave ablation and huachansu, a traditional Chinese medication, proved ineffective, and the patient died within 3 months. Our case suggests that retroperitoneal MPNSTs can deteriorate rapidly even if NF1 independent, that aggressive treatment may not benefit large high-grade MPNSTs, and that novel and effective treatment is urgently needed. Our case also suggests the possibility of using serum tumor markers in the early detection and monitoring of MPNSTs.
恶性外周神经鞘瘤 (MPNST) 通常位于躯干、四肢、头部或颈部，大多数发生于神经纤维瘤病 1 型 (NF1；冯·雷克林豪森氏病)。以前没有发现与肿瘤进展相关的生物标志物。腹膜后 NF1 不相关的 MPNST 很少见；与 NF1 相关的肿瘤相比，它们被认为侵袭性较低，预后较好。目前，整块切除是唯一的共识治疗方法。在一名 27 岁的男性中，患有巨大的腹膜后 MPNST，没有 NF1（神经纤维瘤病 1 型）的体征或家族史，检测到血清 CA125 显著升高。高级别肿瘤表现出显著的进展：原发性病变，直径 25 厘米，在完全切除后不到 50 天就在其先前部位复发为 17 厘米的 MPNST。随后采用微波消融和华蟾素（一种中药）治疗，但无效，患者在 3 个月内死亡。我们的病例表明，即使是腹膜后 MPNST 也可能迅速恶化，即使 NF1 不相关，积极治疗可能对大型高级别 MPNST 无益，迫切需要新的有效治疗方法。我们的病例还表明，血清肿瘤标志物在 MPNST 的早期检测和监测中具有可能性。
