Soufi M, Lupinacci R M, Godiris-Petit G, Vignot S, Genestie C, Menegaux F, Lefranc J P
Eur J Gynaecol Oncol. 2015;36(4):473-6.
Growing teratoma syndrome (GTS) is a rare condition among patients with non-seminomatous germ cell tumors who present with enlarging metastatic masses during appropriate systemic chemotherapy in the context of normalized serum markers. This is an infrequent event in the progression of testicular tumors, and is even less common in the case of ovarian germ cell tumors. The pathogenesis of GTS is not completely understood and diagnosis can only be made with certainty after complete pathologic examination. Although histologically benign, GTS may present an enveloping growth with aggressive local expansion, which can be related to substantial morbidity and mortality. Surgery is the only recommended treatment and early recognition of this syndrome is essential as it offers hope for curative resection and avoids the use of ineffective chemotherapy. The authors present a brief review of the literature, along with the case report of a 37-year-old woman presenting GTS with liver involvement who was successfully treated by debulking surgery followed by major liver resection. This report demonstrates that complete surgical resection results in excellent disease control. More importantly, it highlights that clinicians need to be aware of the possible development of GTS when monitoring their patients with non-seminomatous germ cell tumors. These patients require coordinated care between oncologist, gynecologists, and general surgeons to obtain the best possible outcomes.
生长性畸胎瘤综合征(GTS)在非精原细胞性生殖细胞肿瘤患者中较为罕见,这些患者在血清标志物恢复正常的情况下接受适当的全身化疗时出现转移性肿块增大。这在睾丸肿瘤进展中是一种罕见情况,在卵巢生殖细胞肿瘤中更为少见。GTS的发病机制尚未完全明确,只有在完整的病理检查后才能确诊。尽管组织学上为良性,但GTS可能呈现侵袭性局部扩展的包绕性生长,这可能导致显著的发病率和死亡率。手术是唯一推荐的治疗方法,早期识别该综合征至关重要,因为这为根治性切除带来希望,并避免使用无效的化疗。作者对文献进行了简要综述,并报告了一例37岁患有GTS且肝脏受累的女性病例,该患者通过减瘤手术及随后的肝脏大部切除术成功治愈。本报告表明,完整的手术切除可实现良好的疾病控制。更重要的是,它强调临床医生在监测非精原细胞性生殖细胞肿瘤患者时需要意识到GTS可能发生发展。这些患者需要肿瘤学家、妇科医生和普通外科医生之间的协调护理,以获得最佳治疗效果。