Misra D, Pritchard J, Drake D P, Kiely E M, Spitz L
Department of Paediatric-Surgery and Haematology-Oncology, Hospital for Sick Children, London, UK.
Eur J Pediatr Surg. 1997 Jun;7(3):152-5. doi: 10.1055/s-2008-1071078.
To see the impact of cisplatin- and carboplatin-based protocols on survival in malignant sacrococcygeal teratomas (SCT).
Twenty infants and children with malignant SCT were treated at the Hospital for Sick Children, Great Ormond Street, London, over a 16-year period from 1979 to 1994. There were 5 males and 15 females, age ranged from 1 day to 3.5 years (mean 18 months).
Twelve patients (60%) had distant metastasis: 9 lung metastasis, 2 liver involvement, 2 bony metastases, 1 cerebral metastasis and 1 bilateral inguinal lymph node deposits. From 1982 to 1986, patients received the BEP chemotherapy protocol which included cis-platinum, bleomycin and etoposide (VP 16). After 1986, cis-platinum was replaced with carboplatin in the new JEB protocol. Patient 1 did not receive any chemotherapy, Patients 2-4 received varying protocols (2 deaths), Patients 5-8 received the BEP regime (1 death) and Patients 9-20 received JEB (1 death). The first three deaths were due to uncontrolled local disease and/or metastasis, while the latter death was due to bleomycin toxicity. Overall, 9 of 12 (75%) patients with distant metastasis survived as opposed to 7 of 8 (88%) patients with localised disease. Of the 12 patients who received the JEB protocol, 11 (92%) survived, including 7 patients with metastatic disease and 2 with local recurrence. Seven patients (35%) had relapse while on treatment or follow-up, 4 of these are disease-free with further therapy. In 6 of these children, serum AFP rose before there was clinical or radiological evidence of relapse. In 2 other patients, further chemotherapy was recommenced solely on the basis of rising serum AFP, these patients did not subsequently develop overt metastasis.
We conclude that the treatment of choice for malignant SCT is the JEB regime, to be given for 4 courses or to be continued for 2 courses beyond documented Complete Response (CR). Excision of the primary tumour and coccyx should be done in all cases even if a CR has been documented. Metastases not responding to chemotherapy would need appropriate surgery, radiotherapy is hardly ever needed. An overall cure rate exceeding 90% can be expected.
观察基于顺铂和卡铂的治疗方案对恶性骶尾部畸胎瘤（SCT）患者生存率的影响。
1979年至1994年期间，伦敦大奥蒙德街儿童医院收治了20例患有恶性SCT的婴幼儿及儿童。其中男性5例，女性15例，年龄从1天至3.5岁（平均18个月）。
12例患者（60%）发生远处转移：9例肺转移，2例肝脏受累，2例骨转移，1例脑转移，1例双侧腹股沟淋巴结转移。1982年至1986年，患者接受BEP化疗方案，包括顺铂、博来霉素和依托泊苷（VP16）。1986年后，新的JEB方案中顺铂被卡铂取代。患者1未接受任何化疗，患者2 - 4接受不同方案治疗（2例死亡），患者5 - 8接受BEP方案（1例死亡），患者9 - 20接受JEB方案（1例死亡）。前三例死亡是由于局部疾病和/或转移未得到控制，而最后一例死亡是由于博来霉素毒性。总体而言，12例发生远处转移的患者中有9例（75%）存活，而8例局限性疾病患者中有7例（88%）存活。接受JEB方案的12例患者中，11例（92%）存活，包括7例转移性疾病患者和2例局部复发患者。7例患者（35%）在治疗或随访期间复发，其中4例经进一步治疗后无疾病状态。在其中6例儿童中，血清甲胎蛋白（AFP）在临床或影像学出现复发证据之前升高。在另外2例患者中，仅根据血清AFP升高就重新开始进一步化疗，这些患者随后未发生明显转移。
我们得出结论，恶性SCT的首选治疗方案是JEB方案，应给予4个疗程，或在记录到完全缓解（CR）后继续2个疗程。即使已记录到CR，所有病例均应切除原发肿瘤和尾骨。对化疗无反应的转移灶可能需要适当的手术治疗，几乎不需要放疗。预计总体治愈率超过90%。
