Markedly improved survival in malignant sacro-coccygeal teratomas--16 years, experience

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.