Diagnosis, management and outcome of teratomas in neonates and infants: A multi-institutional study

Purpose: Teratomas, the most common tumor affecting neonates, has an estimated incidence of 1 in 20,000 to 30,000 live births. The study was designed to determine the effect of therapy on the long-term outcome of neonates and infants with teratomas. Methods: A retrospective analysis was conducted of neonates and small infants with teratomas treated from 1972-1997 at 17 Children's Hospitals in the United States. Results: Teratomas, the most common neoplasm in neonates and young infants accounted for over 40% of all solid tumors observed in this age population. In this study, 158 children were diagnosed with teratomas. The sacrococcygeal location was predominant with 131 (84%). Sacrococcygeal teratomas (SCT) were prenatally diagnosed in 33, at birth in 82 and later in infancy in 16. There were 97 females and 34 males. At presentation, 70% of SCT were mature teratoma, 20% were immature teratoma and 10% had endodermal sinus tumor (EST). Mean follow-up was 7 years. Five percent of the infants died within the perioperative period. Nine patients (10%) who had mature SCT developed recurrent disease; 2 had locally recurring mature teratoma and 7 had EST. One child with an immature SCT had recurrent tumor at a local site. Seven of 13 EST infants were disease free after chemotherapy (CTX) and 6 had received no adjunctive CTX. Two of the 6 later developed evidence of metastatic disease within 1 year and were salvaged with CTX (bleomycin, etoposide, vinblastine, cisplatin). Cervicofacial teratomas were diagnosed in 21 (13%). Fifteen neonates had cervical teratomas and 6 infants presented with orofacial teratomas. There were 12 males and 9 females. A diagnostic prenatal ultrasound was performed in 8 cases. Life-threatening airway obstruction occurred in 8 infants (38%) in the early postnatal period. Three neonates died in the delivery room without ever having their airway secured. Two other prenatally diagnosed infants survived only because tracheostomies were performed by pediatric surgeons who were in the delivery room. Three others had oral intubation, one after sustaining a hypoxic cardiac arrest. Eighteen infants had their primary tumor excised. After resection 2 patients had evidence of unilateral recurrent laryngeal nerve injury, and 2 required thyroid hormone replacement. Histology revealed 8 mature and 7 immature teratomas. Four infants (18%) clearly had malignant lesions. Pulmonary metastases occurred in 2 children and contributed to one late death at 6 months. Overall survival was 77% with a mean follow-up of 6 years (range 2-16 years). Twelve of 17 (70%) surviving patients have had excellent functional and cosmetic outcomes. Four children have varying degrees of developmental delay and mental retardation. Hypoxia at birth was felt to have contributed to these problems in 2. Four retroperitoneal and 2 mediastinal tumors were diagnosed and all infants survived surgical therapy. Conclusions: 1. Prenatal diagnosis is a reliable and essential method of detecting these neoplasms in utero. 2. Stabilization and early resection has reduced the mortality in operative cases to a minimum. 3. Surgical resection alone is adequate therapy for non-metastatic malignant tumors. 4. Life-threatening airway obstruction at birth is common in infants with cervicofacial teratomas. 5. Pediatric surgeons should be available in the delivery room to offer these infants the best chance for survival. 6. Benign teratomas have a significant recurrence rate that mandates close follow-up for more than 3 years. 7. The risk of malignant transformation warrants early surgery and long-term surveillance. 8. Survival for malignant lesions with metastases is excellent with CTX.