Perinatal Prognostic Factors of Recurrence or Functional Sequelae in Neonatal Sacrococcygeal Teratoma, and Implications for Prenatal Counselling: A Multicenter Retrospective Study

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OBJECTIVE: To evaluate the risk of recurrence and functional sequelae in sacrococcygeal teratoma (SCT). METHODS: Children with fetal/neonatal SCT (diagnosis <6 months) managed in five different centers (January 2007-Decempber 2017) were retrospectively included (NCT04623658). Syndromic and malignant SCT were excluded. Data from prenatal diagnosis, birth, neonatal surgery, recurrence and functional sequelae (digestive, urinary, orthopedic, cosmetic) were analyzed. Clinical follow-up was reviewed at one, three, ten years and at last follow-up, and occurrence of SCT recurrence or functional sequelae was noted. Digestive sequelae were defined as constipation ≥grade 2, soiling ≥grade 2 or the need for transanal irrigation (Krickenbeck classification). Urinary sequelae were defined as the presence of a neurogenic bladder. RESULTS: Fifty-five children (80%) survived the neonatal period, with a prenatal diagnosis of SCT in 75% of cases. All of them survived to last follow-up. Recurrence occurred in 11% of cases (median age: 25 months), including 50% of malignancy. No significant association was found between tumor recurrence and patients/tumor characteristics or perinatal course. After a median follow-up of 4.9 years [3.4-7.4], 22% (n=12/55) of children had functional sequelae: digestive (22%), urinary (7%), orthopedic (4%), cosmetic (9%). Endopelvic tumors with prenatal signs of urinary tract compression were associated with neurogenic bladder. Children with neurogenic bladder were more likely to have digestive sequelae. CONCLUSION: This study highlights the importance of addressing the three distinct risks of fetal SCT during prenatal counselling: perinatal life-threatening risk, risk of recurrence warranting clinical, radiological and biological monitoring, and long-term risk of functional sequelae. LEVEL OF EVIDENCE: III.

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