Tumor metrics and morphology predict poor prognosis in prenatally diagnosed sacrococcygeal teratoma: A 25-year experience at a single institution

      Abstract

      Purpose

      Some fetuses with sacrococcygeal teratoma (SCT) develop hydrops, but there is no consensus on an appropriate prognostic marker for poor prognosis. The purpose of this study is to establish predictors of poor prognosis in fetuses with SCT.

      Methods

      A retrospective review of patients with prenatally diagnosed SCT from 1986 to 2011 was performed. Patients with outcome data and ultrasound exams before 32 weeks gestational age (GA) were included (n = 37). Tumor volume-to-fetal weight ratio (TFR) and tumor morphology were assessed as sonographic predictors of poor prognosis.

      Results

      Twelve patients (32%) had good prognosis, and twenty-five patients (68%) had poor prognosis. All patients with poor prognosis had a morphology score ≥3, which is a significant predictor of poor prognosis (p <0.0001). TFR was assessed, and a receiver operating characteristic (ROC) analysis identified a cutoff value of 0.12 before 24 weeks GA and 0.11 before 32 weeks GA as predictors for poor prognosis. TFR is a significant predictor of poor prognosis (p < 0.0001).

      Conclusions

      Patients with cystic SCT all had good prognosis. TFR >0.12 was validated as a sonographic predictor of poor prognosis. TFR and tumor morphology can be used to counsel expectant families with prenatally diagnosed SCT regarding prognosis.

      Key words

      To read this article in full you will need to make a payment

      Purchase one-time access:

      Academic and Personal

      Subscribe:

      Subscribe to Journal of Pediatric Surgery
      Already a print subscriber? Claim online access
      Already an online subscriber? Sign in
      Institutional Access: Sign in to ScienceDirect

      References

        • Winderl L.M.
        • Silverman R.K.
        Prenatal identification of a completely cystic internal sacrococcygeal teratoma (type IV).
        Ultrasound Obstet Gynecol. 1997; 9: 425-428
        • Gucciardo L.
        • Uyttebroek A.
        • De Wever I.
        • et al.
        Prenatal assessment and management of sacrococcygeal teratoma.
        Prenat Diagn. 2011; 31: 678-688
        • Bond S.J.
        • Harrison M.R.
        • Schmidt K.G.
        • et al.
        Death due to high-output cardiac-failure in fetal sacrococcygeal teratoma.
        J Pediatr Surg. 1990; 25: 1287-1291
        • Flake A.W.
        • Harrison M.R.
        • Adzick N.S.
        • et al.
        Fetal sacrococcygeal teratoma.
        J Pediatr Surg. 1986; 21: 563-566
        • Holterman A.X.
        • Filiatrault D.
        • Lallier M.
        • et al.
        The natural history of sacrococcygeal teratomas diagnosed through routine obstetric sonogram: a single institution experience.
        J Pediatr Surg. 1998; 33: 899-903
        • Adzick N.S.
        Open fetal surgery for life-threatening fetal anomalies.
        Semin Fetal Neonatal Med. 2010; 15: 1-8
        • Milner R.
        • Adzick N.S.
        Perinatal management of fetal malformations amenable to surgical correction.
        Curr Opin Obstet Gynecol. 1999; 11: 177-183
        • Westerburg B.
        • Feldstein V.A.
        • Sandberg P.L.
        • et al.
        Sonographic prognostic factors in fetuses with sacrococcygeal teratoma.
        J Pediatr Surg. 2000; 35: 322-325
        • Wilson R.D.
        • Hedrick H.
        • Flake A.W.
        • et al.
        Sacrococcygeal teratomas: prenatal surveillance, growth and pregnancy outcome.
        Fetal Diagn Ther. 2009; 25: 15-20
        • Sy E.D.
        • Filly R.A.
        • Cheong M.L.
        • et al.
        Prognostic role of tumor-head volume ratio in fetal sacrococcygeal teratoma.
        Fetal Diagn Ther. 2009; 26: 75-80
        • Rodriguez M.A.
        • Cass D.L.
        • Lazar D.A.
        • et al.
        Tumor volume to fetal weight ratio as an early prognostic classification for fetal sacrococcygeal teratoma.
        J Pediatr Surg. 2011; 46: 1182-1185
        • Neubert S.
        • Trautmann K.
        • Tanner B.
        • et al.
        Sonographic prognostic factors in prenatal diagnosis of SCT.
        Fetal Diagn Ther. 2004; 19: 319-326
        • Schmidt K.G.
        • Silverman N.H.
        • Harrison M.R.
        • et al.
        High-output cardiac failure in fetuses with large sacrococcygeal teratoma: diagnosis by echocardiography and Doppler ultrasound.
        J Pediatr. 1989; 114: 1023-1028
        • Silverman N.H.
        • Schmidt K.G.
        Ventricular volume overload in the human fetus: observations from fetal echocardiography.
        J Am Soc Echocardiogr. 1990; 3: 20-29
        • Rychik J.
        Fetal cardiovascular physiology.
        Pediatr Cardiol. 2004; 25: 201-209
        • Byrne F.A.
        • Lee H.
        • Kipps A.K.
        • et al.
        Echocardiographic risk stratification of fetuses with sacrococcygeal teratoma and twin-reversed arterial perfusion.
        Fetal Diagn Ther. 2011; 30: 280-288
        • Falkensammer C.B.
        • Paul J.
        • Huhta J.C.
        Fetal congestive heart failure: correlation of Tei-index and cardiovascular-score.
        J Perinat Med. 2001; 29: 390-398
        • Hofstaetter C.
        • Hansmann M.
        • Eik-Nes S.H.
        • et al.
        A cardiovascular profile score in the surveillance of fetal hydrops.
        J Matern Fetal Neonatal Med. 2006; 19: 407-413
        • Hedrick H.L.
        • Flake A.W.
        • Crombleholme T.M.
        • et al.
        Sacrococcygeal teratoma: prenatal assessment, fetal intervention, and outcome.
        J Pediatr Surg. 2004; 39 ([discussion 430–8]): 430-438
        • Makin E.C.
        • Hyett J.
        • Ade-Ajayi N.
        • et al.
        Outcome of antenatally diagnosed sacrococcygeal teratomas: single-center experience (1993–2004).
        J Pediatr Surg. 2006; 41: 388-393
        • Ruano R.
        • Duarte S.
        • Zugaib M.
        Percutaneous laser ablation of sacrococcygeal teratoma in a hydropic fetus with severe heart failure—too late for a surgical procedure?.
        Fetal Diagn Ther. 2009; 25: 26-30
        • Brace V.
        • Grant S.R.
        • Brackley K.J.
        • et al.
        Prenatal diagnosis and outcome in sacrococcygeal teratomas: a review of cases between 1992 and 1998.
        Prenat Diagn. 2000; 20: 51-55
        • Roybal J.L.
        • Moldenhauer J.S.
        • Khalek N.
        • et al.
        Early delivery as an alternative management strategy for selected high-risk fetal sacrococcygeal teratomas.
        J Pediatr Surg. 2011; 46: 1325-1332
        • Rychik J.
        • Tian Z.
        • Cohen M.S.
        • et al.
        Acute cardiovascular effects of fetal surgery in the human.
        Circulation. 2004; 110: 1549-1556
        • Makikallio K.
        • Rasanen J.
        • Makikallio T.
        • et al.
        Human fetal cardiovascular profile score and neonatal outcome in intrauterine growth restriction.
        Ultrasound Obstet Gynecol. 2008; 31: 48-54