Sacrococcygeal Teratoma (SCT) Publications

Optimizing surveillance strategies for sacrococcygeal teratoma: A Midwest pediatric surgery consortium multi-institutional study

Srivatsa S, Gil L, Zhang Y, Rymeski B, Gavulic A, Mak G, Mannava SV, Markel TA, Landman MP, Lal DR, Schuh J, Joshi D, Spencer B, Gadepalli S, Goldstein SD, Ranalli M, Van Arendonk KJ, Minneci P, Aldrink JH. Optimizing surveillance strategies for sacrococcygeal teratoma: A Midwest pediatric surgery consortium multi-institutional study. Int J Cancer. 2025 Nov 15;157(10):2103-2113. doi: 10.1002/ijc.70010. Epub 2025 Jun 19. PMID: 40536476.

  • Abstract

    Sacrococcygeal teratomas (SCTs) are the most common germ cell tumors in neonates and infants. While typically benign, SCTs carry an undefined risk of recurrence and malignant transformation, making post-resection surveillance critical. However, no consensus guidelines exist to direct post-resection surveillance. We conducted a multi-institutional retrospective cohort study across 11 pediatric institutions in the Midwest Pediatric Surgery Research Consortium. The study included patients 18 years or younger who underwent SCT resection from January 2010 to December 2020, excluding those with Currarino syndrome. The primary outcome was SCT recurrence, assessed via clinical exams, imaging, and tumor markers. Secondary outcomes included recurrence histology and surveillance practices. Cox proportional hazards modeling evaluated recurrence risk factors. Of the 178 patients, 10% experienced recurrence during a median follow-up period of 2.88 years (IQR: 1.52, 4.80). Overall recurrence-free survival for the entire cohort was 93.7%, 88.8%, and 88.8% at 1, 3, and 5 years, respectively. Malignant histopathology was the only factor significantly associated with recurrence (HR 5.83, p = .014). The timing of SCT diagnosis, completeness of resection, and Altman classification were not significantly associated with recurrence. Surveillance strategies varied significantly across institutions, with no standardized protocol for follow-up. The majority of recurrences occurred within the first 3 years post-resection, with malignant histopathology being the strongest predictor. For lower risk tumors (mature and immature teratomas), a minimum of 3 years of surveillance, including imaging, tumor markers, and clinical exams, is recommended. Standardized surveillance protocols could improve consistency and early detection.

    Cite

    Srivatsa S, Gil L, Zhang Y, Rymeski B, Gavulic A, Mak G, Mannava SV, Markel TA, Landman MP, Lal DR, Schuh J, Joshi D, Spencer B, Gadepalli S, Goldstein SD, Ranalli M, Van Arendonk KJ, Minneci P, Aldrink JH. Optimizing surveillance strategies for sacrococcygeal teratoma: A Midwest pediatric surgery consortium multi-institutional study. Int J Cancer. 2025 Nov 15;157(10):2103-2113. doi: 10.1002/ijc.70010. Epub 2025 Jun 19. PMID: 40536476.

Sacrococcygeal Teratomas in Currarino Syndrome: A Multicenter Review of Tumor Characteristics, Surgical Outcomes, and Recurrence

Srivatsa S, Gil L, Zhang Y, Rymeski B, Gavulic A, Mak G, Mannava SV, Markel TA, Landman MP, Lal DR, Schuh J, Joshi D, Spencer B, Gadepalli S, Goldstein SD, Ranalli M, Minneci P, Van Arendonk K, Aldrink JH. Sacrococcygeal Teratomas in Currarino Syndrome: A Multicenter Review of Tumor Characteristics, Surgical Outcomes, and Recurrence. J Pediatr Surg. 2025 Sep;60(9):162420. doi: 10.1016/j.jpedsurg.2025.162420. Epub 2025 Jun 18. PMID: 40541662.

  • Abstract

    Background: Currarino syndrome is a rare congenital condition characterized by a triad of anorectal malformation, sacral agenesis, and presacral mass, often a teratoma. Comparative outcomes of sacrococcygeal teratomas (SCTs) in Currarino versus non-syndromic cases are not well defined.

    Methods: A multicenter retrospective review of pediatric SCT resections from 2010 to 2020 was conducted across 11 institutions in the Midwest Pediatric Surgery Consortium. Patients were classified based on the presence or absence of Currarino syndrome. Demographic, surgical, pathologic, and long-term outcome data were analyzed. The primary outcome was tumor recurrence.

    Results: Of 203 patients, 25 (12.3 %) had Currarino syndrome. Currarino patients were more often diagnosed postnatally (80 % vs. 25 %, p < 0.001) and had predominantly Altman type IV tumors (87 % vs. 17 %, p < 0.001). All tumors in the Currarino cohort were mature teratomas, while 27 % of non-Currarino tumors were immature and 12 % were malignant (p < 0.001). Tumors in Currarino patients were significantly smaller in size (median 3.3 cm vs. 8.0 cm, p < 0.001). Recurrence rates were low and comparable (4 % Currarino vs. 10 % non-Currarino, p = 0.18). Currarino patients had higher rates of urinary incontinence (44 % vs. 28 %, p = 0.048) and constipation (76 % vs. 32 %, p < 0.001). Kaplan-Meier analysis showed a trend toward improved recurrence-free survival in Currarino patients, though not statistically significant.

    Conclusions: Pediatric patients with Currarino-associated SCTs have excellent long-term outcomes, with low recurrence rates likely attributable to benign tumor histology and high rates of complete resection. These findings suggest that surveillance strategies may be tailored for Currarino patients to reduce unnecessary imaging and long-term follow-up burden.

    Study design: Retrospective cohort study.

    Level of evidence: III.

    Keywords: Currarino syndrome; Sacrococcygeal teratoma; Surveillance.

    Cite

    Srivatsa S, Gil L, Zhang Y, Rymeski B, Gavulic A, Mak G, Mannava SV, Markel TA, Landman MP, Lal DR, Schuh J, Joshi D, Spencer B, Gadepalli S, Goldstein SD, Ranalli M, Minneci P, Van Arendonk K, Aldrink JH. Sacrococcygeal Teratomas in Currarino Syndrome: A Multicenter Review of Tumor Characteristics, Surgical Outcomes, and Recurrence. J Pediatr Surg. 2025 Sep;60(9):162420. doi: 10.1016/j.jpedsurg.2025.162420. Epub 2025 Jun 18. PMID: 40541662.