Shachi Srivatsaa∙ Lindsay Gila ∙ Yueran Zhang ∙ Beth Rymeski ∙ Amelia Gavulic ∙ Grace Mak ∙ Sindhu V. Mannava ∙ Troy A. Markel ∙ Matthew P. Landman ∙ Dave R. Lal ∙ Jennifer Schuh ∙ Devashish Joshi ∙ Brianna Spencer ∙ Samir Gadepalli ∙ Seth D. Goldstein ∙ Mark Ranalli ∙ Peter Minneci ∙ Kyle Van Arendonk ∙ Jennifer H. Aldrink
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.
Intended audience: Healthcare professionals and clinicians.
What if the sacrococcygeal teratomas that we worry most about are actually the least aggressive ones? I'm Lizzie Lee from Cincinnati Children's, and this is an article you should know about. This multi-center review looked at over 200 sacrococcygeal teratoma cases and compared kids with Currarino syndrome to those without it. The differences are dramatic. Currarino patients were almost always diagnosed after birth, and their tumors were almost exclusively Altman type IV buried deep in the pelvis. Every tumor in the Currarino group was a mature one. There were no immature or malignant components at all. They were also much smaller, around 3 centimeters instead of 8. Recurrence was almost nonexistent and not significantly different from non-Currarino cases. So the message here is that Currarino-associated sacrococcygeal teratomas behave incredibly well oncologically, which means we may be able to rethink how aggressively we follow these kids long-term. Let us know what you think in the comments below and stay tuned for more articles that you should know about.
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