Presacral tumors of the Currarino triad: teratomas or hamartomas?

Abstract

Purpose: The aim was to elucidate the nature of the presacral tumors in the Currarino triad through studying their preoperative radiological anatomy and histopathology of excised specimens.

Patients and methods: The study group included three operated cases of Currarino triad. All were women who presented with constipation and demonstrated the typical three components of the disease: anorectal anomaly, sacral bony defect, and presacral tumor. The histopathological slides of excised specimens (presacral tumors) were available for re-examination. For comparison, we included another ‘control’ group representing the standard sacrococcygeal teratomas (without vertebral or anorectal anomalies).

Results: Histopathological examination of presacral tumors in the Currarino triad showed multicystic spaces lined by different types of epithelia mainly keratinized stratified squamous epithelium with focal areas of transitional epithelium. The underlying stroma showed fibrovascular connective tissue admixed with randomly arranged smooth muscle bundles. In contrast to the standard sacrococcygeal teratomas, neither skin adnexal structures nor heterologous mesenchymal tissues were observed; no immature elements could be detected.

Conclusion: In the Currarino triad, several clinical and histopathological observations would suggest the excised presacral tumors to be developmental cysts (retrorectal hamartomas) rather than neoplastic teratomas.

Keywords: Currarino triad, hamartoma, sacrococcygeal, tailgut, teratoma