Congenital masses of the sacrococcygeal region commonly contain multip
le tissues and have variously been subclassified as neoplasms or conge
nital hamartomas based on clinicopathological and embryological observ
ations. We have used a polymerase chain reaction-based assay for nonra
ndom X chromosome inactivation to infer the clonality of three congeni
tal sacrococcygeal tumors previously diagnosed as teratomas. One solid
immature teratoma was monoclonal, and a predominantly cystic histolog
ically mature mass was polyclonal. A third immature teratoma was nonin
formative because of baseline asymmetry of polyclonal tissue X inactiv
ation. We confirm that immature teratomas at this site appear to be mo
noclonal neoplasms and suggest that at least some histologically matur
e ''teratomas'' are more appropriately classified as hamartomas.