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A tumor complex consisting of presacral teratoma and sacral deformity was described along with its occurrence in 6 kindreds. In addition to the tumor and the sacral defect, some patients have had recurrent abscesses, rectal stenosis and skin dimples as manifestation of the tumor. Urinary tract anomalies have also been found to be associated in some patients. The tumor differed considerably from the usual sacrococcygeal teratoma. Only one of the 19 resected tumors had proved to be malignant, although 2 have recurred in a benign manner. This appears to be inherited as a dominant characteristic.

Radiographic findings and related information are described for a series of 26 patients from six unrelated families. The tumor complex consists of a presacral teratoma and a sacrococcygeal bony defect occurring in a familial pattern. Many of the patients also had an associated vesicoureteral reflux, skin dimples, retrorectal abscess, and anorectal stenosis. The main radiographic features were the presence of a unilateral or central developmental deformity of the sacrum and coccyx, and the absence of radiographically visible soft-tissue calcifications.