Sacrococcygeal teratoma: clinical course and prognosis with a special viewto long-term functional results

From 1976 to 1995, 23 children, 4 boys and 19 girls, were treated at our de partment for sacrococcygeal teratomas (SCT). Their records were analyzed re trospectively, considering age at operation, histopathology, recurrences, a nd long-term evolution. One died on the Ist day of life following tumor rup ture with hemorrhagic shock without surgical intervention. All others were operated upon at a mean age of 4.2 days for those 19 (= 82%) who were diagn osed in the neonatal period and whose histology proved benign. In the remai ning 3 children, in whom tumor manifestation did not occur before 11 months , 13 months, and 10 years of age, respectively, histopathologic evaluation revealed 2 carcinomas and 1 yolk-sac tumor, and all 3 recurred. Overall, 5 patients died, the 1 mentioned above, I due to volvulus after laparotomy, a nd 1 from multiple associated congenital malformations. Two deaths were rel ated to malignancy, whereby only 1 was a malignant teratoma diagnosed at th e original operation. Eight children bad recurrences, 2 were benign and 6 m alignant, with 3 of the latter having been graded benign on histology of th e primary tumor. Of the 18 surviving patients, 17 (93.5%) returned for clin ical review following a standardized protocol. The average interval from th e primary surgery was 12.3 years (range 3.5-22 years). Four had malignant t umors with a recurrence-free period of from 9 to 14 years; 5 (29.4%) had ur inary or anorectal functional impairment. One child with a patulous anus pr esented with fecal soiling. Two reported nocturnal enuresis, 1 associated w ith perineal anesthesia. One had a neurogenic bladder with overflow voiding and bilateral third-degree vesicoureteral reflux. Second-degree reflux was found in the last patient. We conclude that follow-up after surgery for SC T should not only search for tumor recurrence but include the diagnosis and treatment of possible secondary urinary and/or fecal incontinence.