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Embryonal adenocarcinomas (a type of malignant teratoma) of the sacrococcygeal region. Clinical and pathologic aspects of 21 cases

Sacrococcygeal teratomas in 21 consecutive patients admitted to Memorial Hospital were all embryonal adenocarcinomas having a common histologic appearance. Seventeen of the patients were female, and all except one were Caucasians. The average age at diagnosis was 2 years, and the average survival after diagnosis was 1 year, with a range of 1 to 68 months. All had large presacral tumors, but 2 had no external deformity. Seven had a midline external protuberance, and 12 presented with enlargement of a buttock. Prior to diagnosis, in 12 patients, the primary tumor produced one or more of a symptom triad of constipation, urinary frequency, and lower extremity weakness, and, in 12 patients, the tumor had metastasized. The primary tumor was resected in 6 patients but recurred in 4 of the 5 patients surviving the operation. Eighteen patients received radiation therapy to the primary tumor and/or metastases, and tumor regression occurred in all. Regression was also obtained in several patients who received chemotherapy. Because of the large size of the tumors and the metastases present at the time of diagnosis at a relatively young age, it is likely that the tumors were present in the presacral space at birth. The failure of surgery to cure the tumors in this series and tumor sensitivity to radiation and chemotherapy suggest that their treatment should consist of a combination of therapeutic modalities.