Anal atresia and embryonic malignant teratoma

We report a unique coincidence of malignant tumor projected via anus in an infant girl operated on for congenital anal atresia without fistula. The case seems to be different from Currarino syndrome. After five month of uneventful follow-up after posterior sagittal anorectoplasty (PSARP), a rapidly enlarging polyp-like bulge protruded from the anus. Blood tumor markers were negative. X-ray computed tomography (CT scan) revealed a tumor located subcutaneously between the coccygeal bone and rectum. The intraoperative biopsy showed malignancy. By perineal approach, the tumor was radically resected en-bloc with anus, part of neorectum, and distal fragment of coccygeal bone. Mature descending colostomy was performed. The postoperative course was uneventful. The final histological diagnosis revealed embryonic malignant teratoma. The bone scintigraphy was negative. Additional chemotherapy was applied without complication. After one year follow-up free of tumor recurrence or metastasis, the perineal anus reconstruction was performed by combined abdominal and posterior sagittal approach. Anal dilatation program was started on 10th postoperative day when the stoma was closed. Five months after the last surgery, the girl presents bowel control and defecates regularly 2–3 times a day without complaints, constipation or cicatricial anal stenosis. Oncologic surveillance is negative.