Abstract

Neurothekeoma is a rare, benign, asymptomatic tumor of the skin. Frequently it is found on the face or limbs. Sacrococcygeal region is a very unusual site for these tumors.

In an 8.5-month-old girl a cystic mass of 4x2.3 cm was detected in the left adnexal area on abdominopelvic ultrasonography. On physical examination a bilobulated, apparently cystic mass was palpated between rectum and sacrum by digital rectal examination. Computed tomography scan of the lower abdomen showed a 42x49x46 mm bilobulated cystic mass in the presacral area without any connection with spinal canal, and thecal sac. As an initial diagnosis ,cystic teratoma was suspected. Under general anesthesia, both bilobulated presacral cystic masses including the coccyx were totally excised through a posterosagittal incision. Histopathology of the specimen was reported as neurothekeoma. The patient was discussed in the medical council, and discharged with advice of regular follow-up.

Sacrococcygeal teratomas are the most common tumor in the newborn, including the stillbirths. Differential diagnosis of neonatal sacrococcygeal masses include meningoceles, lymphangiomas, lipomas, tail-like remnants, meconium pseudocysts, rectal duplications, and several other rare conditions.

Neurothekeoma is rare tumor derived from peripheral nerve sheath cells, which can occur in all age groups. It is frequently found on face and upper limb. Treatment of neurothekeoma is total excision. Recurrence can occur after excision. Only one case with sacrococcygeal neurothekeoma was reported and there has been no evidence of recurrence has been indicated during follow-up period after total excision.