Teratoma is a rare tumor of mixed dermal structures derived from three germ layers. The majority of congenital teratoma is identifiable in the human body and usually occurs within the ovaries of young women and testes of young men. Extragonadal teratoma arises at the midline structures such as anterior mediastinum, retroperitoneal, sacrum, ischial region, and pineal gland. Primary retroperitoneal teratoma accounts for 1–11% of all teratomas. In author’s database, the incidence of retroperitoneal teratoma is 7.7% in adults with retroperitoneal tumors. The age of onset exhibits double peak that appears at 6 months after birth and in early adulthood, respectively. It is observed in less than 10–20% of patients over 30 years. The incidence is higher in females than in males. Primary retroperitoneal teratoma is the third most common tumor secondary to neuroblastoma and Wilms tumor in pediatric retroperitoneal tumors, accounting for 2–5% of pediatric tumors. A total of 32 cases (15 women and 17 men; age range: 20–82 years) of adult retroperitoneal teratoma have been reported between 1937 and 1987.
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