Fetus in fetu in the scrotal sac: Case report and literature review

Abstract

Fetus in fetu (FIF) is a rare congenital anomaly. The most common site at which FIF occurs is the retroperitoneum. The mechanisms underlying the development of FIF have not been fully elucidated. The monozygotic twin theory postulates that FIF results from the unequal division of the totipotent cells of the blastocyst. However, the monozygotic twin theory does not explain all cases of FIF. Herein, we describe the clinical characteristics of a 20-day-old infant with scrotal sac swelling. Ultrasonography and computed tomography revealed the presence of a mass consistent with a FIF rather than a teratoma. Surgical removal and a subsequent pathological evaluation demonstrated that the anencephalic fetus exhibited limb buds adjacent to a palpable vertebral column, supporting the diagnosis of FIF. The infant had an uneventful recovery and was discharged on the fifth postoperative day. In the present report, the pathogenesis, presentation, diagnosis, and management of FIF, as well as new concepts emerging in this area of research, are discussed. Although the majority of cases of FIF may be diagnosed preoperatively, FIF should be distinguished from teratoma because the latter has substantial malignant potential. The recommended treatment for FIF is complete resection. To confirm the diagnosis of FIF, pathological examination, karyotyping, serologic marker assessment, and DNA restriction site mapping should beperformed after removing the mass. Although FIF is thought to be a benign disorder, follow-up is necessary as a precaution against malignant recurrence, which has been described once.