Pigmented median raphe cyst of the scrotum. A rare pediatric case and review of the literature.

Aim: Median raphe cyst are uncommon malformations of male genitalia, in which are rarely described melanin pigments or melanocytes; less than ten cases have been reported in literature. The aim of our study is to describe a rare ormations, case of pigmented median raphe cyst of the scrotum, successfully treated in our hospital.

Case Experience: A 6-years-old boy underwent surgical removal of a melanocytic lesion of the ventral surface of the scrotum in Day Surgery regimen. He reported no surgical complication or recurrence.

Results: Histology showed multiple cystic nodules, lined by squamous and pluri-stratified columnar epithelium, some of which contained melanic deposits and were anti-MART-positive.

Discussion: Even though the first case has been reported in 1985, the etiology of median raphe cysts remains unclear. Infrequently associated with trauma or infections, these lesions seem to origin from an abnormal development of the periurethral glands or atypical closure of the median raphe. Rarely melanin pigments or melanocytes are described in the histological examination, and the cause of the pigmentation is still unknown.

Conclusion: Median raphe cysts present a non-negligible variety of clinical presentations and histological features. Pigmented ones represent the rarest form: further studies may be necessary to clarify their pathogenesis and describe their clinical evolution.

Key Words: Median raphs, Male genitalia, Malformations.