Canaliform median raphe cysts (MRCs) lined by squamous epithelium in a 5 year old male patient; report of a rare case and comprehensive review of the literature.

UNLABELLED: Median raphe cysts (MRCs) are rare, benign congenital lesions of unknown origin, that can be found anywhere on the ventral side of the genital area, between the urethral meatus and the anus. The rarity of our case is attributed to the canaliform type, the scrotal and perineal localization and the epidermoid epithelium. A 5 year old boy, with free perinatal and family history, was admitted to our department as an outpatient due to the presence of an elongated and mildly painful lesion in the middle of the scrotum, gradually increasing in size. During physical examination the presence of a painful, subcutaneous, yellowish lesion, extending from the scrotal to the perineal raphe, was documented. Patient underwent elective surgery, under general endotracheal anesthesia, and complete resection of the lesion was conducted. Histopathological examination revealed the presence of a canaliform lesion consisting of five cysts, lined by squamous epithelium and filled with lamellate keratin.

IN CONCLUSION: a) preventive removal of MRCs is considered as the safest treatment option, in order to be avoided future, potential complications regarding urination and sexual intercourse, b) if therapeutic intervention is delayed, especially a er development of inflammation of MRCs, then the likelihood both of iatrogenic injury to underlying structures, mainly to the penile or perineal urethra, and of relapse a er resection increases significantly and c) if orchidopexy precedes the development of MRCs, the possibility of presence of ovarian serous border line tumor with Müllerian duct remnants should always be excluded.