Urothelial-lined perineal masses in hypospadias: clinical and pathological insights.

INTRODUCTION

To present a case series of the proximal hypospadias associated with perineal mass, which the perineal lesions were lined by urothelium containing Von Brunn's nests. A literature review was conducted to summarize the clinical and pathological features of similar cases.

METHODS

Two cases of proximal hypospadias with perineal mass treated at our institute were analyzed. Clinical presentation, diagnostic evaluation, surgical management, and histopathological findings are described. A literature review was performed in PubMed with the terms "('perineal mass' OR 'perineal anomaly' OR 'perineal') AND ('hypospadia' OR 'anorectal malformations' OR 'duplication')".

RESULTS

Both patients presented were detected by physical examination. One case had a normal anus, while the other had anorectal malformations (ARMs). Both cases showed normal Karyotype (46XY). Magnetic resonance imaging (MRI) demonstrated a soft tissue lesion that has not connection with or communication with adjacent organs. Both of then underwent mass excision and urethroplasty after cystoscopy, which revealed a normal bladder and posterior urethra. Pathological result of perineal mass suggested the histological structures of urothelium with von Brunn's Nests. There were totally 8 cases of hypospadia with perineal mass reported in the literature. Reported histological findings included 3 (37.5%) colonic structure, 2 (25%) lipoma, 2 (25%) accessory scrotum with hamartom and 1 (12.5%) sacrococcygeal teratomain.

DISCUSSION: Perineal mass with proximal hypospadias is an exceptionally rare anomaly. The histopathological founding of perineal mass lined by urothelium with von Brunn's nests has not been previously reported.