An unusual case of female hypospadias associated with a pelvic mass Beatrice Vezzù a , Marco Castagnetti a, ⁎ , Gianni Bisogno b , Giovanni Cecchetto c , Waifro Rigamonti a a Section of Paediatric Urology, Urology Unit, Department of Oncological Sciences, University Hospital of Padova, 35100 Padua, Italy b Division of Haematology/Oncology, Department of Paediatrics, University Hospital of Padova, 35100 Padua, Italy c Division of Paediatric Surgery, Department of Paediatrics, University Hospital of Padova, 35100 Padua, Italy Received 30 January 2008; revised 18 March 2008; accepted 21 March 2008 Key words: Female hypospadias; Soft tissue sarcoma; Pelvic mass; Urogenital sinus; Children Abstract Female hypospadias is a rare malformation. We report on an unusual case that presented with a urethral dimple in the perineum in association with a pelvic soft tissue sarcoma compressing and displacing the urethra. The diagnosis was made by cystoscopy, and, as the patients remained asymptomatic, no treatment was eventually required for her urologic malformation. This is, to our knowledge, the first reported case of female hypospadias associated with a perineal urethral dimple. The possible pathogenesis of the malformation is discussed also in relation to the simultaneous presence of a pelvic mass. © 2008 Elsevier Inc. All rights reserved. Female hypospadias is a rare malformation in which the urethra opens on the anterior vaginal wall at any site between the introitus and the fornix. Owing to the lack of a urethral opening on the perineum, cases of female hypospadias usually present a single perineal opening, the vagina. This poses the issue of a differential diagnosis with the urogenital sinus [1]. We report on a case of female hypospadias which was unusual in 2 respects: first, the patient presented a urethral dimple on the perineum; second, the malformation was associated with a pelvic mass compressing and displacing the urethra. 1. Case report The child had an unremarkable antenatal history with normal antenatal scans, the last of which had been performed at 32 weeks of gestation. At birth, she presented with a severe edema of the labra majora and a huge mass was palpable in the left iliac fossa and in the left buttock. An ultrasound scan performed on day 1 of life confirmed the presence of an abdominal mass of 10 cm in diameter and showed a mild bilateral ureterohydronephrosis. Magnetic resonance imaging scan confirmed the presence of a retroperitoneal mass, in a prevertebral position, with the cranial margin adjacent to the lower pole of the right kidney, and the caudal margin extending to the pelvic floor and the muscles of the left buttock. The lesion was about 10 cm in size and displaced the bowel, the bladder, the urethra, and the rectum (Fig. 1A). Magnetic resonance imaging also confirmed bilateral hydronephrosis. ⁎ Corresponding author. Tel.: +39 049 8212737; fax: +39 049 8212721. E-mail address: marcocastagnetti@hotmail.com (M. Castagnetti). www.elsevier.com/locate/jpedsurg 0022-3468/$ – see front matter © 2008 Elsevier Inc. All rights reserved. doi:10.1016/j.jpedsurg.2008.03.058 Journal of Pediatric Surgery (2008) 43, E37–E40