BJU International 2000 86 (3), 402

M.C. Mchoney¹, P.G. Ransley¹ and L. Spitz¹

¹ Department of Urology, Great Ormond Street Hospital For Children, London, UK

Comment 

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Authors 

A full-term male infant was born by elective Caesarean section; antenatal ultrasonography was reportedly normal. Bladder exstrophy was noted at birth and the boy also had bilateral vascularized cornea. Chromosomal studies showed a normal 46XY complement. Ultrasonography showed two normal kidneys and echocardiography showed a patent ductus arteriosus with turbulent flow distal to the ductus, but inconclusive evidence of any coarctation. The bladder exstrophy was closed 4 days after birth, with no osteotomies; echocardiography was normal after surgery, the patent ductus arteriosus having closed spontaneously. Cystoscopy at 6 months of age before the second-stage reconstruction revealed a small-capacity bladder. At 9 months the boy underwent bladder neck reconstruction, epispadias repair and bilateral pelvic osteotomy, after which his blood pressure was elevated and repeat echocardiography revealed coarctation of the aorta. His subsequent recovery was uneventful and he was discharged. Ultrasonography 3 weeks after reconstruction revealed mild bilateral hydronephrosis with an empty bladder. One week later, after admission with a UTI, ultrasonography showed a 4 × 3 × 2 cm fluid collection in the right iliac fossa, superolateral to the bladder (Fig. 1). On the presumptive diagnosis of an abscess or a urinoma, aspiration produced only a small quantity of liquefied haematoma, which was sterile on culture. Thereafter, the patient was apyrexial. Three weeks later he was re-admitted with a 1-week history of rapidly increasing abdominal distension, lethargy and occasional vomiting. On examination he had a grossly distended abdomen that was firm and not tender. His haemoglobin level was 98 g/L. Ultrasonography (Fig. 2) and CT (Fig. 3) were consistent with a large retroperitoneal haematoma. Over the next 48 h the abdominal distension increased and surgical intervention became necessary. At laparotomy there was a huge mass occupying the entire abdominal cavity. It was pale greyish with solid and cystic areas. After dissection it was found to be arising from the dome of the bladder. A near-total resection was performed, although there was major tumour spillage during the dissection. Frozen-section analysis revealed a spindle-cell tumour, and subsequent histology confirmed a pleomorphic embryonal rhabdomyosarcoma. No metastases were found and chemotherapy was commenced.

Case report 

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Authors 

Genitourinary rhabdomyosarcoma is an unusual childhood tumour, with an incidence of < 1 per million [1]. There appear to be two peaks of incidence at ages 2–6 years and 15–19 years [1,2]. Although there have been many reports of adenocarcinoma occurring in the exstrophied bladder [3], the occurrence of rhabdomyosarcoma after repairing bladder exstrophy is rare. Rhabdomyosarcoma is the fifth most common solid malignancy of childhood [1], with involvement of the genitourinary tract in 5–20% of cases [1,2]. A study of rhabdomyosarcoma in children aged < 1 year reported a three-fold higher incidence of genitourinary primaries than in older children [2]. Rhabdomyosarcomas in the bladder arise predominantly from the trigonal region [1]. In 1972, Semerdjian et al.[4] reported a 4-year-old boy with rhabdomyosarcoma occurring in a repaired exstrophied bladder; the tumour arose from the trigone. Histology revealed a pleomorphic embryonal rhabdomyosarcoma. This is the only previously reported case of rhabdomyosarcoma in a repaired exstrophy. The present patient was diagnosed at 11 months of age. He had no evidence of any lesion on ultrasonography 1 month before presentation. It can therefore be assumed that most of the tumour growth had occurred within this period. Rhabdomyosarcomas can be aggressive, and up to 20% have metastases at the time of diagnosis [1,2]. Despite the local aggressiveness present in this patient, no distant metastases have been detected. Pleomorphic rhabdomyosarcoma accounts for < 10% of rhabdomyosarcomas in children, and is more commonly found in the extremities and trunk. The association of coarctation of the aorta and bladder exstrophy has not been reported previously. The association of rare symptoms in this patient, including a family history of cranio-cleidodystosis, suggests a genetic abnormality. Close follow-up of the family is mandatory, given their history and the reported association between rhabdomyosarcoma and other familial tumours.

Case report 

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Authors 

Case report 

Comment 

M.C. McHoney, FRGS, SHO in Paediatric Urology.

P.G. Ransley, FRCS, Consultant in Paediatric Urology.

L. Spitz, FRCS, PhD, Nuffield Professor of Paediatric Surgery.