[Bladder pseudosarcoma in pediatrics: clinico-pathological features and treatment].

OBJECTIVES

To review the most common clinical signs and symptoms of inflammatory pseudotumor of the bladder in children and to distinguish this benign lesion from malignant neoplasms such as rhabdomyosarcoma and leiomyosarcoma.

METHODS

Two cases os pseudosarcomatous tumor of the bladder in children are described. In the first patient, the tumor had appeared spontaneously in a 9-year-old boy. The second had developed in a 6-year-old girl and was considered to be reactive to prior surgery. The literature is reviewed and data from 16 cases of inflammatory pseudotumor of the bladder in patients under 16 years of age are also presented.

RESULTS

The mean age at presentation was 8 years. The male-to-female ratio was 3:1. Hematuria (56%), dysuria (37%) and abdominal pain with a palpable mass (18%) were the most commonly described clinical manifestations. Tumor size ranged from 3 to 10 cm and they were frequently located in the lateral walls and base of the bladder. Histological analysis showed an inflammatory pseudosarcomatous reaction. Immunohistochemical analysis showed moderate staining for vimentin, mild staining for focal muscle actin and negative for keratin and S-100 protein. Patients were treated by local resection (7 pts), partial cystectomy (5 pts), radical cystectomy (2 pts) and pelvic exenteration (2 pts). The mean follow-up was 34 months. All patients that had been followed (14/16) were reported to be free of disease with no evidence of recurrences or metastases.

CONCLUSIONS

Inflammatory pseudotumor of the bladder is an unusual benign tumor that is very rare in children. Long-term follow-up confirms the benign nature of the lesion and conservative management is therefore advocated. However, given its histological similarity to malignant tumors, a close follow-up is recommended.