BOUTY, A.; DOBREMEZ, E.; HARPER, L.; HARAMBAT, Jerome; BOUTEILLER, C.; ZAGHET, B.; WOLKENSTEIN, P.; DUCASSOU, S.; LEFEVRE, Y.

doi:10.1159/000487193

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Urologia internationalis. 2018, vol. 100, n° 3, p. 339-345

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AIM: Neurofibromatosis type 1 (NF1) is a rare autosomal dominant disorder. Malignant transformation into malignant peripheral nerve sheath tumors (MPNST) can occur. However, urinary tract involvement is rare. We report 4 cases of NF1 with bladder dysfunction. METHODS: A retrospective single center analysis of 4 patients was conducted over a 17-year period, focusing on urinary tract involvement. RESULTS: NF1 was diagnosed at a median of 16.5 months (4-36) and urinary involvement occurred at a median of 5.25 years (4-9) after diagnosis. Bladder dysfunction was due to spinal cord compression in 2 cases, bladder invasion in 1 case, and cerebral lesions in 1 case. Malignant transformation of neurofibromas into MPNST occurred in 2 patients. Mechanisms of urinary involvement in NF1 are diverse and no pre-established protocol of management and follow-up exists. CONCLUSION: Although rare, dysfunction of the bladder can arise in NF1 and innovative strategies then need to be considered. This is best achieved with the help of a multidisciplinary team and a national reference center when available.< Réduire

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Bladder Dysfunction in Children with Neurofibromatosis Type I: Report of Four Cases and Review of the Literature

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