Diffuse ganglioneuromatosis and plexiform neurofibroma of the urinary bladder: report of a pediatric example and literature review |
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Authors: | Scheithauer Bernd W Santi Mariarita Richter Eric R Belman Barry Rushing Elisabeth J |
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Institution: | a Department of Pathology and Laboratory Medicine, Mayo Clinic, Rochester, MN 55905, USA b Division of Pathology, Children's National Medical Center, Washington DC 20010, USA c Division of Urology, Children's National Medical Center, Washington DC 20010, USA d Urology Department, Walter Reed Army Medical Center, Washington DC 20307, USA e Department of Neuropathology and Ophthalmic Pathology, Armed Forces Institute of Pathology, Washington DC 20306, USA |
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Abstract: | As an initial manifestation of neurofibromatosis type 1, isolated neurofibroma of the urinary bladder occurs only rarely in the first 2 decades of life. We report a case of a 6-year-old African-American girl who presented in this manner and was found to have a plexiform neurofibroma as well as diffuse ganglioneuromatosis of the urinary bladder. We describe the clinical presentation, diagnostic procedures, and pathologic features encountered. In addition, we review the literature with respect to etiology, current treatment strategies, and the issue of surveillance for this complex lesion occurring in the pediatric population. Neurofibroma of the bladder should be considered in the differential diagnosis of painless hematuria in childhood. Its presentation in such patients warrants a complete evaluation to establish the diagnosis of neurofibromatosis type 1 and begin long-term surveillance for its associated manifestations. At present, there are no established criteria for the treatment of this rare lesion. |
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Keywords: | Urinary bladder Ganglioneuromatosis Plexiform neurofibroma Neurofibromatosis type 1 Immunohistochemistry |
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