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Neurofibroma of the urinary bladder
L Cheng1, B W Scheithauer, B C Leibovich
1Department of Pathology and Urology, Indiana University School of Medicine, Indianapolis, USA. lcheng@iupu.edu
Cancer
|August 3, 1999
Summary
Bladder neurofibromas are rare, often occurring in patients with neurofibromatosis type 1. These tumors typically present early in life and do not show malignant transformation during long-term follow-up.
Area of Science:
- Uropathology
- Oncology
- Genetics
Background:
- Neurofibroma of the urinary bladder is an exceptionally rare condition, with limited data on long-term patient outcomes.
- Existing literature primarily consists of isolated case reports, highlighting a significant knowledge gap.
Purpose of the Study:
- To investigate the clinical presentation, pathological characteristics, and long-term outcomes of urinary bladder neurofibromas.
- To assess the potential for malignant transformation in bladder neurofibromas over an extended follow-up period.
Main Methods:
- A retrospective analysis of four cases of bladder neurofibroma diagnosed between 1965 and 1990.
- Comprehensive histopathological examination including immunohistochemistry for S-100 protein, neurofilament protein, EMA, cytokeratins, Type IV collagen, MIB-1, and p53, along with Alcian blue staining.
- Mean follow-up duration of 9.6 years (range, 2-18 years) to evaluate long-term complications and outcomes.
Main Results:
- All four patients presented with physical stigmata of neurofibromatosis type 1 and were diagnosed at a mean age of 17 years.
- Clinical manifestations included hematuria, irritative voiding symptoms, and pelvic mass, with long-term complications such as bladder atony and recurrent urinary tract infections.
- Histopathology revealed a diffuse and plexiform growth pattern, with tumor cells immunoreactive for S-100 protein and Type IV collagen; no malignant transformation was observed.
Conclusions:
- Urinary bladder neurofibromas are typically diagnosed early in life, associated with generalized neurofibromatosis type 1, and exhibit a plexiform growth pattern.
- The study found no evidence of malignant transformation in these cases over a mean follow-up of 9.6 years, suggesting a benign long-term prognosis.