Neurofibromatosis: Bladder Involvement with Malignant Degeneration

Hulse, Charles A.

doi:10.1016/s0022-5347(17)39572-1

Cited by 24 publications

(22 citation statements)

References 9 publications

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“…The malignant counterpart of these benign tumors, MPNST of the bladder, is an extremely rare entity. The present case represents just the 5th reported MPNST of the bladder in a patient with NF1 [2][3][4][5].…”

Section: Discussionmentioning

confidence: 62%

“…MPNSTs of the bladder seem not to respond to standard chemotherapy or radiation therapy [3][4][5]. In the future, advances in medical technology may lead to the development of an effective gene therapy.…”

Section: Discussionmentioning

confidence: 99%

“…The most commonly observed malignancy in this cancerprone disease is malignant preripheral nerve sheath tumor (MPNST) [1]. Although it may be recognized nearly in any tissue or organ, the development of such a tumor in the urinary bladder is an exceptionally rare phenomenon [2][3][4][5].…”

Section: Introductionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumor of the Urinary Bladder in von Recklinghausen Disease

Kalafatis

Kavantzas²,

Pavlopoulos³

et al. 2002

Urol Int

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Section: Discussionmentioning

confidence: 62%

Section: Discussionmentioning

confidence: 99%

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Malignant Peripheral Nerve Sheath Tumor of the Urinary Bladder in von Recklinghausen Disease

Kalafatis

Kavantzas²,

Pavlopoulos³

et al. 2002

Urol Int

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“…Most examples are benign, although approximately 5% of plexiform examples are known to undergo malignant transformation [14]. Only 2 published reports describe neurofibromas of the urinary bladder having undergone malignant transformation [8,11]. In the report by Hulse [8], a 52-year-old man with a history of multiple cutaneous neurofibromas presented with a flaccid, neurogenic bladder thought to be related to a neurofibroma in the lumbosacral region.…”

Section: Discussionmentioning

confidence: 99%

“…Other bladder tumors are exceedingly uncommon in the pediatric age group. These include transitional cell papillomas, papillary carcinoma, inflammatory myofibroblastic tumors, and very rarely neurofibromas [1][2][3][4][5][6][7][8][9][10][11]. Of these, some are associated with genetic risk factors [2,4].…”

Section: Introductionmentioning

confidence: 99%

Diffuse ganglioneuromatosis and plexiform neurofibroma of the urinary bladder: report of a pediatric example and literature review

et al. 2008

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Neurofibroma of the urinary bladder

Liu

Scheithauer

Leibovich

et al. 1999

Cancer

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BACKGROUND Neurofibroma of the urinary bladder is rare. Only isolated case reports have appeared. Information regarding the long term follow‐up of patients with neurofibroma is limited. METHODS The authors studied 4 cases of neurofibroma of the bladder diagnosed at Mayo Clinic from 1965 through 1990. Immunostains for S‐100 protein, neurofilament protein, epithelial membrane antigen (EMA), cytokeratin (CAM 5.2; AE 1/3), Type IV collagen, MIB‐1, and p53 protein were performed in all cases, as was Alcian blue at pH 2.5. The mean follow‐up was 9.6 years (range, 2–18 years). RESULTS The mean age at diagnosis was 17 years (range, 7–28 years); the male‐to‐female ratio was 1:1. All four patients exhibited physical stigmata of neurofibromatosis type 1. Clinical presentations included hematuria (one patient), irritative symptoms (two patients), and pelvic mass (one patient). Long term urinary complications included bladder atony (two patients), neurogenic bladder (one patient), and recurrent urinary tract infection with hematuria (one patient). Subsequently, two patients underwent partial cystectomy and one a complete cystectomy. Involvement of the bladder was generalized in all four cases. Three tumors were transmural, showing a diffuse and plexiform pattern of growth; in the fourth case, a superficial biopsy showed only diffuse submucosal growth with conspicuous pseudo‐Meissnerian corpuscle formation. An Alcian blue positive, variably collagenized matrix was present in all cases. Tumor cells displayed immunoreactivity for S‐100 protein and Type IV collagen in all cases. Neurofilament protein positive axons were evident in three cases; all other immunostains were negative. The mean MIB‐1 labeling index was 3.2% (range, 0.9–7.3%). No malignant transformation was observed during a mean follow‐up of 9.6 years. CONCLUSIONS Neurofibroma of the bladder presents early in life, is of the plexiform type with a diffuse component, and usually occurs in the setting of generalized neurofibromatosis type 1 rather than as isolated visceral neurofibromatosis. Malignant transformation did not occur in any of these 4 patients during a mean follow‐up time of 9.6 years. Cancer 1999;86:505–13. © 1999 American Cancer Society.

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Neurofibromatosis: Bladder Involvement with Malignant Degeneration

Abstract: A solitary neurofibroma of the urinary tract is rare. More rare in the urinary tract are neurofibromas associated with the neurofibromatosis of Von Recklinghausen's disease. A case of multiple neurofibromas of the bladder degenerating into neurofibrosarcoma is described.

Cited by 24 publications

References 9 publications

Malignant Peripheral Nerve Sheath Tumor of the Urinary Bladder in von Recklinghausen Disease

Malignant Peripheral Nerve Sheath Tumor of the Urinary Bladder in von Recklinghausen Disease

Diffuse ganglioneuromatosis and plexiform neurofibroma of the urinary bladder: report of a pediatric example and literature review

Neurofibroma of the urinary bladder

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