Neurofibroma of the Bladder: Case Report and Review of the Literature

Buskirk, Kryder E. Van; Clark, Paul K.; Snoga, J. R.; Attwood, Charles

doi:10.1016/s0022-5347(17)64099-0

Cited by 22 publications

(3 citation statements)

References 12 publications

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“…Thirty-five out of thirty-eight cases with pathologically diagnosed plexiform or mixed type neurofibromas of the bladder were associated with neurofibromatosis. [2][3][4][5][6][7][9][10][11][12][13][14][19][20][21]27,28,30,32,34,39,41,44,45,47,48 In our series, of the 2 plexiform neurofibromas, 1 was documented to arise in the setting of neurofibromatosis. Another patient had plexiform neurofibromas involving multiple organs, which although suspicious for neurofibromatosis was not diagnostic of the disease.…”

Section: Discussionmentioning

confidence: 98%

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Wang¹,

Montgomery²,

Epstein³

2008

American Journal of Surgical Pathology

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Benign nerve sheath tumors (schwannoma and neurofibroma) involving the urinary bladders are rare with only case reports and limited series. We identified 6 neurofibromas and 2 schwannomas involving the urinary bladder. Of the 8 cases, 7 were sent to one of the authors in consultation and the last came for treatment to The Johns Hopkins Hospital. Patients (3 males, 5 females) ranged in age from 3 to 69 years old (average 36 y old). Presenting symptoms included recurrent infection in 6 patients, hematuria in 3 patients, and irritative symptoms in 3 patients (some patients with 2 symptoms). Three patients had a pelvic mass on physical examination. Three patients (3/8) had a solitary lesion in the bladder, 4 patients (4/8) had multifocal lesions, and 1 patient had no information available. Five patients underwent local resection. One patient, who was 3 years old, had multiple plexiform neurofibromas within the bladder and did not have surgical treatment and another patient is also undergoing surveillance. One patient had no treatment information available. Seven patients had clinical follow-up information available. The length of follow-up varied from 2 to 124 months (average 47 mo). Three out of the seven patients with follow-up information had neurofibromas in other sites, including the skin, uterus, mesentery, and ureter. One patient was known to have other stigmata of neurofibromatosis. Both cases with schwannoma had only bladder involvement. No family history of neurofibromatosis and no genetic studies were performed on any of the patients. Three neurofibromas were of the diffuse type, with 2 cases plexiform, and 1 case could not be subtyped. Neither recurrence nor malignant transformation was demonstrated on follow-up. The importance of accurately diagnosing plexiform neurofibromas of the bladder is that a subset harbors neurofibromatosis. If the lesion is focal, conservative excision is the choice of treatment with a low risk of recurrence. Diffuse neurofibromas can be difficult to diagnose leading to delay of treatment and potentially the need for a more extensive excision. Once recognized as a neurofibroma, it is important to identify it as a diffuse neurofibroma, given its lack of relationship to neurofibromatosis.

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Section: Discussionmentioning

confidence: 98%

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Wang¹,

Montgomery²,

Epstein³

2008

American Journal of Surgical Pathology

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“…There have been approximately 50 reported cases of neurofibroma of the urinary bladder since 1932 (Table 3). 6–39 The tumors typically occur in young patients with neurofibromatosis type 1. Clinical presentations are similar to those related to posterior urethral valves in male patients.…”

Section: Discussionmentioning

confidence: 99%

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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“…A 25-month-old Caucasian girl was seen for congeni- Craven and Bresnahan, 1983Diekman et al, 1967Franceschini et al, 1978Greer and Pederson, Griebel et al, 1991Haddad and Jones, 1960Haraoka et al, 1988Hoffman, 1962Kaneti et al, 1988Kearse and Ritchey, Kenny et al, 1966Knudson and Amromin, Labardini et al, 1968 McKeown and Frazer, Messina and Strauss, 198119931961Nogita et al, 1990Nonomura et al, 1992Petsche and Radlinger, 1954Ravikumar and Lakshmanan, 1983Rink and Mitchell, 1983 Schepel and Tolhurst, Schreiber, 1963Thomas et al, 1989Van Buskirk et al, 1964Vitse et al, 1968 tal clitoromegaly that had slowly increased in size. Pregnancy and delivery were unremarkable.…”

Section: Patientmentioning

confidence: 99%

Clitoromegaly in neurofibromatosis

1995

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Genitourinary neurofibromas are rare and clitoral involvement in neurofibromatosis (NF) has been reported infrequently. However, when it occurs, clitoromegaly is often the presenting sign. In many cases, it is congenital. In 236 families with type 1 neurofibromatosis (NF-1) evaluated through the USF Regional Genetics Program between January 1982 and September 1993, four patients had clitoral involvement. In three, involvement was limited to the clitoris. Biopsy/surgical excision in two of them showed a neurofibroma in one and non-specific hamartomatous soft tissue overgrowth in the other. In the fourth patient, the involvement was asymmetric and extended to the labia majora and mons pubis. Endocrine studies and chromosomes in all patients were normal; there was no exposure to androgens, progestins, or coumadin. There was no gestational history of maternal luteomas. Review of the literature documented 26 patients with NF and clitoral involvement. Clitoral involvement in NF-1 appears to be more common than previously reported and the differential diagnosis of ambiguous genitalia should include clitoromegaly due to NF. Pathogenesis of clitoral lesions appears similar to other lesions of NF. Biopsy of such lesions appears to be justified only when malignancy is suspected.

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Neurofibroma of the Bladder: Case Report and Review of the Literature

Cited by 22 publications

References 12 publications

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Neurofibroma of the urinary bladder

Clitoromegaly in neurofibromatosis

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