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

Kalafatis, Panagiotis; Kavantzas, Nikolaos; Pavlopoulos, Petros M.; Agapitos, Emmanouil; Politou, Mary; Kranides, Aggelos

doi:10.1159/000065568

Cited by 16 publications

(19 citation statements)

References 9 publications

(15 reference statements)

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“…A small number of patients have experienced recurrences, and only limited number of tumors underwent malignant transformation. 13,18,26,29,36,37 Among our 6 cases of neurofibroma of the urinary bladder, 3 have undergone local excision without recurrence. One patient did not receive any treatment and developed neurofibromas in the distal ureter and mesentery complicated by hydronephrosis.…”

Section: Discussionmentioning

confidence: 99%

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: 99%

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Wang¹,

Montgomery²,

Epstein³

2008

American Journal of Surgical Pathology

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“…48 Of 2 patients with GN and malignant peripheral nerve sheath tumour, 1 had NF1, and recurrence of GN as malignant PNST has been suggested by the authors. 49…”

Section: Genetic Backgroundmentioning

confidence: 99%

Ganglioneuromas across age groups: Systematic review of individual patient data

Fliedner

Winkelmann

Wesley

et al. 2020

Clinical Endocrinology

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“…Eine Beteiligung des Harntraktes wird insgesamt selten beschrieben. Im Falle einer Harntraktbeteiligung scheint jedoch die Harnblase am häufigsten betroffen zu sein [1,2,3,4]. Das primär diffuse Neurofibrom der Harnblase ist unter den Fibromen der Harnblase eine Rarität.…”

Section: Diskussionunclassified

Primäres diffuses Neurofibrom der Harnblase

Rink

Minner

Mc³

et al. 2010

Urologe

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Diffuse neurofibromas are benign mesenchymal tumours with nerve sheath differentiation. Only a few cases have been reported in the urinary bladder. We present a well-documented case report of a 62-year-old man presenting with gross haematuria and histopathological confirmation of a diffuse neurofibroma. We further reviewed the current literature with regard to clinical features and treatment options.

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

Cited by 16 publications

References 9 publications

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Benign Nerve Sheath Tumors on Urinary Bladder Biopsy

Ganglioneuromas across age groups: Systematic review of individual patient data

Primäres diffuses Neurofibrom der Harnblase

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