title: Inflammatory myofibroblastic tumour of the bladder in children: a review.
abstract: BACKGROUND
  Inflammatory myofibroblastic tumours of the bladder (IMTB) are rare, and feature a benign and reactive proliferation of myofibroblasts. 25% of the reported IMTB cases in the literature occur in children. The present study presents a review of IMTB in children.
  
  
  CONCLUSIONS
  The data from 42 reported cases of paediatric IMTB in the world literature are summarised, including two recent cases from the present centre. Paediatric IMTB equally affects males and females. It mainly presents with haematuria, dysuria or abdominal pain. Lesions can vary in size, but mean size is 5.5 cm. Mean age is 7.5 years. The aetiology of IMTB is poorly understood, but includes infective or traumatic aetiologies, or a possible clonal lesion. IMTB may specifically show clonal gene rearrangements involving the anaplastic lymphoma kinase (ALK-1) gene. To differentiate IMTB from rhabdomyosarcoma, tissue diagnosis and careful histological analysis are essential. Tumour biopsy can be achieved by a transurethral approach or a transcutaneous approach with ultrasound guidance. Between 35 and 89% of cases of IMTB express ALK-1 by immunohistochemistry. ALK-1 expression is much less common in other bladder soft tissue tumours. ALK-1 is thus useful in the diagnosis of IMTB. The treatment of choice for IMTB is complete surgical resection of the lesion. In children, no proven recurrent or metastatic IMTB episodes are reported after excision. However IMTB recurrences are reported in adults, likely due to incomplete excision. Follow-up after excision is therefore recommended.
  
  
  CONCLUSIONS
  Paediatric IMTB is uncommon. Tissue biopsy is essential for diagnosis. Careful histological assessment is required to differentiate IMTB from malignant paediatric bladder tumours such as rhabdomyosarcoma. ALK-1 expression is useful in confirming the diagnosis of IMTB. Treatment of choice is complete surgical resection of the lesion. Recurrence is reported in adult IMTB. Follow-up is therefore recommended.
authors.affiliation: Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: mcollin@me.com.
authors.forename: Michael
authors.initials: M
authors.lastname: Collin
authors.affiliation: Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Adrian.Charles@health.wa.gov.au.
authors.forename: Adrian
authors.initials: A
authors.lastname: Charles
authors.affiliation: Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Andrew.Barker@health.wa.gov.au.
authors.forename: Andrew
authors.initials: A
authors.lastname: Barker
authors.affiliation: Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Japinder.Khosa@health.wa.gov.au.
authors.forename: Japinder
authors.initials: J
authors.lastname: Khosa
authors.affiliation: Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia; University of Western Australia, Hackett Drive, Nedlands, Western Australia 6009, Australia. Electronic address: Naeem_samnakay@hotmail.com.
authors.forename: Naeem
authors.initials: N
authors.lastname: Samnakay
mesh_terms: D014408:Biomarkers, Tumor / Q000378:metabolism*; D002648:Child; D003952:Diagnostic Imaging / Q000379:methods*; D014943:Global Health; D006801:Humans; D015994:Incidence; D058628:Myofibroblasts / Q000473:pathology*; D009367:Neoplasm Staging / Q000379:methods*; D009379:Neoplasms, Muscle Tissue / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism; D001749:Urinary Bladder Neoplasms / Q000175:diagnosis / Q000453:epidemiology / Q000378:metabolism
chemical_list: D014408:Biomarkers, Tumor
keywords: Anaplastic lymphoma kinase (ALK-1); Haematuria; Inflammatory myofibroblastic tumour bladder