Inflammatory myofibroblastic tumour of the bladder in children: a review

Michael Collin; Adrian Charles; Andrew Barker; Japinder Khosa; Naeem Samnakay

doi:10.1016/j.jpurol.2015.03.009

Inflammatory myofibroblastic tumour of the bladder in children: a review

J Pediatr Urol. 2015 Oct;11(5):239-45. doi: 10.1016/j.jpurol.2015.03.009. Epub 2015 Apr 20.

Authors

Michael Collin¹, Adrian Charles², Andrew Barker³, Japinder Khosa⁴, Naeem Samnakay⁵

Affiliations

¹ Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: mcollin@me.com.
² Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Adrian.Charles@health.wa.gov.au.
³ Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Andrew.Barker@health.wa.gov.au.
⁴ Princess Margaret Hospital for Children, Roberts Road, Subiaco, Western Australia, 6008, Australia. Electronic address: Japinder.Khosa@health.wa.gov.au.
⁵ 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.

PMID: 25982020
DOI: 10.1016/j.jpurol.2015.03.009

Abstract

Introduction: 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.

Discussion: 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.

Keywords: Anaplastic lymphoma kinase (ALK-1); Haematuria; Inflammatory myofibroblastic tumour bladder.

Publication types

Review

MeSH terms

Biomarkers, Tumor / metabolism*
Child
Diagnostic Imaging / methods*
Global Health
Humans
Incidence
Myofibroblasts / pathology*
Neoplasm Staging / methods*
Neoplasms, Muscle Tissue* / diagnosis
Neoplasms, Muscle Tissue* / epidemiology
Neoplasms, Muscle Tissue* / metabolism
Urinary Bladder Neoplasms* / diagnosis
Urinary Bladder Neoplasms* / epidemiology
Urinary Bladder Neoplasms* / metabolism

Substances

Biomarkers, Tumor