title: Wilms Tumor in a Child With Bilateral Polycystic Kidneys and PHACE Syndrome: Successful Treatment Outcome Using Partial Nephrectomy and Chemotherapy.
abstract: Congenital anomalies may be associated with Wilms tumor either as isolated anomalies or as part of a congenital malformation syndrome. Nephroblastoma occurring in association with polycystic kidneys is very rare. The optimal surgical management of nephroblastoma in the setting of polycystic kidneys is not defined because of the rarity of this presentation. PHACE syndrome includes posterior fossa anomalies, hemangioma, arterial lesions, cardiac abnormalities/coarctation of aorta, and eye abnormalities. We report a 17-month-old baby with bilateral polycystic kidneys and PHACE syndrome who developed nephroblastoma in the right polycystic kidney which was treated successfully with nephron-sparing partial nephrectomy and chemotherapy.
authors.affiliation: *Paediatric Oncology, Regional Cancer Centre †Paediatric Surgery, Ananthapuri Hospitals and Research Institute ‡Department of Pathology, Regional Cancer Centre §Imageology, Regional Cancer Centre, Trivandrum, Kerala, India.
authors.forename: Priyakumari
authors.initials: P
authors.lastname: Thankamony
authors.forename: Venugopal
authors.initials: V
authors.lastname: Sivarajan
authors.forename: Rari P
authors.initials: RP
authors.lastname: Mony
authors.forename: Venugopal
authors.initials: V
authors.lastname: Muraleedharan
mesh_terms: D000015:Abnormalities, Multiple / Q000473:pathology; D001017:Aortic Coarctation / Q000150:complications*; D005124:Eye Abnormalities / Q000150:complications*; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000150:complications* / Q000473:pathology / Q000601:surgery*; D009392:Nephrectomy; D020752:Neurocutaneous Syndromes / Q000150:complications*; D007690:Polycystic Kidney Diseases / Q000150:complications*; D013577:Syndrome; D009396:Wilms Tumor / Q000150:complications* / Q000473:pathology / Q000601:surgery*