title: Clear cell sarcoma of the kidney in a child with Fanconi anemia.
abstract: Patients with Fanconi anemia subgroup D1, attributable to biallelic mutations in BRCA2, have an increased risk of solid tumors. Tumors in the kidneys of these patients are almost exclusively Wilms tumor. We report the first recorded case, to our knowledge, of a Clear Cell Sarcoma of the Kidney in a patient with this cancer predisposition syndrome. We review different aspects of the need for careful clinical observation in patients of this complementation group, given their risk for malignancy.
authors.affiliation: 1  Division of Pediatric Pathology, Children's Hospital of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, Pennsylvania, USA.
authors.forename: Humberto E
authors.initials: HE
authors.lastname: Trejo Bittar
authors.forename: Josiah E
authors.initials: JE
authors.lastname: Radder
authors.forename: Sarangarajan
authors.initials: S
authors.lastname: Ranganathan
authors.forename: Abhay
authors.initials: A
authors.lastname: Srinivasan
authors.forename: Suneeta
authors.initials: S
authors.lastname: Madan-Khetarpal
authors.forename: Miguel
authors.initials: M
authors.lastname: Reyes-Múgica
mesh_terms: D000328:Adult; D001706:Biopsy; D017024:Chemotherapy, Adjuvant; D005199:Fanconi Anemia / Q000150:complications*; D017809:Fatal Outcome; D006801:Humans; D007680:Kidney Neoplasms / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D008279:Magnetic Resonance Imaging; D008297:Male; D009364:Neoplasm Recurrence, Local; D009392:Nephrectomy; D012307:Risk Factors; D018227:Sarcoma, Clear Cell / Q000209:etiology* / Q000473:pathology / Q000628:therapy; D017211:Treatment Failure
keywords: CCSK; Clear Cell Sarcoma of Kidney; Fanconi anemia; cancer predisposition; pediatric renal cancer