Clear cell sarcoma of the kidney in a child with Fanconi anemia.
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.
['Trejo Bittar|Humberto E|HE|', 'Radder|Josiah E|JE|', 'Ranganathan|Sarangarajan|S|', 'Srinivasan|Abhay|A|', 'Madan-Khetarpal|Suneeta|S|', 'Reyes-Múgica|Miguel|M|']
D000328:Adult D001706:Biopsy D017024:Chemotherapy, Adjuvant D005199:Fanconi Anemia D017809:Fatal Outcome D006801:Humans D007680:Kidney Neoplasms D008279:Magnetic Resonance Imaging D008297:Male D009364:Neoplasm Recurrence, Local D009392:Nephrectomy D012307:Risk Factors D018227:Sarcoma, Clear Cell D017211:Treatment Failure
Fanconi anemia cancer predisposition CCSK Clear Cell Sarcoma of Kidney pediatric renal cancer