Familial cystic nephroma in two siblings with pleuropulmonary blastoma.
Cystic nephroma (CN) and pleuropulmonary blastoma (PPB) are rare tumors. In the cases presented here, a 13-month-old boy underwent right radical nephrectomy for CN. From the family history we learned that four years ago the patient's older sister underwent left radical nephrectomy for CN at a different center when she was 4 years old. A lung tumor was detected in the sister one year after nephrectomy. Biopsy from the lung tumor revealed PPB, and the sister died within one year after biopsy. To the knowledge of the authors, these cases represent the second reported familial occurrence of CN and the fourth of CN and PPB.
Bal
Nebil
N
Department of Pathology, Adana Teaching and Medical Research Center, Adana 01250, Turkey. nebilbal@baskent-adn.edu.tr
Kayaselçuk
Fazilet
F
Polat
Ayşe
A
Bolat
Filiz
F
Yilmaz
Zerrin
Z
Tuncer
Ilhan
I
D002675:Child, Preschool; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007223:Infant; D007680:Kidney Neoplasms / Q000235:genetics / Q000473:pathology*; D008175:Lung Neoplasms / Q000235:genetics / Q000473:pathology*; D008297:Male; D010997:Pleural Neoplasms / Q000235:genetics / Q000473:pathology*; D018202:Pulmonary Blastoma / Q000235:genetics / Q000473:pathology*; D035781:Siblings; D009396:Wilms Tumor / Q000235:genetics / Q000473:pathology*