Paediatric renal tumors: An insight into molecular characteristics, histomorphology and syndromic association

Table 3 Genetic syndromes associated with paediatric renal tumors

Genetic syndromes	Renal tumors	Extra renal manifestations	Ref.
DICER 1 syndrome	Wilms tumor, cystic nephroma, anaplastic sarcoma of kidney	Pinealoblastoma, pleuropulmonary blastoma	Caroleo et al[83]
WAGR syndrome	Wilms tumor	Aniridia, genitourinary anomaly, developmental delay	Hol et al[10]
Denys–Drash syndrome	Wilms tumor, rapid progressive nephropathy	Male pseudo-hermaphroditism	Kucinskas et al[9]
Beckwith–Wiedemann syndrome	Wilms tumor	Hepatoblastoma, neuroblastoma, hemihypertrophy, macroglossia, macrosomia, organomegaly, omphalocele	MacFarland et al[11]
Simpson-Golabi-Behmel Syndrome Type I	Wilms tumor	Hepatoblastoma, adrenal neuroblastoma, macrocephaly, cardiovascular and skeletal abnormalities, visceromegaly	Tenorio et al[57]
Rhabdoid tumor predisposition syndrome	Malignant rhabdoid tumor of the kidney	Malignant rhabdoid tumors at various sites like central nervous system, liver, bladder, mediastinum	Nemes et al[66]