Table 2.

Hereditary Component of Various Solid Neoplasms Presenting in Childhood

Tumor Type	Gene	Hereditary (%)
Medullary Thyroid Carcinoma	RET (10q11.2)	>90%
Pleuropulmonary Blastoma	DICER1 (14q32.13)	65 – 70%
Adrenocortical Neoplasia	TP53 (17p13.1)	50 – 80%
Optic Glioma	NF1 (17q11.2)	45%
Retinoblastoma	RB1 (13q14.2)	40%
Pheo-Paraganglioma	VHL, NF1, RET, SDHB, SDHD	40%
ATRT/MRT	SMARCB1 (22q11.23)	25%
Hepatoblastoma	APC (5q21-q22)	8 – 9%
Wilms Tumor	WT1 (11p13)	3 – 5%
Rhabdomyosarcoma	TP53, NF1, NBS1, ESC02, PTPH11, BUBIB, CREBBP, NF2, TSC1/2, WRN	2 – 4%
Neuroblastoma	ALK1 (2p23)	1 – 2%
CNS Tumors	PTCH1, RAS-NF1, NF2, WNT, RB1, TP53, VHL	<1 – 3%

Abbreviations: ATRT/MRT = Atypical teratoid rhabdoid tumor tumor / malignant rhabdoid tumor; CNS = Central nervous system