Familial or individual associations of

 malignant rhabdoid tumor
 cerebral atypical teratoid/rhabdoid tumor
 spinal canal or cerebral primitive neuroectodermal tumor (cPNET)

Etiology

2 loci

 germline SMARCB1 mutations
 other locus unknown

References

 Fruhwald MC, Hasselblatt M, Wirth S, Kohler G, Schneppenheim R, Subero JI, Siebert R, Kordes U, Jurgens H, Vormoor J. Non-linkage of familial rhabdoid tumors to SMARCB1 implies a second locus for the rhabdoid tumor predisposition syndrome. Pediatr Blood Cancer. 2005 Oct 3; PMID: #16206192#

 Lee HY, Yoon CS, Sevenet N, Rajalingam V, Delattre O, Walford NQ. Rhabdoid tumor of the kidney is a component of the rhabdoid predisposition syndrome. Pediatr Dev Pathol. 2002 Jul-Aug;5(4):395-9. PMID: #12016529#

 Taylor MD, Gokgoz N, Andrulis IL, Mainprize TG, Drake JM, Rutka JT. Familial posterior fossa brain tumors of infancy secondary to germline mutation of the hSNF5 gene. Am J Hum Genet. 2000 Apr;66(4):1403-6. PMID: #10739763#

 Sevenet N, Sheridan E, Amram D, Schneider P, Handgretinger R, Delattre O. Constitutional mutations of the hSNF5/INI1 gene predispose to a variety of cancers. Am J Hum Genet. 1999 Nov;65(5):1342-8. PMID: #10521299#