Table 1.

Clinicopathologic features and molecular data in 17 pediatric GISTs

No.Age/sexSiteMultifocalTypeMF/50 HPFKIT/PDGFRAMetsImatinib response/mo/dose(mg/d)Sunitinib response/mo/dose(mg/d)Follow-up (mo)
1*10FStomachYesE6WTPPOD/18/800SD/8/2569/AWD
2*§12FStomachYesS+E2WTLN,P,LNoNo78/AWD
3*8FStomachYesS+E15WTLNNoNo23/NED
416FStomachYesE3WTPNANA6/NED
516FStomachYesS+E4WTLNDI/3/600DI/1/37.560/AWD
6*§14FStomachYesS+E10WTPSD+POD/6/800POD/5/2536/AWD
7*12FStomachYesE3WTLN,P,LNoNo80/NED
8*15FStomachYesE48WTP,LPOD/4No138/DOD
910FStomachYesE6WTP, LSD/9/300No188/AWD
1018FOmentumNAE76WTP,LPOD/3/400PR/8/5048/AWD
11*13FStomachYesE20WTNoNANA3/NED
1214FStomachYesS+E40WTnoA/24No30/AWD
13*12MStomachNoS3WTPNoNo49/AWD
1418MStomachYesS6WTNoNANA24/NED
1515MStomachYesS+E1WTNoNANANA
1614MStomachNoE1D842VNoNANANA
1717MSBNoS1558K delNoNoNo22/NED
  • Abbreviations: S, spindle; E, epithelioid, NA, not available, SB, small bowel; SD, stable disease; POD, progression of disease; DI, drug intolerance; PR, partial response; A, adjuvant therapy; NED, no evidence of disease; AWD, alive with disease; DOD, dead of disease; P, peritoneum; L, liver; LN, perigastric lymph nodes; MF, mitotic figure; HPF, high-power field; Mets, metastasis.

  • * Gene expression analysis.

  • Cases 1, 2, and 7 to 9 were previously reported in ref. 2.

  • Full-length KIT and PDGFRA cDNA sequencing.

  • § Cases with Carney's triad.

  • Suspected of Carney's triad.