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Childhood Gastrointestinal Stromal Tumor

MedGen UID:
906937
Concept ID:
C4086159
Neoplastic Process
Synonym: Childhood GIST

Definition

A gastrointestinal stromal tumor (GIST) occurring in childhood. Pediatric GISTs differ biologically from adult GISTs in that only 11% of pediatric GISTs have activating mutations of KIT and PDGFRA. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVChildhood Gastrointestinal Stromal Tumor

Professional guidelines

PubMed

Barber HR, Kwon TH
Cancer 1976 Jul;38(1 SUPPL):610-9. doi: 10.1002/1097-0142(197607)38:1<610::aid-cncr2820380186>3.0.co;2-t. PMID: 1277113

Recent clinical studies

Etiology

Chen Y, Su Y, Cao X, Siavelis I, Leo IR, Zeng J, Tsagkozis P, Hesla AC, Papakonstantinou A, Liu X, Huang WK, Zhao B, Haglund C, Ehnman M, Johansson H, Lin Y, Lehtiö J, Zhang Y, Larsson O, Li X, de Flon FH
Adv Sci (Weinh) 2024 Nov;11(41):e2404510. Epub 2024 Sep 10 doi: 10.1002/advs.202404510. PMID: 39257029
Zhang W, Chen H, Zhu L, Kong Z, Wang T, Li W
J Int Med Res 2022 May;50(5):3000605221100772. doi: 10.1177/03000605221100772. PMID: 35632985Free PMC Article
Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Hoekstra AS, Bayley JP
Biochim Biophys Acta 2013 May;1827(5):543-51. Epub 2012 Nov 20 doi: 10.1016/j.bbabio.2012.11.005. PMID: 23174333

Diagnosis

Schultz KAP, Williams GM, Kamihara J, Stewart DR, Harris AK, Bauer AJ, Turner J, Shah R, Schneider K, Schneider KW, Carr AG, Harney LA, Baldinger S, Frazier AL, Orbach D, Schneider DT, Malkin D, Dehner LP, Messinger YH, Hill DA
Clin Cancer Res 2018 May 15;24(10):2251-2261. Epub 2018 Jan 17 doi: 10.1158/1078-0432.CCR-17-3089. PMID: 29343557Free PMC Article
Rednam SP, Erez A, Druker H, Janeway KA, Kamihara J, Kohlmann WK, Nathanson KL, States LJ, Tomlinson GE, Villani A, Voss SD, Schiffman JD, Wasserman JD
Clin Cancer Res 2017 Jun 15;23(12):e68-e75. doi: 10.1158/1078-0432.CCR-17-0547. PMID: 28620007
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Kang HC, Menias CO, Gaballah AH, Shroff S, Taggart MW, Garg N, Elsayes KM
Radiographics 2013 Oct;33(6):1673-90. doi: 10.1148/rg.336135507. PMID: 24108557Free PMC Article
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582

Therapy

Zhang W, Chen H, Zhu L, Kong Z, Wang T, Li W
J Int Med Res 2022 May;50(5):3000605221100772. doi: 10.1177/03000605221100772. PMID: 35632985Free PMC Article
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582
Barber HR, Kwon TH
Cancer 1976 Jul;38(1 SUPPL):610-9. doi: 10.1002/1097-0142(197607)38:1<610::aid-cncr2820380186>3.0.co;2-t. PMID: 1277113

Prognosis

Chen Y, Su Y, Cao X, Siavelis I, Leo IR, Zeng J, Tsagkozis P, Hesla AC, Papakonstantinou A, Liu X, Huang WK, Zhao B, Haglund C, Ehnman M, Johansson H, Lin Y, Lehtiö J, Zhang Y, Larsson O, Li X, de Flon FH
Adv Sci (Weinh) 2024 Nov;11(41):e2404510. Epub 2024 Sep 10 doi: 10.1002/advs.202404510. PMID: 39257029
Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Gill AJ
Pathology 2012 Jun;44(4):285-92. doi: 10.1097/PAT.0b013e3283539932. PMID: 22544211
Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582
Chiarugi M, Galatioto C, Lippolis P, Zocco G, Seccia M
BMC Cancer 2007 May 9;7:79. doi: 10.1186/1471-2407-7-79. PMID: 17490483Free PMC Article

Clinical prediction guides

Rosenbaum T, Wimmer K
Klin Padiatr 2014 Nov;226(6-7):309-15. Epub 2014 Jul 25 doi: 10.1055/s-0034-1382021. PMID: 25062113
Gill AJ
Pathology 2012 Jun;44(4):285-92. doi: 10.1097/PAT.0b013e3283539932. PMID: 22544211
Chiarugi M, Galatioto C, Lippolis P, Zocco G, Seccia M
BMC Cancer 2007 May 9;7:79. doi: 10.1186/1471-2407-7-79. PMID: 17490483Free PMC Article
Chilton-Macneill S, Ho M, Hawkins C, Gassas A, Zielenska M, Baruchel S
Pediatr Dev Pathol 2004 Sep-Oct;7(5):493-8. Epub 2004 Jul 30 doi: 10.1007/s10024-004-1116-7. PMID: 15547773
Kodet R, Stejskal J, Malis J, Horák J
Pathol Res Pract 1994 Sep;190(8):750-8. doi: 10.1016/S0344-0338(11)80421-8. PMID: 7831150

Recent systematic reviews

Benesch M, Wardelmann E, Ferrari A, Brennan B, Verschuur A
Pediatr Blood Cancer 2009 Dec 15;53(7):1171-9. doi: 10.1002/pbc.22123. PMID: 19499582

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