Gastrointestinal stromal tumors of the small intestine in pediatric populations: a case report and literature review.
An 18-year-old girl presented with abdominal pain and a tumor was subsequently detected in the jejunum. We therefore carried out a wedge resection of the jejunum. The diagnosis of GIST was confirmed histologically, and a mutation in exon 9 of the c-kit gene was observed. GISTs are rare in pediatric populations and pediatric GISTs occur predominantly in females and are characterized by a multifocal gastric location and a wild-type phenotype for the c-kit genes. The features of pediatric GISTs of the small intestine have not yet been categorized, and to date, only 11 cases in patients younger than 18 years have been reported. These cases did not occur primarily in females and tended to present as single tumors with mutations in the c-kit gene. This suggests that these cases do not have the same features as pediatric gastric GISTs, but instead are similar to adult GISTs. In pediatric populations, GISTs of the small intestine were expected to show a better response to imatinib treatment than gastric GISTs because of the alterations in the c-kit gene.
Shimomura
Manabu
M
Department of Surgery, Division of Frontier Medical Science, Programs for Biomedical Research, Graduate School of Biomedical Sciences, Hiroshima University, 1-2-3 Kasumi, Minami-ku, Hiroshima 7348551, Japan. manabus@fuga.ocn.ne.jp
Ikeda
Satoshi
S
Takakura
Yuji
Y
Kawaguchi
Yasuo
Y
Tokunaga
Masakazu
M
Takeda
Haruka
H
Sumitani
Daisuke
D
Yoshimitsu
Masanori
M
Hinoi
Takao
T
Okajima
Masazumi
M
Ohdan
Hideki
H
D000293:Adolescent; D005260:Female; D046152:Gastrointestinal Stromal Tumors / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery; D006801:Humans; D007150:Immunohistochemistry; D007580:Jejunal Neoplasms / Q000175:diagnosis* / Q000235:genetics / Q000601:surgery
Science. 1998 Jan 23;279(5350):577-80
9438854
Am J Surg Pathol. 2005 Oct;29(10):1373-81
16160481
J Clin Oncol. 2003 Dec 1;21(23):4342-9
14645423
Am J Surg Pathol. 2006 Apr;30(4):477-89
16625094
Med Pediatr Oncol. 2000 Jan;34(1):70-1
10611593
J Pediatr Hematol Oncol. 2009 Feb;31(2):108-12
19194193
J Pediatr Gastroenterol Nutr. 2007 Sep;45(3):358-60
17873750
Pediatr Blood Cancer. 2009 Dec 15;53(7):1171-9
19499582
Clin Cancer Res. 2008 May 15;14(10):3204-15
18483389
Cancer Res. 2007 Oct 1;67(19):9084-8
17909012
J Pediatr Hematol Oncol. 2005 Apr;27(4):179-87
15838387
Arch Pathol Lab Med. 1999 Sep;123(9):842-5
10458837
Pediatr Surg Int. 2006 May;22(5):462-4
16463034
Hum Pathol. 2002 May;33(5):459-65
12094370
J Pediatr Gastroenterol Nutr. 2008 Nov;47(5):667-9
18979588
J Surg Res. 2009 Sep;156(1):95-102
19560163
BMC Cancer. 2007 May 09;7:79
17490483
Arch Pathol Lab Med. 2006 Oct;130(10):1466-78
17090188
Cancer. 2004 Jul 1;101(1):39-50
15221987
Cancer Invest. 2008 Mar;26(2):182-4
18259950
Histopathology. 2000 Oct;37(4):316-22
11012738