In:
APMIS, Wiley, Vol. 121, No. 10 ( 2013-10), p. 938-944
Abstract:
Studies on gastrointestinal stromal tumors ( GIST s) in young patients are limited due to their rarity, and none have been conducted in Asian populations. GIST s from patients under the age of 30 were retrospectively reviewed and were analyzed for clinicopathologic features, immunohistochemistry for SDHB (succinate dehydrogenase subunit B), and mutations for exon 9, 11, 13, and 17 of KIT gene and exon 12, 14, and 18 of PDGFRA gene. We found two pediatric ( 〈 18 years old) and 20 young adult (18–30 years old) GIST cases. Pediatric GIST s occurred in two girls, both as solitary masses with epithelioid histology in the stomach. Both GIST s were wild type for KIT and PDGFRA genes, were negative for SDHB , and there was no recurrence during follow‐up. Of the 20 GIST s in young adults, 12 (60%) were from extra‐gastric locations (six duodenum, five jejunum, and one esophagus), and 16 (80%) showed a spindle cell morphology. Mutations of KIT or PDGFRA genes were identified in 14 (78%) of the 18 cases. One patient with multiple gastric GIST s with perigastric lymph node metastases at presentation developed multiple distant metastases and died of the disease 7.3 years after diagnosis. Of the 19 R0‐resected young adult patients, one patient with small intestinal GIST harboring KIT exon 11 deletion mutation developed recurrence and showed partial responses for imatinib. In summary, compared with pediatric GIST cases, young adult GIST s are heterogeneous and share the characteristics of both pediatric and adult GIST s. When a mesenchymal tumor is clinically suspected in the small intestine of young adults, a GIST should be included in the differential diagnoses. Further mutation studies and extensive treatments are recommended for these cases.
Type of Medium:
Online Resource
ISSN:
0903-4641
,
1600-0463
DOI:
10.1111/apm.2013.121.issue-10
Language:
English
Publisher:
Wiley
Publication Date:
2013
detail.hit.zdb_id:
2098213-6
SSG:
12
