Surgical Pathology Criteria

Gastrointestinal Stromal Tumor (GIST)

Succinate Dehydrogenase Subunit B (SDHB) Deficient (PediatricType) GIST

SDHB Deficient (Pediatric Type) GIST Usual GIST, (SDHB Positive)
Predominantly pediatric and young adult, rare middle age to older adults Predominantly older adults
F:M ratio as high as 9:1 M = F
All are gastric, most in antrum May occur throughout gastrointestinal tract
Frequently multiple, simultaneous or metachronous Usually solitary
Frequently multilobular Generally one dominant mass
Predominantly epithelioid, occasionally mixed May be epithelioid or spindled
Lymph node metastases common Lymph node metastases rare
Poor response to imatinib Responsive to imatinib
No CKIT or PDGFRA mutations CKIT or PDGFRA mutations present in about 90%
Protracted course (e.g. 15 years), even if metastatic Poor prognosis if metastatic

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