Table 1.
CT-GIST (case 1) | Pediatric GIST (case 2) | Young adult GIST (case 3) | |
---|---|---|---|
Age at diagnosis (years) | 15 | 13 | 23 |
Gender | female | female | female |
Tumor localisation | stomach, greater curvature, antrum |
stomach, lesser curvature, antrum |
stomach, corpus |
Growth pattern | multinodular | multinodular | multinodular |
Metastases | liver (lymphangioinvasion) |
liver lymph nodes |
liver lymph nodes |
Histomorphology | biphasic (spindled and epithelioid), hypercellular, plasmacytoid aspect |
biphasic (spindled and epithelioid), hypercellular, plasmacytoid aspect |
liver metastasis: hypercellular, plasmacytoid aspect lymph node metastasis: closely packed hypercellular tumor nodules with lymphangioinvasion |
Immunohisto- chemistry |
C-KIT/CD34 + (predominantly membranous) |
C-KIT/CD34 + (predominantly membranous) |
C-KIT/CD34 + (predominantly membranous) |
Mutational status | WT for common C-KIT (exon 9,11,13,17) and PDGFRA (exon 12,14,18) mutations PDGFRA Exon 10 polymorphism (c. 1432 T > C; p. S478P, no alteration on protein level) |
WT for common C-KIT (exon 9,11,13,17) and PDGFRA (exon 12,14,18) mutations |
WT for common C-KIT (exon 9,11,13,17) and PDGFRA (exon 12,14,18) mutations |
Associated diseases | paraaortal paraganglioma |
? | ? |
CT = Carney triad, GIST = gastrointestinal stromal tumor, WT = wild type, ? = not to date