Table 1 Comparison between Carney triad GIST (case 1), pediatric (case 2) and young adult GIST (case 3)
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 | ? | ? |