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

?

?

CT = Carney triad, GIST = gastrointestinal stromal tumor, WT = wild type, ? = not to date

Otto et al. Diagnostic Pathology 2011 6:52   doi:10.1186/1746-1596-6-52