SDHB and metastatic malignant neoplasm in the lymph nodes: There are several subsets of GISTs with features distinct from those of conventional KIT- or PDGFRA-mutated GISTs, including pediatric GIST, neurofibromatosis type 1 associated GIST (NF1-GIST), Carney–Stratakis syndrome, the Carney triad, and familial GISTs (Table 1):Pediatric GISTs, which are predominantly found in the female stomach, are frequently associated with predominant epithelioid features, lymph node metastasis, and mutations in the SDH complex.