Table 4.

Disorders featuring GIST and Neuroendocrine tumors (NET)

Disease	Associated gastrointestinal and abdominal tumors	Gene/s affected	Mode of inheritance
NF-1	Multiple GIST, NET (adrenal, ampulla, pancreas and other sites)	NF-1	Autosomal dominant
Multiple endocrine neoplasia type 1 & 2	Multifocal NETs, very rare cases of GISTs were reported in MEN-1 &MEN-2 patients	MEN-1 or RET	Autosomal dominant
Carney triad	Multiple gastric GIST and extra-adrenal paraganglioma	Unknown	Non-heritable
Carney-Stratakis syndrome	Familial GIST and multiple paragangliomas	SDH A,B,C,D	Autosomal dominant
Von Hippel Lindau disease	Renal cell carcinoma, endocrine pancreas tumors, one case of GIST reported	vHL	Autosomal dominant
Miscellaneous	GIST at different sites and various NET types (carefully exclude underlying syndromes)	Unknown	Sporadic, non-hereditary, etiology unknown