Gastrointestinal stromal tumors (CIST) represent an extremely rare group of
tumors, which are mostly of smooth muscle origin like leiomyomas, leiomyos
arcomas and leiomyoblastomas. With the introduction of immunohistochemical
analysis an epitheloid and an autonomic nerve variant can be distinguished.
The purpose of this review is to demonstrate the image morphological appea
rance of these rare tumors together with the pathology based upon a retrosp
ective analysis of five of our own cases since 1997. There are no pathognom
onic imaging findings for characterizing a gastrointestinal stromal tumor;
however, it should be included in the differential diagnosis if one or mult
iple large, round or oval, well-delineated gastrointestinal tumors occur in
combination with central necrosis. Carney's syndrome is characterized by t
he syndromal association of a gastrointestinal stromal tumor (originally: g
astric leiomyosarcoma) with an extra-adrenal paraganglioma and a pulmonary
chondroma. In this rare syndrome, the radiological approach is important to
diagnose or rule out the - simultaneous or consecutive - appearance of at
least two of the three tumor entities (GIST, extra-adrenal paraganglioma, p
ulmonary chondroma).