Unusual presentation of a large GIST in an extraintestinal site: a challenging diagnosis dilemma

Gastrointestinal stromal tumour (GIST) is a recent recognised tumour entity. In the past, those tumours were classified as leiomyomas, leiomyosarcomas and leiomyoblastomas, but it is now evident that GIST is a separate tumour entity and is the most common sarcoma of the gastrointestinal tract especially with advances in immunohistochemical staining techniques and improvements in microscopic structural imaging. We present a case of GIST of unusual location and presentation pattern, with an overview over current GISTs’ diagnosis and management strategies. The precise incidence and tumour behaviour of rare extragastrointestinal stromal tumour (EGIST) remain to be clarified. Further research is needed in large series with long duration of follow-up and modified risk stratification assessment tailored for EGISTs.