There are two main types of gastrointestinal connective tissue tumors: diff
erentiated connective tissue tumors arising from smooth muscle tissue, schw
anommas or fibroblastic tissue, and non-differentiated connective tissue tu
mors with no precise origin. Pathologically non-classifiable tumors compris
e a group of real gastrointestinal stromal tumors. Stromal tumors are rare
and generally develop in the stomach. Immunohistochemistry has been able to
identify three entities: stromal tumors with skenoid fibers, gastoinestina
l autonomic nerve tumors, and intraabdominal desmoplastic small round-cell
tumors. After resection, survival is almost 50% at 5 years and depends part
iculary on tumors size, the presence of synchronous metastases and mitosis
count.