Bilateral gluteal metastases from a misdiagnosed intrapelvic gastrointestinal stromal tumor

BACKGROUND: The location of gastrointestinal stromal tumors (GIST) outside of the gastrointestinal system is a rare event. CASE PRESENTATION: A 56-year old woman presented with a GIST of the pelvis was misdiagnosed and treated as a uterine leiomyosarcoma. The diagnosis was made after the CD117 (KIT) positivity in the biopsy of the excised bowel mass four years from the first presentation. During this period she presented a bilateral muscle and subcutaneous metastasis in the gluteal area. CONCLUSION: The correct diagnosis of the extra-gastrointestinal stromal tumor is a challenge even for experienced pathologists. CD117 (KIT) positivity is the most important immunohistochemical feature in the histological diagnosis. To our knowledge a metastatic EGIST (extra-gastrointestinal stromal tumor) to the skeletal muscle bilaterally has not been described previously in the English medical literature.