Leiomyosarcoma of the stomach is a very rare malignancy that was not distinguished from the more frequent gastrointestinal stromal tumors until early 2000s. Here we report on a case of a metastatic disease that developed in a 47-year-old man 2 years after he was diagnosed with the primary tumor and treated with curative surgical excision and adjuvant doxorubicin. The primary and metastatic lesions were positive for smooth muscle markers α-smooth muscle actin and h-caldesmon and negative for CD117, DOG-1 and S100 by immunohistochemistry. Metastatic disease progressed on additional monotherapy with doxorubicin and docetaxel-gemcitabine combination, and stable disease was achieved upon treatment with pazopanib. Patient is surviving 35 months since diagnosis of the primary tumor and 11 months since diagnosis of metastatic disease.
Keywords: GIST; docetaxel; doxorubicin; gastrointestinal stromal tumors; gemcitabine; leiomyosarcoma; pazopanib; soft tissue sarcoma.