Primary leiomyosarcoma of femur: A rare entitycase report and review of literature

Background: We report this case to highlight clinical and pathological features of this rare entity. Methods: It is about a 48 year-old woman with an osteolytic lesion of the lower extremity of the left femur. Result: A biopsy of the lesion showed densely cellular malignant mesenchymal proliferation. It was made by bundles of smooth muscle cells with nuclei of moderate to severe atypia. The immunohistochemical study showed an intense and diffuse cytoplasmic positivity of tumor cells with the smooth anti-muscle. Desmin was focal positive. After neoadjuvant chemotherapie, a knee block resection was made. Macroscopically, it was a 10 cm sized tumor located in the distal part of tumor. Histologically tumor showed post chemotherapy effects estimated at 20%. The bone and muscle surgical limits were unscathed. Conclusion: Primary leiomyosarcoma of bone is a rare and a diagnostically challenging tumor entity