Intramedullary Well-differentiated Osteosarcoma: Imaging and Pathologic Findings in 17 Patients

Background: Intramedullary well-differentiated osteosarcoma (IMWDOS) is rare and may easily be misdiagnosed. Objective: This study aimed at investigating the clinical, imaging, and pathological features of IMWDOS for correct diagnosis. Materials and Methods: Seventeen patients with IMWDOS were enrolled, and their clinical, imaging, and pathological data were analyzed. Results: Total 13 males and 4 females aged 19-55 years (mean 36. 1) were selected. The lesion was located at long bones in 16 patients and the second region of the acetabulum in one patient. Except for three patients with limited areas of lesions, all the other patients had a wide distribution of tumor, and the lesion in long bones involved the metaphysis area with possible extension towards the diaphysis. In imaging, the lesion usually had an unclear boundary with the destruction of bone cortex, uneven thickness of the bone cortex, thick and coarse trabecula in the lesion, but few periosteal reactions and soft tissue masses. The lesion was histologically composed of spindle cells with slight atypia. Follow-up was performed 2-101 months (mean 31.9m) in 14 cases, 10 years in one case, and 26 years in the remaining two. At follow-up, 12 patients (12/17 or 70.6%) who had a complete resection, including amputation (n=2), wide excision (n=8), and endoprosthetic replacement (n=2), showed no recurrence or metastasis. Among five patients who underwent curettage, three (3/17 or 17.6%) had recurrent lesions, leading to death in two of them, and the third one died during post-operation chemotherapy. Conclusion: Intramedullary well-differentiated osteosarcoma tends to occur at the metaphysis of long bones, especially at the distal femur. Histological, clinical, and imaging findings lack specific characteristics and should be examined collectively to reach a correct diagnosis. The prognosis of patients with complete lesion resection is good, while incomplete lesion curettage or resection will lead to recurrence and transformation into a highly malignant tumor.