Bone Tumors Occurring in the Soft Tissues: A Review of the Clinical, Imaging, and Histopathologic Findings

Abstract

Although rare in everyday practice, malignancies that classically arise from bone or cartilage have been reported to arise de novo in various soft tissues in the body, resulting in a diagnostic challenge for the clinician, radiologist, and pathologist. Differential diagnoses of bone tumors often depend on anatomic location of the lesion. For example, the classic location of osteosarcoma is in the metaphysis of long bones about the knee. Histologically osteosarcoma is characterized by tumor cells that directly produce osteoid, bone, or cartilaginous matrix. In extraskeletal osteosarcoma, the clinical and radiologic picture is very different from a conventional osteosarcoma. They occur in older patients, present as a soft tissue mass often coincidentally following trauma and have a worse prognosis. The imaging characteristics are often nonspecific with mineralized elements in a well-defined soft tissue mass. The mineralized elements may or may not be visible. Magnetic Resonance sequences demonstrate a well circumscribed soft tissue mass with hemorrhagic and enhancing solid components. The pathologic features of extraskeletal osteosarcoma on a microscopic scale are identical to that of skeletal lesions. Likewise, conventional chondrosarcomas present in older patients with a growing, painful soft tissue prominence most commonly involving the long tubular bones. In extraskeletal chondrosarcoma however, the presentation is in somewhat younger patients with a painful soft tissue prominence typically in the head (meninges), neck, or upper leg. The pathologic features are most often that of a myxoid chondrosarcoma which is characterized by strands of small cells over a myxoid matrix. Imaging features include chondroid matrix, heterogenous contrast enhancement, and amorphous internal calcification on Computed Tomography. On Magnetic Resonance sequences the matrix has a low signal on all sequences, and variable inhomogeneity depending on grade of the lesion. Other extraskeletal bone tumors include Ewing's sarcoma and osteoid osteoma amongst other lesions. Although these malignancies may be rare clinical entities, they often exhibit characteristic clinical, imaging, and histopathological findings although differing in treatment and prognosis. Knowledge of these and other common mimicking lesions will help guide the clinician and radiologist to make an accurate diagnosis.