Telangiectatic osteosarcoma

Introduction

Occur in same location as ABC (look similar radiographically as well)

Symptoms

Presentation 25% present with pathologic fracture

Imaging

Radiographs lytic, destructive, and expansile lesion Bone scan hot

Histology

Characterized by few cellular components (those present have malignant characteristics) not as much osteoid as intramedullary osteosarcoma  lakes of blood mixed with malignant cells (not in ABC)

Treatment

Same as intramedullary osteosarcoma   multi-agent chemotherapy and limb salvage resection chemotherapy preoperative chemotherapy given for 8-12 weeks followed by maintenance chemotherapy for 6-12 months after surgical resection 98% necrosis with chemo is good prognostic sign expression of multi-drug resistence (MDR) gene portends very poor prognosis cells can pump chemo out of cell present in 25% of primary lesions and 50% of metastatic lesions surgical resection trend towards limb salvage whenever possible

Prognosis

More chemosensitive but same survival as intramedullary osteosarcoma