Howard Rosenthal, M.D. Associate Professor of Orthopedic Surgery University of Kansas Sarcoma Center I have no disclosures or any conflicts related to the content of this presentation. Objectives 1. Describe malignant and benign bone and soft tissue tumors. 2. Analyze treatment options for the patient with a musculoskeletal neoplasm. 1
Malignant Bone and Soft Tissue Tumors Neoplastic growths arising from the mesenchymal system (mesoderm) In contrast to carcinoma derived from ectoderm and endoderm Termed Sarcoma (Greek fleshy, or fish flesh) Soft Tissue Sarcoma 40 subtypes Bone Sarcoma 20 subtypes All together, accounting for 1% of all cancers May occur in any part of body Occurs in all age ranges Specific types of tumor occur in specific age ranges No specific risk factors or predisposing features Exceptions include: NF, Li Fraumeni s, Gardner s ) Certain chemical exposures (agent orange) No specific serum markers, signs or symptoms Malignant Bone Tumors 6 % of all childhood malignancies Annual U.S. Incidence 8.7 per million Approx. 650 700 children/year Most common osteosarcoma, Ewing s Sarcoma Carcinomas Annual Incidence Overall 4697 per million Lung 610 per million Breast 633 per million 2
Sarcomas are derived from the primitive pluripotential mesenchymal cells These pluripotential mesenchymal stem cells: Bone Cartilage Fat Blood Vessels Nerves Fibrous Tissue Soft tissue and bone viscera (gastrointestinal, genitourinary, and gynecologic organs) nonvisceral soft tissues (muscle, tendon, adipose, pleura, and connective tissue) By differentiation (usually with IHC staining) adipocytic tumors fibroblastic/myofibroblastic tumors fibrohistiocytic tumors smooth muscle tumors pericytic (perivascular) tumors primitive neuroectodermal tumors (PNETs) skeletal muscle tumors vascular tumors osseous tumors tumors of uncertain differentiation 3
Telangiectatic OGS Small Cell OGS Osteoblastic Chondroblastic Fibroblastic Defined and described based on the mature tissue that they resemble most. (Pattern of Differentiation) Lipomatous tumor = liposarcoma Fibrous tumor fibrosarcoma BioMolecular Markers are redefining the definition of these cancers MDM2 positive fat cell is liposarcoma EWS round cell is Ewing s Sarcoma Initially starting as a chance mutation coding for specific sarcoma, in given environment Clonal growth in centripetal spherical fashion Pushing aside of normal tissues to form pseudocapsule (which is laden with malignant cells) Enzymatic degradation of BM, vascular walls, followed by hematogenous spread to other organs, most specifically lungs. Both local and distant destruction of adjacent tissues until demise of host. 4
Normal Muscle (Tissue) Pseudocapsule Tumor Reactive Zone or Pseudocapsule Tumor Compressing Muscle and Infiltrating between Muscle Fibers 5
Local growth obeys fascial borders and compartments Fascial borders are good biological barriers to tumor spread Extent of tumor growth defines stage of disease Size, depth, compartmentalization, and grade all define stage Purpose Defines expectation of tumor biology Determines treatment plans Determines prognosis Enables results of treatments among many patients to be compared and evaluated Delineates extent of local and disseminated disease Based on specific cancer type (sarcoma staging versus carcinoma staging) History Length of time mass has been present Rate of growth Pain, (with exercise, at rest, nocturnal, radiating) Ecchymosis, trauma, injury, N/T/W Physical Examination Size Mobility Location, depth, juxtaposition to other structures Texture 6
Radiographic evaluation Plain Films CT Scan (w/wo contrast) MRI Scan (with gadolinium) Nuclear Medicine Studies (Bone Scan, PET Scan) MRA, Angiography, U/S, Thallium (less used now) Staging Studies Biopsy CT CAP 1. Where is the tumor and what is rate of growth? 2. What is tumor doing to surrounding tissues? 3. What are surrounding tissues doing to tumor? 4. What is the matrix or identifiable features of the tumor as determined by radiographic and PE? 7
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Geographic Lesion, diaphyseal, without Matrix, without periosteal reaction, slighty thickened cortices 10
Geographic, epiphyseal, without matrix, cortical disruption, but with some increased reactive bone margins - GCT Metaphyseal, expansile, fluid levels cortical disruption ABC! Soft Tissue Sarcomas (Biological Behavior) Tumors that are typically High Grade Round Cell Liposarcoma Intramuscular Leiomyosarcoma Rhabdomyosarcoma Angiosarcoma ES Ewing s Sarcoma ES Osteosarcoma Synovial Sarcoma 11
Soft Tissue Sarcomas (Biological Behavior) Tumors that are typically Low Grade Well Differentiated Liposarcoma DermatoFibroSarcoma Protuberans Cutaneous Leiomyosarcoma Desmoid Retroperitoneal Liposarcoma Retroperitoneal Liposarcoma 12
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Myxofibrosarcoma of Biceps 14
High Grade Liposarcoma with pseudocapsule 15
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Any soft tissue mass > 5 cm. laying deep the the limiting fascia must raise concern for STS Any soft tissue mass in a child Hematomas have to occur for a reason Lipomas feel and behave like lipomas When you hear hoof beats, look for horses not zebras All zoos have zebras (none have horses) 19
No Pain Slowly enlarging Any part of body Any age Any texture No risk factors Not hereditary 20
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