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When is a musculoskeletal condition a tumor? Recognizing common and tumors

Christian M. Ogilvie, MD Assistant Professor of Orthopaedic Surgery University of Pennsylvania

University of Pennsylvania Department of Orthopaedic Surgery Purpose

• Recognize that tumors can present in the extremities of patients treated by athletic trainers • Know that tumors may present as a lump, pain or both • Become familiar with some bone and soft tissue tumors

University of Pennsylvania Department of Orthopaedic Surgery Summary • Introduction – Pain – Lump • Bone tumors – Malignant – Benign • Soft tissue tumors – Malignant – Benign

University of Pennsylvania Department of Orthopaedic Surgery Summary

• Presentation • Imaging • History • Similar conditions –Injury

University of Pennsylvania Department of Orthopaedic Surgery Introduction • tumors -Bone - -Muscle -Fat -Synovium (lining of , tendons & bursae) -Nerve -Vessels •Malignant (cancerous): •Benign

University of Pennsylvania Department of Orthopaedic Surgery Introduction: Pain

• Malignant bone tumors: usually • Benign bone tumors: some types • Malignant soft tissue tumors: not until large • Benign soft tissue tumors: some types

University of Pennsylvania Department of Orthopaedic Surgery Introduction: Pain

• Bone tumors – Not necessarily activity related – May be worse at night – Absence of trauma, mild trauma or remote trauma • Watch for referred patterns – Knee pain for hip problem – Arm and leg pains in spine lesions

University of Pennsylvania Department of Orthopaedic Surgery Introduction: Lump

• Malignant soft tissue tumors – Usually become noticeable over several months – Synovial sarcoma, commonly seen in young adults • May be present and unchanged for years • Most tumors grow steadily – Decrease in size generally means a nontumor process or hemangioma (benign blood vessel tumor)

University of Pennsylvania Department of Orthopaedic Surgery Introduction

• Be vigilant! • Exceptions exist to most guidelines

University of Pennsylvania Department of Orthopaedic Surgery Malignant Bone Tumors

• – Central – Surface • Ewings Sarcoma

University of Pennsylvania Department of Orthopaedic Surgery Central Osteosarcoma • High grade • Metaphyseal: knee, proximal • Permeative, erosions, soft tissue mass, variable bone formation, periosteal elevation • DDx: GCT, ABC, Ewing’s, lymphoma, , met • Wide resection •

University of Pennsylvania Department of Orthopaedic Surgery Surface Osteosarcoma

Parosteal Periosteal • Low grade 90% • High grade • Slow growth, painless mass • and ; may be • Posterior distal femur diaphyseal • Well ossified on intact cortex • Well ossified at base • DDx: myositis, periosteal , exostosis • Cortical involvement, erosion with soft tissue mass • Lacks cellular atypia • May dedifferentiate • Wide resection • Wide resection • Chemotherpay • Chemo if high grade

University of Pennsylvania Department of Orthopaedic Surgery Ewing’s Sarcoma

• 5 – 30 years old • Small round blue cells; • Pain, mass, anemia, CD99+; EWS/FLI-1 leucocytosis •Chemotherapy • Pelvis and lower extremity • Surgery if resectable, • Large soft tissue mass with radiation considered if not small resectable • DDx: osteomyelitis, lymphoma, OGS, EG, neuroblastoma met

University of Pennsylvania Department of Orthopaedic Surgery Benign Bone Tumors

• tumor • • oseoma • Solitary – AKA unicameral bone cyst

University of Pennsylvania Department of Orthopaedic Surgery Giant Cell Tumor • Aggressive • 20s to 30s; <5% with open physes; 80% > 20 yo • M:F = 2:3 • Pain, fracture, swelling; mets in 2% • 50% around knee; epiphyseal/metaphyseal, may be subchondral • Lytic, expansile, geographic, neocortex

University of Pennsylvania Department of Orthopaedic Surgery Giant Cell Tumor

• Campanacci stages: I lytic; II expansile; III destructive, soft tissue mass

• DDx: ABC, brown tumor, telangiectatic osteosarcoma, spindle cell sarcoma of • Stroma nuclei same as giant bone, osteoblastoma cell nuclei • Curretage, burr, adjuvant, graft/cement, +/- fixation; resect if expendible

University of Pennsylvania Department of Orthopaedic Surgery Osteoblastoma • Aggressive, may form • DDx: ABC, osteosarcoma, bone , • 10-35 yo , Brodie’s abcess, giant cell tumor, • M:F=2:1 osteoid • Pain, min. NSAID relief. • Post. spine, femur, tibia, • May have osteoid, ; metaph., diaph. fibrovascular stroma.

• Lytic, sclerotic or mixed, • Curettage and cementation, nidus usu. >2cm; grafting geographic; neocortex

University of Pennsylvania Department of Orthopaedic Surgery Chondroblastoma

• Aggressive • Teens • Small • Epiphyseal, next to • Inflammatory

University of Pennsylvania Department of Orthopaedic Surgery Chondroblastoma

University of Pennsylvania Department of Orthopaedic Surgery Osteochondroma • Latent • Pedunculated, sessile; • 10-35 yo cortical and medullary • M:F=2:1 continuity; bursa, cartilage cap – malignant if >2cm • Growth until maturity; usu. painless; late pain, • DDx: Reactive periosteal growth – impingement bone, sclerotic mets, vs. malignancy (<1%, 30 parosteal osteosarcoma, yo); MHE: 5-25% malig, chondrosarcoma Madelung, valgus knees • Cartilage cap, trabecular • Surface, firm, fixed; knee bone, normal marrow shoulder, hip - metaphyseal • Excision if symptomatic University of Pennsylvania Department of Orthopaedic Surgery Simple Bone Cyst • Latent or active • 80-90% < 20 yo • DDx: ABC, FD, CMF, telangiectatic osteosarcoma, • M>F , NOF • Fracture • Cyst lining: flattened spindle • Metaphyseal, grow cells away from joint, full • Injection of steroids vs width; 80% in proximal marrow; injection of graft – humerus and femur calcium phosphate; open • Lytic, some expansion, grafting, recurrence risk geographic, thin during growth sclerotic margin University of Pennsylvania Department of Orthopaedic Surgery

• Intracapsular: lacks sclerosis; • Active, bone forming Subperiosteal: min. periosteal • 10-35 yo response, cortical scalloping. • M:F=2:1 • DDx: , • Night pain, NSAID Brodie’s abscess, bone island, relief; effusion; increased osteoblastoma. bone growth, • Osteoid at center of nidus • Sclerotic with nidus usu. lined by , <1cm; long bones and fibrovascular stroma. posterior spine elements • RFA vs. resection

University of Pennsylvania Department of Orthopaedic Surgery Stress Fracture

• 26 yo female runner • Worsening pain for weeks to months

University of Pennsylvania Department of Orthopaedic Surgery Malignant Soft Tissue Tumors

• Treatment and challenges • Rhabdomyosarcoma • Synovial sarcoma • Malignant Fibrous Histiocytoma • Liposarcoma

University of Pennsylvania Department of Orthopaedic Surgery Malignant Soft Tissue Tumors

• Treatment – Wide resection – need to get it all out – Preop or postop radiation – Chemotherapy for 5cm or larger – Area of controversy / investigation but widely practiced • Problems – metastases – Wide resection may alter function

University of Pennsylvania Department of Orthopaedic Surgery Rhabdomyosarcoma • Most common soft tissue sarcoma in kids • 50% are in first decade • Second peak: 15 - 20 years old • Most common in head and neck and GU track • About 15% are in the extremities • Chemotherapy sensitive

University of Pennsylvania Department of Orthopaedic Surgery MFH

• Most common soft tissue sarcoma in adults • Painless mass

University of Pennsylvania Department of Orthopaedic Surgery Synovial Sarcoma •Cell of origin unknown •May grow slowly or be present for years •May calcify or ossify or contain cysts •May be monophasic or biphasic with more and less cellular areas; epithelioid •Translocation: t(x:18); 2 major types

University of Pennsylvania Department of Orthopaedic Surgery Synovial Sarcoma

University of Pennsylvania Department of Orthopaedic Surgery Myositis Ossificans •Usually history of trauma •MRI with contrast helpful: rule out enhancing soft tissue component of soft tissue tumor

University of Pennsylvania Department of Orthopaedic Surgery Liposarcoma

•May be associated with lipoma •Lipoblasts: indented nuclei •Round cell: >5% = worse prognosis

University of Pennsylvania Department of Orthopaedic Surgery Hematoma

•History of trauma or anticoagulant use

University of Pennsylvania Department of Orthopaedic Surgery Benign Soft Tissue Tumors

• Lipoma • Hemangioma • Pigmented villonodular (PVNS) • Myxoma • Benign peripheral nerve sheath tumor – AKA schwannoma, neurilemoma

University of Pennsylvania Department of Orthopaedic Surgery Lipoma • Can diagnose on MRI • Treatment – Excision if growing or has atypical features – : if discrete area of atypia on MRI • “Atypical” – Sometime referred to as grade I liposarcoma – Higher recurrence rate and rate

University of Pennsylvania Department of Orthopaedic Surgery Hemangioma

• May be able to diagnose on MRI • Treatment – Embolization for AV malformation – Sclerotherapy: EtOH • High recurrence rate after excision • Can be painful

University of Pennsylvania Department of Orthopaedic Surgery Myxoma • Most common adult benign muscle tumor • Typically middle aged female in hip region • Cannot distinguish from myxoid with 100% reliability using MRI • Biopsy

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis • Interarticular – Diffuse – Localized • Extrarticular – Diffuse – Nodular: giant cell tumor of tendon sheath • Malignant form – 3/8 previous PVNS; 4/8 lung mets, 2/8 inguinal node mets Bertoni F, et al Am J Surg Pathol. 1997

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis

• Peak 20s to 30s • M ankle/ • Tendon sheaths, bursae

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis

• Months to years of symptoms • Mass, restriction of motion, pain, swelling/effusion, locking,

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis • Xray: periarticular erosions

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis • MRI: T1 dark; T2 dark

University of Pennsylvania Department of Orthopaedic Surgery Pigmented Villonodular Synovitis

• Differential – Granulomatous lesions – Tendonous xanthoma – Fibroma of tendon sheath – Rheumatoid – – Posttraumatic reactive synovitis – Hemophilia

University of Pennsylvania Department of Orthopaedic Surgery BPNST

• May not have Tinel’s if not a sensory nerve • Treatment – Marginal excision • > 5 cm consider malignant variety

University of Pennsylvania Department of Orthopaedic Surgery BPNST

University of Pennsylvania Department of Orthopaedic Surgery Summary • Consider a tumor for any mass or unusual pain • Pain without significant trauma, persistent, night – Consider xray – Check for swelling/mass: if so, add MRI – Persistent pain with no or subtle xray changes: MRI • Bump that does not resolve quickly – May come up gradually or have just been noticed – May be painless – Consider MRI

University of Pennsylvania Department of Orthopaedic Surgery Thank You!

University of Pennsylvania Department of Orthopaedic Surgery

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