8-year-old with osteosarcoma of the right humerus Amy Millar March 2013 James Cameron, MD

8 yo F diagnosed w/ resectable, localized osteosarcoma of R humerus 4/19/12 at OSH Family refused treatment; received non-conventional therapy in AZ (ozone, IV vitamin C) When patient did not improve, parents took her to a hospital in NY where new pulmonary mets were seen. She received “palliative radiation” to humerus and lungs Presented to Rush on 8/19/12 due to severe pain in right arm Limb salavage not feasible/pulmonary mets not resectable at this time Patient Presentation 2

Most common malignant bone tumor Rare in pts <5 yo, increases with age Most common in long bones near metaphyseal growth plate Femur (42%), tibia (19%), humerus (10%) are most common locations Classic/conventional OS is most common subtype Most commonly presents with pain with activity Osteosarcoma

Ddx solitary lucent bone lesions FEGNOMASHIC F - fibrous dysplasia OR fibrous cortical defect (FCD) E - enchondroma OR eosinophilic granuloma (EG) G - giant cell tumour (GCT) N - non-ossifying fibroma (NOS) O - osteoblastoma M - metastasis(es) A - aneurysmal bone cyst (ABC) S - simple (unicameral) bone cyst H - hyperparathyroidism (brown tumour) I - infection (osteomyelitis) C - chondroblastoma OR chondromyxoid fibroma

1º Bone Tumors: ACR Appropriateness Criteria Radiologic Procedure RatingCommentsRelative Radiation Level Xray area of interest9Absolute requirement in pt with suspected bone lesion Varies US area of interest10 MRI area of interest10 Tc-99m bone scan whole body 1 ☢ ☢ ☢☢ ☢ ☢ CT area of interest w/o contrast 1Varies FDG-PET/CT whole body1 ☢ ☢ ☢ ☢☢ ☢ ☢ ☢ Screening, First Study Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate

1º Bone Tumors: ACR Appropriateness Criteria Radiologic Procedure RatingCommentsRelative Radiation Level MRI area of interest w/ or w/o contrast 9Contrast can provide more information. Useful for vascularity and necrotic areas. 0 CT area of interest w/o contrast 5May be useful if MRI not available or possible. Useful for evaluation of calcification, cortical breakthrough and pathological fractures. Varies FDG-PET/CT whole body5Can be useful for problem solving. ☢ ☢ ☢ ☢☢ ☢ ☢ ☢ Tc-99m bone scan whole body 3Probably not indicated, except to look for additional lesions. ☢ ☢ ☢☢ ☢ ☢ US area of interest10 Suspicious for malignant characteristics on radiograph Rating Scale: 1,2,3 Usually not appropriate; 4,5,6 May be appropriate; 7,8,9 Usually appropriate

Radiographic findings of OS 7 Elevation of periosteum 2/2 reactive bone formation (“Codman triangle”) Mixed sclerotic/lytic appearance is common Mineralized osteoid may be seen in adjacent soft tissue

Radiographic findings of OS Intramedullary skip lesions may be present Occasionally may present as a pathologic fx

Radiographic findings, cont. 9 - Abnormal medullary signal intensity - Soft tissue mass - Decr signal intensity - Soft tissue mass - Extension into epiphysis Evaluate for longitudinal extent of interosseous disease, involvement of adjacent epiphyses, presence of skip lesions Evaluate for extent of extraosseous disease MRI to assess tumor distribution and associated soft tissue mass (staging)

Normal comparison Right Shoulder AP Mixed sclerotic and lytic lesion Soft tissue mass and swelling

Right Humerus AP and Lateral Periosteal reaction

Right Shoulder T1 Coronal MRI Pre Contrast

Right Shoulder T1 Coronal MRI Post Contrast

Axillary vasculature

Right Shoulder T1 Coronal MRI Post Contrast

Right Shoulder T1 Axial MRI

Court-ordered chemo started on 9/6/12 Underwent resection of several small pulmonary mets in 2/2012 and 3/2012 Primary tumor remains unresectable Pt MRN Hospital Courseto Date