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xxx00.#####.ppt 9/23/2012 9:01:18 AM
Musculoskeletal Sarcomas
Robert C. Orth, M.D., Ph.D.
Edward B. Singleton Department of Pediatric Radiology Texas Children’s Hospital
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No disclosures
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Outline
•Bone -MR protocol -Report contents -Tumor types
•Osteosarcoma
•Ewing sarcoma
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Outline
•Soft tissue -Tumor types
•Rhabdomyosarcoma •Synovial sarcoma •Infantile fibrosarcoma
-Fatty lesions •DDx under 5 years old •Liposarcoma
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Extremity tumor protocol
•T1 coronal: joint to joint
•STIR coronal: joint to joint
•Small FOV -T1 axial
-T2 FS axial
-PD FS sagittal
-Post-Gd T1 FS axial and sagittal or coronal
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10 year-old boy with 1 month leg pain
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Chondroblastic osteosarcoma
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Osteosarcoma
•Vast majority conventional -Metaphysis– majority around knee
-High grade
-Osteoblastic, chondroblastic, and fibroblastic
•Rare non-conventional variants -Telangiectatic
-Surface variants
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Osteosarcoma
•Well-established treatment algorithm
•Surgical plan usually from initial MR
MRI Neoadjuvant chemo Resection
Adjuvant chemo Biopsy MRI
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In report
•Longitudinal medullary extent -T1
-T2 –nonspecific
•Epiphysis
•Joint
•Soft tissue mass – neurovascular bundle
5.7 cm
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13 year-old boy with painless growth
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Chondroblastic osteosarcoma
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Diaphyseal osteosarcoma
•Prognosis similar to medullary osteosarcoma
•More likely to be osteolytic
•DDX -Ewing sarcoma
-Lymphoma
-LCH
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16 year-old boy Chondroblastic osteosarcoma with skip lesion
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9 year-old girl with pain
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Telangiectatic osteosarcoma
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15 year-old boy
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Ewing sarcoma
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Ewing sarcoma
•DDx – lymphoma, LCH, infection
•10 – 25 year of age (median 15)
•Large soft tissue component – responds well to initial chemotherapy
•Non-metastatic survival – 65%
•Lung mets
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Initial
Post 8 weeks chemotherapy
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Soft tissue masses
•Vast majority benign
•STS 4-8 % childhood cancer
•Big is bad
•MR very sensitive and very nonspecific
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9 year-old boy with slowly growing right foot mass
Alveolar rhabdomyosarcoma
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Rhabdomyosarcoma
•Nearly half of all soft tissue sarcomas
•Embryonal and alveolar subtypes most common
•Alveolar
-Older children
-Extremities
•PET
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3-year old girl with RMS
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16 year-old girl with right upper thigh pain and swelling for 1 month
Synovial sarcoma
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Synovial sarcoma
•Second most common soft tissue sarcoma
•5-10 percent of sarcomas
•Up to half younger than 20
•Rarely arises within joint or bursa
•Mimics benign processes
•Calcification and hemorrhage
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Prenatally diagnosed mass
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Infantile rhabomyofibrosarcoma
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Infantile fibrosarcoma
•Most common soft-tissue sarcoma <1 year
•Median age at diagnosis 3 months - 40% diagnosed at birth
•Distal extremities most common
•Locally aggressive, metastasis rare
•50% require amputation
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11 days old
Post 6 weeks VAC
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17 day-old girl 5 day-old boy
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Fatty masses in children under 5
•Lipoblastoma
•Fibrous hamartoma of infancy
•Involuting hemangioma
•Lipoma
•Liposarcoma
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4 year-old girl with left palm mass for several years
Lipoblastoma
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Lipoblastoma
•Usually under 8 years old - average 3.6
•Histology -Immature adipocytes
-Myxoid stroma
-Vascular septa
•Lipoblastomatosis: diffuse, infiltrative
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4 month-old boy with left buttock lipoblastoma
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6 month-old with right knee mass Fibrous hamartoma of infancy
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Fibrous hamartoma of infancy
•90% first year of life
•Histology -Trabeculae of fibrocollagenous tissue
-Islands of primitive mesenchyma
-Mature fat
•Rapidly growing, solitary, mobile
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6 month-old with fibrous hamartoma of infancy
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8 month-old girl with growing bluish mass over shoulder
Infantile hemangioma
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17 year-old female with mass Myxoid liposarcoma
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Liposarcoma
•Very rare in children
•3/4 myxoid
•Not in DDx under 5
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Summary
•Bone sarcomas -Medullary extent: T1
-Limb sparing
•Epiphysis •Intra-articular •Neurovascular bundle
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Summary
•Soft tissue sarcomas -Rhabdomyosarcoma - PET
-Synovial sarcoma – can mimic benign lesions
-Fat-containing lesion under 5 → benign
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Thank you
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