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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