Musculoskeletal Sarcomas Robert C. Orth, M.D., Ph.D. Edward B. Singleton Department of Pediatric Radiology Texas Children s Hospital Page 0 xxx00.#####.ppt 9/23/2012 9:01:18 AM
No disclosures Page 1 xxx00.#####.ppt 9/23/2012 9:01:20 AM
Outline Bone -MR protocol -Report contents -Tumor types Osteosarcoma Ewing sarcoma Page 2 xxx00.#####.ppt 9/23/2012 9:01:21 AM
Outline Soft tissue -Tumor types Rhabdomyosarcoma Synovial sarcoma Infantile fibrosarcoma -Fatty lesions DDx under 5 years old Liposarcoma Page 3 xxx00.#####.ppt 9/23/2012 9:01:22 AM
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 Page 4 xxx00.#####.ppt 9/23/2012 9:01:23 AM
10 year-old boy with 1 month leg pain Page 5 xxx00.#####.ppt 9/23/2012 9:01:24 AM
Chondroblastic osteosarcoma Page 6 xxx00.#####.ppt 9/23/2012 9:01:25 AM
Osteosarcoma Vast majority conventional -Metaphysis majority around knee -High grade -Osteoblastic, chondroblastic, and fibroblastic Rare non-conventional variants -Telangiectatic -Surface variants Page 7 xxx00.#####.ppt 9/23/2012 9:01:27 AM
Osteosarcoma Well-established treatment algorithm Surgical plan usually from initial MR MRI Biopsy Neoadjuvant chemo MRI Resection Adjuvant chemo Page 8 xxx00.#####.ppt 9/23/2012 9:01:27 AM
In report Longitudinal medullary extent -T1 -T2 nonspecific Epiphysis 5.7 cm Joint Soft tissue mass neurovascular bundle Page 9 xxx00.#####.ppt 9/23/2012 9:01:28 AM
13 year-old boy with painless growth Page 10 xxx00.#####.ppt 9/23/2012 9:01:29 AM
Chondroblastic osteosarcoma Page 11 xxx00.#####.ppt 9/23/2012 9:01:30 AM
Diaphyseal osteosarcoma Prognosis similar to medullary osteosarcoma More likely to be osteolytic DDX -Ewing sarcoma -Lymphoma -LCH Page 12 xxx00.#####.ppt 9/23/2012 9:01:31 AM
16 year-old boy Chondroblastic osteosarcoma with skip lesion Page 13 xxx00.#####.ppt 9/23/2012 9:01:32 AM
9 year-old girl with pain Page 14 xxx00.#####.ppt 9/23/2012 9:01:33 AM
Telangiectatic osteosarcoma Page 15 xxx00.#####.ppt 9/23/2012 9:01:35 AM
15 year-old boy Page 16 xxx00.#####.ppt 9/23/2012 9:01:35 AM
Ewing sarcoma Page 17 xxx00.#####.ppt 9/23/2012 9:01:37 AM
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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 Page 19 xxx00.#####.ppt 9/23/2012 9:01:38 AM
Initial Post 8 weeks chemotherapy Page 20 xxx00.#####.ppt 9/23/2012 9:01:39 AM
Soft tissue masses Vast majority benign STS 4-8 % childhood cancer Big is bad MR very sensitive and very nonspecific Page 21 xxx00.#####.ppt 9/23/2012 9:01:40 AM
9 year-old Alveolar boy rhabdomyosarcoma with slowly growing right foot mass Page 22 xxx00.#####.ppt 9/23/2012 9:01:41 AM
Rhabdomyosarcoma Nearly half of all soft tissue sarcomas Embryonal and alveolar subtypes most common Alveolar PET -Older children -Extremities Page 23 xxx00.#####.ppt 9/23/2012 9:01:42 AM
3-year old girl with RMS Page 24 xxx00.#####.ppt 9/23/2012 9:01:43 AM
16 year-old Synovial girl with sarcoma right upper thigh pain and swelling for 1 month Page 25 xxx00.#####.ppt 9/23/2012 9:01:44 AM
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 Page 26 xxx00.#####.ppt 9/23/2012 9:01:45 AM
Prenatally diagnosed mass Page 27 xxx00.#####.ppt 9/23/2012 9:01:45 AM
Infantile rhabomyofibrosarcoma Page 28 xxx00.#####.ppt 9/23/2012 9:01:46 AM
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 Page 29 xxx00.#####.ppt 9/23/2012 9:01:47 AM
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11 days old Post 6 weeks VAC Page 31 xxx00.#####.ppt 9/23/2012 9:01:49 AM
5 day-old boy 17 day-old girl Page 32 xxx00.#####.ppt 9/23/2012 9:01:50 AM
Fatty masses in children under 5 Lipoblastoma Fibrous hamartoma of infancy Involuting hemangioma Lipoma Liposarcoma Page 33 xxx00.#####.ppt 9/23/2012 9:01:51 AM
4 year-old Lipoblastoma girl with left palm mass for several years Page 34 xxx00.#####.ppt 9/23/2012 9:01:52 AM
Lipoblastoma Usually under 8 years old - average 3.6 Histology -Immature adipocytes -Myxoid stroma -Vascular septa Lipoblastomatosis: diffuse, infiltrative Page 35 xxx00.#####.ppt 9/23/2012 9:01:53 AM
4 month-old boy with left buttock lipoblastoma Page 36 xxx00.#####.ppt 9/23/2012 9:01:54 AM
6 Fibrous month-old hamartoma with right of knee infancy mass Page 37 xxx00.#####.ppt 9/23/2012 9:01:54 AM
Fibrous hamartoma of infancy 90% first year of life Histology -Trabeculae of fibrocollagenous tissue -Islands of primitive mesenchyma -Mature fat Rapidly growing, solitary, mobile Page 38 xxx00.#####.ppt 9/23/2012 9:01:55 AM
6 month-old with fibrous hamartoma of infancy Page 39 xxx00.#####.ppt 9/23/2012 9:01:56 AM
8 month-old Infantile girl hemangioma with growing bluish mass over shoulder Page 40 xxx00.#####.ppt 9/23/2012 9:01:57 AM
17 year-old Myxoid female liposarcoma with mass Page 41 xxx00.#####.ppt 9/23/2012 9:01:58 AM
Liposarcoma Very rare in children 3/4 myxoid Not in DDx under 5 Page 42 xxx00.#####.ppt 9/23/2012 9:01:59 AM
Summary Bone sarcomas -Medullary extent: T1 -Limb sparing Epiphysis Intra-articular Neurovascular bundle Page 43 xxx00.#####.ppt 9/23/2012 9:02:00 AM
Summary Soft tissue sarcomas -Rhabdomyosarcoma - PET -Synovial sarcoma can mimic benign lesions -Fat-containing lesion under 5 benign Page 44 xxx00.#####.ppt 9/23/2012 9:02:01 AM
Thank you Page 45 xxx00.#####.ppt 9/23/2012 9:02:01 AM