Other sarcomas
Key points
Soft tissue sarcomas
Desmoplastic Small Round Cell Tumour
Abdominal or pelvic region
Requires aggressive therapy combining chemotherapy, extensive surgery, and whole abdomen radiation
The use of Heated Intraperitoneal Chemotherapy (HIPEC) remains under discussion
Infantile Fibrosarcoma
Hands or feet in infants
Under two years old
Shows localised aggression but seldom spreads to other parts of the body
Responsive to chemotherapy
Dermatofibrosarcoma Protuberans
Trunk skin
Adolescents
Rarely spreads to distant sites
Has a high recurrence rate due to tumour extensions; wide surgical removal is advised
Resistant to chemotherapy
Malignant Peripheral Nerve Sheath Tumour
Limbs (in patients with a history of neurofibromatosis 1 in prior neurofibroma)
Involves chromosome 17q deletion
Does not respond well to chemotherapy
Synovial Cell Sarcoma
Lower limbs
Children over ten years
Sensitive to chemotherapy
For all, use the same investigation and management principles as Rhabdomyosarcoma (RMS)
Use the Intergroup Rhabdomyosarcoma Studies (IRS) table for post surgical staging (see RMS page)
For chemo/radiotherapy - use European Paediatric Soft tissue sarcoma study group guidelines for non-RMS sarcomas 2005:
Summary:
Surgery only for group 1
Add Ifosfamide + Doxorubicin for rest
Radiotherapy for some adult-type sarcomas
Bone sarcomas
Osteosarcoma
Most common 2nd decade of life
2-3 per million/year
M>F
90% metaphyseal (usually knee)
Ewings sarcoma
3 per million/y
M<F
Axial skeleton
Ewing's more common in <10y
Askin tumour is a type of Ewing sarcoma - usually of the chest wall
Associations
Familial retinoblastoma
Li Fraumeni
Growth spurt
Bone dysplasia
Classic history is that of a lump that is painful when playing sport
Investigations
ALP may be high
Biopsy at tertiary centre as biopsy track will need to be within resection specimen
CXR + CT Chest
Bone scan
Radiographic features
Feature | Osteosarcoma | Ewing's Sarcoma |
Location | Metaphysis of long bones (e.g., distal femur, proximal tibia) | Diaphysis of long bones, pelvis, ribs |
Bone Destruction | Mixed lytic and sclerotic lesions | Predominantly lytic lesions |
Periosteal Reaction | Codman triangle, Sunburst pattern | Onion skinning (lamellated), Hair-on-end appearance |
Soft Tissue Mass | Often present, with ossification | Often present, less likely to show ossification |
Margins | Poorly defined | Poorly defined |
Management
Osteosarcoma not radiosensitive, Ewing's is
Amputation
Endoprosthesis (can be lengthened magnetically)/Graft
Rotationplasty
National MDT decision on metastectomy - select cases only
Metastases can ossify and not cause problem
Outcome
OS 65% 5 year survival
Ewing's 60% 5 year survival
References
Ferrari A, Brennan B, Casanova M, Corradini N, Berlanga P, Schoot RA, Ramirez-Villar GL, Safwat A, Guillen Burrieza G, Dall'Igna P, Alaggio R, Lyngsie Hjalgrim L, Gatz SA, Orbach D, van Noesel MM. Pediatric Non-Rhabdomyosarcoma Soft Tissue Sarcomas: Standard of Care and Treatment Recommendations from the European Paediatric Soft Tissue Sarcoma Study Group (EpSSG). Cancer Manag Res. 2022 Sep 23;14:2885-2902. doi: 10.2147/CMAR.S368381. PMID: 36176694; PMCID: PMC9514781.
Gaillard F, Campos A, Murphy A, et al. Osteosarcoma. Reference article, Radiopaedia.org (Accessed on 20 Jul 2024) https://doi.org/10.53347/rID-1170
Gaillard F, Campos A, Sharma R, et al. Ewing sarcoma. Reference article, Radiopaedia.org (Accessed on 20 Jul 2024) https://doi.org/10.53347/rID-7852