Bone and soft-tissue sarcomas

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Target exams

Red flags

• Persistent deep non-mechanical limb pain in a child or adolescent, especially night pain that wakes the child and is unrelated to activity, is a bone sarcoma until imaging proves otherwise
• A palpable mass in the limb, the head and neck or the pelvis of a child, particularly a firm deep mass greater than five centimetres, is a sarcoma until biopsy proves otherwise
• A permeative diaphyseal bone lesion with an onion-skin or sunburst periosteal reaction and a large soft-tissue mass is Ewing sarcoma until the fusion and the histology settle it
• Never perform an excisional or unplanned incisional biopsy of a suspected sarcoma: the biopsy tract is contaminated and must be excised with the tumour, and a poorly placed biopsy compromises or prevents limb-salvage surgery
• A child with a parameningeal mass and cranial nerve palsies, or a pelvic mass with cord compression, has spinal cord or neural compromise and needs emergency imaging and steroids
• Bulky chemosensitive sarcoma at the start of treatment places the child at risk of tumour lysis syndrome, so hydration and urate-lowering prophylaxis begin before the first dose

Life stages

Care settings

Clinical exam formats

Board mappings

• Recognises that persistent non-mechanical limb pain and a palpable mass in a child or adolescent is a sarcoma until imaging and biopsy prove otherwise
• Names the three commonest paediatric sarcomas: osteosarcoma, Ewing sarcoma and rhabdomyosarcoma
• States that a suspected sarcoma is referred to the specialist sarcoma service before any biopsy, because a poorly placed biopsy compromises limb-salvage surgery
• Explains how the defining molecular alterations, the EWSR1-FLI1 fusion of Ewing sarcoma and the PAX-FOXO1 fusion of alveolar rhabdomyosarcoma, drive the diagnosis and the risk stratification
• Coordinates the staging pathway from magnetic resonance imaging of the compartment through computed tomography of the chest, isotope bone scan or positron-emission tomography, and bone-marrow sampling, to the multidisciplinary treatment plan
• Synthesises the risk-adapted chemotherapy backbones with surgery and radiotherapy into a survivorship plan that addresses cardiotoxicity, second malignancy and late effects
• Persistent deep limb pain, night pain unrelated to activity, and a palpable mass in a child is a sarcoma until imaging proves otherwise
• A suspected bone sarcoma shows a lytic or sclerotic lesion with a sunburst, Codman triangle or onion-skin periosteal reaction, and magnetic resonance imaging of the whole compartment is the local staging test
• The chemotherapy backbones are methotrexate-doxorubicin-cisplatin for osteosarcoma, vincristine-doxorubicin-cyclophosphamide alternating with ifosfamide-etoposide for Ewing sarcoma, and vincristine-actinomycin-cyclophosphamide for rhabdomyosarcoma
• Counsels an adolescent newly diagnosed with an osteosarcoma of the distal femur on limb-salvage surgery versus amputation, the chemotherapy, the fertility implications and the prognosis
• Builds the multidisciplinary plan that runs paediatric oncology, orthopaedic oncology, radiation oncology, pathology, radiology and the family together for a child with Ewing sarcoma of the pelvis
• Synthesises the long-case child cured of a sarcoma into a survivorship plan covering cardiotoxicity, second malignancy, musculoskeletal late effects and vocational reintegration
• Level 1: Recognition of the red flag features of a bone or soft-tissue sarcoma in a child and the urgent referral for imaging and specialist review
• Level 2: Application of the diagnostic and staging pathway and the safe biopsy principle through the multidisciplinary team
• Level 3: Coordination of risk-adapted definitive therapy and long-term survivorship surveillance across oncology, surgery and late-effects services
• The commonest primary malignant bone tumours of childhood and adolescence: osteosarcoma and Ewing sarcoma, their sites and their radiographic patterns
• The commonest soft-tissue sarcoma of childhood: rhabdomyosarcoma, its embryonal and alveolar subtypes, its sites and its fusion markers
• The principles of multimodal therapy with neoadjuvant chemotherapy, surgery and radiotherapy, and the survivorship burden
• Structured examination of a child with a suspected bone or soft-tissue mass, and the recognition of the red flags that mandate urgent imaging
• Communication of a new sarcoma diagnosis to a child and family, addressing the surgery, the chemotherapy, the fertility implications and the prognosis
• Recognition of the oncologic emergencies of spinal cord compression and tumour lysis syndrome in the child with a sarcoma
• Bone and soft-tissue sarcomas as a leading group of solid tumours of childhood and adolescence
• The clinical presentation by tumour type: limb pain and mass for osteosarcoma, diaphyseal pain and systemic symptoms for Ewing sarcoma, and a head-and-neck or genitourinary mass for rhabdomyosarcoma
• The principles of multimodal therapy and the age-related and late-effects considerations
• Recognition of the red flag clinical features of a paediatric sarcoma that mandate urgent imaging and referral to the specialist service
• Application of the initial stabilisation of a child with a sarcoma including analgesia, the safe referral for biopsy and the management of cord compression and tumour lysis syndrome
• Coordination of the diagnostic, staging and multidisciplinary pathway for a child with a bone or soft-tissue sarcoma
• Canadian approach to the urgent evaluation and referral of a child with a suspected sarcoma
• The molecular and radiographic features that distinguish osteosarcoma, Ewing sarcoma and rhabdomyosarcoma
• The long-term late effects of sarcoma therapy and the surveillance of the paediatric sarcoma survivor