01OverviewDefinition, clinical context and the essential points that orientate the chapter.
Ask the patient to point to the pain and define duration, progression, night waking, rest pain, swelling and functional loss. Clarify whether trauma plausibly explains the trajectory. Osteosarcoma often affects the distal femur, proximal tibia or proximal humerus in adolescents and young adults; Ewing sarcoma may present in diaphysis, pelvis or chest wall with fever; chondrosarcoma more often affects adult pelvis, shoulder girdle or proximal long bone. Constitutional symptoms may be absent.
Inspect and measure any mass, noting skin, veins and relation to a joint. Palpate gently for warmth, firmness and tenderness without stressing a weakened bone. Assess the joint above and below, distal nerves and pulses, lymph nodes and chest symptoms. Record performance status and a baseline functional examination that will inform reconstruction. If a fracture is possible, immobilise before transfers and avoid repeated examination under load.
Obtain full two-plane radiographs that include the lesion and enough bone to understand its compartment. Describe site, matrix, transition, periosteum, cortex and soft tissue. Osteoid may look dense and cloudlike, cartilage mineralises in rings and arcs, and Ewing can be permeative with lamellated periosteal bone, but no single sign replaces integrated diagnosis. A radiograph reported normal should be reviewed or repeated if symptoms remain focal and progressive.
Contact the regional bone-sarcoma service as soon as imaging is suspicious. Do not arrange a convenient local biopsy, curettage or intramedullary device first. The centre coordinates MRI before tissue, usually covering the whole bone, both adjacent joints and the complete soft-tissue component. MRI shows marrow extent, skip lesions, neurovascular proximity and the best viable target. CT adds cortical and matrix detail in pelvis, spine or mineralised tumours.
Staging and biopsy are designed together. CT chest evaluates pulmonary metastases; whole-body bone scintigraphy, PET-CT or whole-body MRI is selected by histology and protocol to identify skeletal spread. Image-guided core biopsy usually provides diagnosis with less morbidity than open sampling. The needle travels through one compartment, avoids neurovascular bundles and joint contamination and ends within a planned resection field. Samples go for histology, molecular testing and microbiology when infection remains plausible.
If a pathological fracture occurs, use a splint or brace, analgesia and safe transfer while the tumour centre determines biopsy and treatment order. A nail passed down the medullary canal can disseminate tumour through the whole bone and convert a limb-sparing resection into a far larger procedure. Closed reduction may also contaminate tissues through haematoma. Selected confirmed sarcoma fractures can still undergo neoadjuvant treatment and limb salvage under specialist control.
Treatment depends on histology. High-grade osteosarcoma receives multi-agent chemotherapy and wide surgical excision; Ewing sarcoma uses systemic chemotherapy with surgery, radiotherapy or both for local control. Conventional chondrosarcoma is primarily treated by adequate surgical resection because standard chemotherapy and radiotherapy have limited effect, while molecular subtypes differ. Reconstruction may use endoprosthesis, allograft, autograft, rotationplasty or amputation according to margin, function and patient priorities.
Care continues for years. Early rehabilitation protects reconstruction while preserving strength and independence. Surveillance examines the primary site and lungs according to histology and time from treatment. Late care addresses prosthetic loosening or infection, fracture, cardiotoxicity, renal or hearing harm, fertility, second malignancy and psychological recovery. New pain or swelling after treatment is imaged promptly rather than waiting for the next routine surveillance visit.
Key points
- The common presenting symptom is progressive localised bone pain; night or rest pain, swelling, a palpable mass and symptoms worsening after an assumed sports injury are high-yield warnings.
- First-line investigation is urgent plain radiography of the entire symptomatic region in two planes, not repeated physiotherapy or a screening blood test.
- Aggressive imaging features include a wide transition zone, permeative destruction, cortical breach, interrupted periosteal reaction and a soft-tissue mass.
- Normal inflammatory markers and normal ALP do not exclude sarcoma; blood tests assess physiology and differentials but cannot clear an aggressive lesion.
- MRI of the whole involved bone and compartment is the reference local-staging study and should be completed before biopsy whenever clinically feasible.
- Never biopsy a suspected bone sarcoma outside the specialist pathway: the multidisciplinary team selects a representative target and a tract removable during definitive resection.
- A pathological fracture is splinted and protected without definitive fixation until tumour type and oncological plan are established.
- Staging commonly includes CT chest and whole-body skeletal assessment; treatment then combines tumour-specific chemotherapy, wide resection and selected radiotherapy through a specialist sarcoma service.
02AetiologyUnderlying causes, associations and risk factors, with why each one matters.
Growth and age pattern
Osteosarcoma clusters around adolescent growth and long-bone metaphyses, Ewing sarcoma affects younger people and axial or diaphyseal sites, while chondrosarcoma is usually adult.
Inherited cancer predisposition
Li-Fraumeni syndrome, hereditary retinoblastoma and selected DNA-repair disorders increase osteosarcoma risk and influence wider genetic and surveillance decisions.
Pre-existing bone disorder
Paget disease, multiple osteochondromas, enchondromatosis and other rare lesions can undergo secondary malignant transformation, usually signalled by changing adult symptoms or growth.
Radiation-associated sarcoma
A new aggressive tumour can arise years after therapeutic irradiation within the treated field, requiring histological distinction from recurrent primary cancer.
03PathophysiologyThe causal sequence from the underlying abnormality to symptoms and harm.
- 1Malignant matrix production
Osteosarcoma cells form malignant osteoid, chondrosarcoma produces tumour cartilage and Ewing sarcoma is a small-round-cell tumour driven by characteristic gene fusion.
- 2Infiltrative local growth
Tumour replaces marrow, breaches cortex and dissects through soft-tissue planes, creating pain, mass and a larger surgical compartment at risk.
- 3Periosteal response
Rapid cortical irritation outpaces orderly new bone, causing interrupted patterns such as lamellated, spiculated or Codman-triangle reaction rather than proving one histology.
- 4Haematogenous spread
Primary bone sarcoma commonly metastasises through blood to lungs and other bones, making chest and whole-body staging integral before definitive treatment.
- 5Biopsy-track contamination
Needle or incision carries tumour cells into traversed tissue, which must be removed later; a poorly placed track can sacrifice uninvolved structures or compartments.
04Clinical features and red flagsSymptoms, examination findings, patterns of presentation and time-critical warnings.
Persistent localised pain that intensifies, occurs at rest or wakes sleep is the central clinical warning regardless of age.
A deep fixed swelling arising from bone, particularly with venous prominence or joint restriction, requires urgent imaging.
An indistinct boundary between abnormal and normal bone indicates growth too rapid for a contained host response.
Lamellated, spiculated or elevated periosteum signals aggressive irritation but must be integrated with matrix and clinical context.
Destruction through cortex into a soft-tissue mass establishes locally aggressive behaviour and enlarges the oncological compartment.
Failure after minimal force through a solitary aggressive lesion may be the first manifestation and requires tumour-aware immobilisation.
05InvestigationsWhat to request, why it matters and how to interpret it.
Read from the initial assessment onwards. Tests may run in parallel in urgent care; first-line, preferred, confirmatory, definitive and gold-standard labels appear only when the chapter explicitly states them.
- 01
First-line two-plane plain radiographsFirst stepFirst line - Why
- Detect and describe matrix, margin, cortex, periosteal response, fracture and soft-tissue mineralisation at the symptomatic site.
- Interpretation and limitations
- Any aggressive or clinically discordant lesion warrants immediate specialist referral; a normal study does not end evaluation of persistent focal symptoms.
- 02
MRI of whole bone and compartment - Why
- Provide reference local staging, defining intramedullary length, soft-tissue extent, skip lesions, joint and neurovascular relationships.
- Interpretation and limitations
- Complete before biopsy when possible so procedure-related haemorrhage does not obscure true tumour boundaries.
- 03
CT chest for metastatic staging - Why
- Detect pulmonary nodules before curative treatment and provide a baseline for response and surveillance.
- Interpretation and limitations
- Indeterminate small nodules require sarcoma-MDT interpretation; absence of lung disease does not establish low-grade histology.
- 04
Whole-body skeletal staging - Why
- Identify other bone metastases using scintigraphy, PET-CT or whole-body MRI according to tumour protocol.
- Interpretation and limitations
- Choice depends on suspected histology and centre practice; correlate additional lesions before changing intent.
- 05
Specialist-planned core biopsy - Why
- Establish histology, grade and molecular diagnosis using viable representative tissue after imaging and staging design.
- Interpretation and limitations
- This is the diagnostic reference standard; concordance among imaging, pathology and clinical findings is required before treatment.
06Differential diagnosisRealistic alternatives and the features that help distinguish them.
Osteomyelitis
Fever, inflammatory response, abscess or sequestrum favours infection, but Ewing sarcoma can mimic it and tissue must undergo both pathology and microbiology.
Benign aggressive lesion
Aneurysmal bone cyst, giant-cell tumour and chondroblastoma may expand rapidly, yet still require specialist imaging and planned biopsy when morphology is indeterminate.
Metastasis or myeloma
Older age, multiple lesions, known carcinoma, anaemia or paraprotein suggests secondary malignancy but does not make an uncharacterised solitary lesion safe for local fixation.
Stress fracture
Linear cortical change, credible repetitive load and serial healing support stress injury; a mass, wide transition or progressive destruction opposes it.
Lymphoma of bone
Permeative marrow disease with disproportionate soft tissue can resemble Ewing or metastasis and requires representative core biopsy and haematopathology.
07ManagementImmediate care, first-line treatment, alternatives and escalation.
01RecognitionImage persistent focal warning painFirst stepBone pain is unexplained, progressive, nocturnal or associated with swelling or a mass.+
- 1Re-examine the precise site and obtain urgent radiographs in two planes rather than extending empirical therapy.
- 2Review the images for margin, matrix, cortex, periosteum and soft tissue and compare all prior studies.
- 3EscalationEscalate suspicious imaging or continuing clinical-radiological discordance to the regional sarcoma pathway.
- 4Advise the patient to avoid impact and return immediately for sudden pain or loss of weight bearing.
02Specialist stagingDefine extent before tissueRadiography shows an aggressive or indeterminate primary bone lesion.+
- 1Send the clinical history and full imaging to the designated bone-sarcoma service without local intervention.
- 2Obtain specialist-protocol MRI of the whole bone and compartment before biopsy whenever possible.
- 3Complete CT chest and selected whole-body skeletal staging under the multidisciplinary plan.
- 4Let radiology, pathology and surgeon choose the viable target and resectable biopsy tract together.
03FracturePrevent contamination after failureA suspected sarcoma has fractured or is at imminent risk of doing so.+
- 1Immobilise in a comfortable position, protect weight bearing and document distal neurovascular status.
- 2Provide analgesia, VTE assessment and safe transfer without forceful reduction or intralesional injection.
- 3Contact the tumour centre before internal fixation or biopsy and transfer images immediately.
- 4Follow the histology-specific sequence of systemic treatment, resection and reconstruction decided by the sarcoma MDT.
04Definitive careCombine oncological control and functionDefinitiveSpecialist biopsy confirms a primary bone sarcoma and staging is complete.+
- 1Use histology-specific chemotherapy for osteosarcoma or Ewing sarcoma according to national specialist protocols.
- 2Plan wide resection with reconstruction that achieves an oncological margin while preserving useful function where possible.
- 3Use radiotherapy for Ewing and selected unresectable, close-margin or palliation indications according to tumour biology.
- 4Begin rehabilitation and surveillance early and monitor treatment-specific organ and reconstruction complications.
Key medicines and prescribing safety2 treatments · regimens, roles and cautions+
Paracetamol before definitive tumour treatment
Give 500 to 1,000 mg orally as required at intervals of at least four hours, not exceeding 4 g daily and using a lower maximum in low body weight, malnutrition or hepatic risk.Check combination analgesics and liver or alcohol risk; improved pain must not permit unprotected loading through a weakened lesion.
Morphine for severe pathological bone pain
In an opioid-naive adult with severe pain, titrate small immediate-release oral doses under local acute-pain guidance, reducing the starting dose in frailty or renal impairment and prescribing antiemetic and laxative support.Monitor sedation, respiratory rate, delirium, nausea and constipation; choose an alternative or specialist regimen in advanced renal failure and reassess escalating pain for fracture or compartment compromise.
08ComplicationsImportant consequences, why they occur and why they matter clinically.
Pathological fracture
Structural failure causes pain and bleeding and may spread tumour locally, but limb salvage can remain possible with immediate specialist handling.
Pulmonary metastasis
Lung spread may be asymptomatic at presentation and strongly affects treatment intensity, resection planning and prognosis.
Neurovascular involvement
Tumour encasement or displacement of major nerves and vessels changes resectability, reconstruction and expected postoperative function.
Treatment toxicity
Chemotherapy can cause infection, cardiotoxicity, renal injury, neuropathy and infertility, while radiotherapy and surgery create site-specific late effects.
Local recurrence
Inadequate margin, resistant biology or contaminated planes permit recurrence, which can require more extensive resection or amputation and worsens prognosis.
09Monitoring and follow-upTreatment response, safety checks and longer-term review.
- While awaiting specialist review, reassess pain, swelling, skin, distal neurovascular findings and ability to transfer without loading.
- Track image transfer and named sarcoma-centre acceptance rather than relying on a generic urgent referral entry.
- After biopsy, inspect bleeding, wound and neurovascular status and avoid crossing the tract with drains or later incisions.
- During systemic treatment, monitor blood count, infection, renal, cardiac, neurological and fertility effects according to regimen.
- After reconstruction, follow wound, infection, union or endoprosthetic fixation, range, strength and agreed loading progression.
- During surveillance, investigate new local pain, mass, respiratory symptoms or unexplained decline without waiting for a scheduled scan.
10Special situationsVariants, exceptions and circumstances that change the usual approach.
Sarcoma pain may follow sport
Activity can draw attention to an existing tumour, so a minor incident does not explain a worsening nocturnal trajectory.
Blood tests cannot clear bone
ALP and inflammatory markers may be normal, leaving radiographic and clinical discordance as the important signal.
MRI precedes the needle
Local staging identifies viable tissue and true compartments before biopsy haemorrhage and inflammation distort anatomy.
A nail contaminates length
Intramedullary fixation can transport tumour along the canal and transform a focal resection into whole-bone oncological disease.
Histology changes systemic therapy
Osteosarcoma, Ewing sarcoma and chondrosarcoma differ fundamentally, making empiric treatment before specialist tissue diagnosis unsafe.
Fracture need not end salvage
Specialist immobilisation and planned multimodal care can preserve limb-sparing options despite tumour-related structural failure.
11Common pitfallsFrequent interpretation and management errors.
- 01
Repeating physiotherapy or analgesia for progressive night bone pain without obtaining radiographs.
- 02
Reassuring from normal ALP or CRP when symptoms and imaging remain suspicious.
- 03
Requesting a local biopsy before whole-lesion MRI and specialist tract planning.
- 04
Passing an intramedullary nail through an uncharacterised solitary pathological fracture.
- 05
Assuming infection from fever and permeative bone without sending planned tissue for pathology as well as microbiology.
- 06
Waiting for constitutional symptoms even though many patients with localised sarcoma remain otherwise well.