Difficult to characterise radiographically - histology required
Management surgical resection with negative margins, theoretically 2cm margins is ideal
Liposarcoma
Three subtypes
well-differentiated liposarcoma
Most common
Aka atypical lipomatous tumour when seen in extremities
No metastatic potential, but can differentiate into dedifferentiated liposarcoma (high grade disease)
myxoid round cell liposarcoma
Mid grade tumour
pleomorphic liposarcoma
High grade disease
No consensus on whether CT/MRI is best
Surgical resection best chance for cure - standard of care 2cm margin, but if impossible, even resection with limited margins has a survival benefit
Role of RTX varies depending no subtype
CTX explorative
Neoadjuvant CTX is probably not useful if resection is planned
Close follow up critical
Langerhans cell histiocytosis
Rare tumour derived from uncontrolled proliferation and subsequent migration of Langerhans cells. Unclear aetiology.
Can be multi focal sometimes, especially affecting ribs
Chest CT quite good for diagnosis
Biopsy needed for diagnosis - round cells with 'coffee bean' nucleus
Mainstay of treatment is CTX, but surgery is occasionally required for diagnosis or impingement on other structures
Primary pleuropulmonary synovial sarcoma
Synovial sarcoma that affects chest wall, pleural, lung, heart or mediastinum. Subtype of spindle cell sarcoma. Vast majority occur as a result of translocation between chromosomes 18 and X
Most common presentation is obstructive symptoms, cough, dyspnoea, haemoptysis or chest pain
CT good for diagnosis, MRI sometimes helps define invasion/spread
Complete resection is ideal, with negative margins
Most common benign lesion in the rib - progressive replacement of calcified bone and marrow with fibrous tissue due to abnormal proliferation of BM stromal cells
Usually either incidental finding or bone pain at site
CT is best for diagnosis, bone scans can be helpful
Biopsy can be performed if diagnostic uncertainty
Management guided by symptoms - can be resected if necessary
Osteochondroma
Osseous growth with cartilaginous cap on the affected bone
Can present with pathological fracture or chronic pain
CT good for diagnosis
Malignant transformation rare
Indications for resection: chronic pain, impingement, cosmesis, fast growing tumour
Entire cartilaginous cap should be removed to prevent recurrence
If more than one osteochondroma, hereditary cause suspected
Chondroma
Cartilaginous cyst that can arise completely within or on the surface of bone
Hyaline cartilage producing tumours
Usually incidental
MRI more sensitive than CT for detecting malignant degeneration
Resection reserved for pathologic fractures or a concern for secondary chondrosarcoma, although can do early resection if it's easily accessible
Aneurysmal bone cyst
Not really a cyst, more of a benign skeletal tumour
Commonly presents with pain or pulmonary symptoms from compression
Can grow >10cm in size
CT or MRI are good for diagnosis
Biopsy usually inconclusive and just results in blood products
Curative treatment is resection - no huge urgency but can cause secondary symptoms
Malignant
Osteosarcoma
Most commonly a tumour of long bones in children and adolescents - uncommon in chest wall
Classically presents with pain severe enough to wake from sleep
CT diagnostic
WLE mainstay of treatment - can remove ribs/most of sternum with immediate reconstruction
Neoadjuvant CTX is effective
Chondrosarcoma
Most common primary malignancy of sternum
Commonly causes pain and swelling
CT is best diagnosis, with MRI reserved for determining pattern of invasion
Histology is usually distinctive enough to be diagnostic
Complete resection desired for initially therapy with at least 4mm margins
Treatment of disseminated disease is essentially palliative
