Editing Chest wall tumours (section)

== Soft tissue ==

* Benign
** Lipoma
*** Most common soft tissue tumour in adults, especially obese patients
*** Fatty attenuation on imaging, with mild enhancement of their septations
*** Deeper lesions are generally less well circumscribed than more superficial ones - may require pre-op tagging
*** Needs complete removal of capsule to prevent recurrence
** Fibromatosis colli
** Leiomyoma
** Desmoid tumour
*** Soft tissue tumours developing from deep tendons and connective tissue
*** Officially benign without metastatic potential, but clinically behave like low-grade sarcomas with high rates of local recurrence
*** MRI is best at diagnosing and identifying
*** Treatment is usually resection - without it, they continue to grow
*** Can infiltrate other structures
*** If positive margins, re-resection should be undertaken if feasible
*** May be a role for systemic therapy
*** Should have extended follow-up
* Malignant
** Rhabdomyosarcoma
*** Most common paediatric soft tissue tumour, but can also present in adults
*** Usually rapid growing
*** Originates in skeletal muscle but can quickly invade bone
*** MRI>CT for specific diagnosis
*** Resection represents best chance for cure - immediate reconstruction may be required if breathing mechanics are compromised
*** RTX and CTX routinely used
** Malignant fibrosis histiocytoma/undifferentiated pleomorphic sarcoma
*** Sarcoma of uncertain aetiology
*** Can arise from either soft tissue or bone
*** Extremities, retroperitoneum, or chest wall
*** 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
*** CTX generally given