Primary Pulmonary Sarcoma

Epidemiology

  • Very rare

Physiology

  • Primary malignant lung neoplasm arising from any mesenchymal structure in the lung
  • Diagnosis of primary lung sarcoma requires exclusion of sarcoma elsewhere (exclude osteosarcoma with bone scan, exclude otehr types with CT scans)

Diagnosis

  • FOB: TBB usually diagnostic
    -Special stains/ EM may be useful

CXR/Chest CT Pattern:
1) Solitary lung mass
2) Pleural effusion
3) Airway lesion


Clinical

  • See also Obstructive Lung Disease
    Symptoms/ signs:
    -Obstructive symptoms/ signs: when present in or near airway
    -Fever: uncommon

Complications: PE (associated with pulmonary vascular sarcomas)/ pulm-onary osteoarthropathy (uncommon)/ hypoglycemia (uncommon)


Treatment

Small, well-differentiated type: surgery may be curative

Poorly-differen-tiated type: less curative

Unresectable/recurrent sarcoma: chemo/ XRT are used but survival is usually <1 year


Prognosis

  • Prognosis: survival varies with histologic grade (usually 5-25%)