Primary pulmonary artery leiomyosarcoma

Primary pulmonary artery leiomyosarcoma is a form of thoracic sarcoma and an extremely rare tumour of the thorax.

The reported incidence rate is very low at around  ~0.001-0.03%. Most patients are around 40 and 60 years at time of diagnosis. 

They are a smooth muscle tumour and are thought to arise from pleuripotent cells found in the intima of the vessel. They may account for around 20% of all primary pulmonary artery sarcomas 7.

Symptoms are often non-specific and include cough, exertional dyspnoea, chest pain, right ventricular failure and therefore can be misinterpreted as being related to pulmonary embolism. Diagnosis can often be delayed.

CT

May be seen as a unilateral mass continuously filling the pulmonary artery with inhomogenous, heterogenous enhancement. Features of vascular distension, extravascular invasion into adjacent structures may be present.

MRI

Useful for assessing presence of enhancement.

  • T1 C+ (Gd): shows enhancement or areas of enhancement 13
FDG-PET

May show uptake in the area of tumour 8.

It is an extremely malignant tumour with median  median survival times of all patients according to one publication of around 1.5 months with possible prolonged to 10 months with surgery such as pneumonectomy +/- excision of the tumor from the vascular bed. Early detection and surgical resection improves patient survival, the benefits of adjuvant therapy are not well understood. Systemic metastases have been reported in around 20% cases at diagnosis and sites include the liver, pancreas, mesentery, brain, adrenals, kidneys, thyroid and the skin 9. Frequency of metastatic disease is not thought to be related to original tumour size,

Consider

  • large occlusive pulmonary embolus 2,12
  • it may very rarely mimic disseminated tuberculosis 9
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Article Information

rID: 53121
System: Chest
Synonyms or Alternate Spellings:
  • Pulmonary arterial leiomyosarcoma
  • Pulmonary artery leiomyosarcoma
  • Pulmonary artery leiomyosarcomas
  • Primary leiomyosarcoma of the pulmonary artery
  • Primary leiomyosarcoma of pulmonary artery
  • Primary leiomyosarcomas of pulmonary artery
  • Primary leiomyosarcoma of pulmonary arteries
  • Leiomyosarcomas of the pulmonary artery

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