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.
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.
Useful for assessing presence of enhancement.
- T1 C+ (Gd): shows enhancement or areas of enhancement 13
May show uptake in the area of tumour 8.
Treatment and prognosis
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,
- primary pulmonary angiosarcoma
- large occlusive pulmonary embolus 2,12
- it may very rarely mimic disseminated tuberculosis 9
- 1. Croitoru AG, Klein MJ, Galla JD, Fallon JT. Primary pulmonary artery leiomyosarcoma. Cardiovascular pathology : the official journal of the Society for Cardiovascular Pathology. 12 (3): 166-9. Pubmed
- 2. Lv Y, Wang F, Qian W, Sun G. Treatment of a recurrent pulmonary artery leiomyosarcoma with a combination of surgery, chemotherapy, and radiotherapy: A case report and literature review. Oncology letters. 9 (4): 1545-1548. doi:10.3892/ol.2015.2957 - Pubmed
- 3. Chan SH, Tsai LM, Tsai WC, Li YH, Chen JH, Luo CY. Pulmonary artery leiomyosarcoma. Journal of the Formosan Medical Association = Taiwan yi zhi. 98 (8): 578-81. Pubmed
- 4. Coli A, Parente P, Bigotti G. Pulmonary artery sarcoma: an insidious tumor still diagnosed too late. Analysis of the literature and report of a case. Journal of experimental & clinical cancer research : CR. 26 (1): 151-6. Pubmed
- 5. Hoffmeier A, Semik M, Fallenberg EM, Scheld HH. Leiomyosarcoma of the pulmonary artery--a diagnostic chameleon. European journal of cardio-thoracic surgery : official journal of the European Association for Cardio-thoracic Surgery. 20 (5): 1049-51. Pubmed
- 6. Schlecht L, Bittner RC, Schnoy N, Loddenkemper R, Felix R. [Leiomyosarcoma of the pulmonary artery]. Aktuelle Radiologie. 6 (2): 105-7. Pubmed
- 7. Colak N, Nazli Y, Alpay MF, Haltas H, Cakir O. Surgical treatment of pulmonary artery leiomyosarcoma: a good survival without adjuvant therapy. The Annals of thoracic surgery. 92 (6): 2252-4. doi:10.1016/j.athoracsur.2011.05.041 - Pubmed
- 8. Kim JH, Gutierrez FR, Lee EY, Semenkovich J, Bae KT, Ylagan LR. Primary leiomyosarcoma of the pulmonary artery: a diagnostic dilemma. Clinical imaging. 27 (3): 206-11. Pubmed
- 9. Respiratory Medicine CME Volume 3, Issue 1, 2010, Pages 44–47 Case Report Leiomyosarcoma of the pulmonary artery masquerading as disseminated tuberculosis Angela Atalla,- https://doi.org/10.1016/j.rmedc.2009.01.013
- 10 .Lee S, Park IK, Cho SH, Kim DK. Images in cardiovascular medicine. Leiomyosarcoma involving main and left pulmonary artery treated surgically with homograft replacement and concomitant left pneumonectomy. Circulation. 116 (24): e559-61. doi:10.1161/CIRCULATIONAHA.107.698092 - Pubmed
- 11. Modi K, Dhillon S, Kumar A, Ylagan L, Harris K. Leiomyosarcoma of the pulmonary artery diagnosed by endobronchial ultrasound-guided transbronchial needle aspiration. Endoscopic ultrasound. 3 (4): 249-51. doi:10.4103/2303-9027.144547 - Pubmed
- 12. Minakata K, Konishi Y, Matsumoto M, Aota M, Nonaka M, Yamada N. Primary leiomyosarcoma of the pulmonary artery mimicking massive pulmonary thromboembolism. Japanese circulation journal. 64 (10): 783-4. Pubmed
- 13. Gladish GW, Sabloff BM, Munden RF, Truong MT, Erasmus JJ, Chasen MH. Primary thoracic sarcomas. Radiographics : a review publication of the Radiological Society of North America, Inc. 22 (3): 621-37. doi:10.1148/radiographics.22.3.g02ma17621 - Pubmed