Pulmonary Artery Sarcoma



Pulmonary Artery Sarcoma











Pulmonary artery sarcoma shows a high-grade sarcoma image in close association with the wall of the pulmonary artery image. Identification of the pulmonary vessel is essential for the diagnosis.






High-grade sarcoma shows extensive areas of pleomorphic cells and atypical mitotic figures image. In many cases, the type of sarcoma is unclassified.


TERMINOLOGY


Abbreviations



  • Pulmonary artery sarcoma (PAS)


Synonyms



  • Intimal sarcoma


Definitions



  • Malignant mesenchymal tumor originating from wall of pulmonary trunk


ETIOLOGY/PATHOGENESIS


Etiology



  • Has been suggested to arises from pluripotential cells


  • Most probably arises from pulmonary trunk


CLINICAL ISSUES


Epidemiology



  • Incidence



    • Rare tumors that represent < 1% of all malignant mesenchymal tumors of lung


  • Age



    • Usually occurs in adults


  • Gender



    • No gender predilection has been observed in these tumors


Presentation



  • Cough


  • Chest pain


  • Shortness of breath


  • Fatigue


  • Syncope


  • Often confused for other medical conditions, such as pulmonary hypertension, pneumonia, or asthma


Treatment



  • Surgical approaches



    • Lobectomy or pneumonectomy


  • Adjuvant therapy



    • Chemotherapy &/or radiation may be of use depending on type of sarcoma


Prognosis



  • Intrathoracic metastasis may occur in 50% of patients


  • Extrathoracic metastasis may occur in about 15% of patients


  • Death may occur in a period of 12 months after initial diagnosis


MACROSCOPIC FEATURES


General Features



  • Tumor obliterating lumen of artery


  • Identification of pulmonary trunk


  • Identification of pulmonary artery


  • Polypoid tumor in intraluminal location


Sections to Be Submitted



  • Tangential sections to properly evaluate arterial wall

Jul 9, 2016 | Posted by in PATHOLOGY & LABORATORY MEDICINE | Comments Off on Pulmonary Artery Sarcoma

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