TABLE 1

Case series of pulmonary artery sarcoma published in the past 5 years

First author [ref.]PatientsAge at diagnosis yearsClinical presentationHistological typeTreatmentMedian survival timePatients initially diagnosis of PE#
Srivali [1]9 (4 M/5 F)55 (24–74)Dyspnoea: 9 (100)
Cough: 2 (22)
Syncope/presyncope: 2 (22)
Haemoptysis: 1 (11)
  • High-grade spindle cell sarcoma=2

  • Intermediate-grade spindle-cell sarcoma=1

  • High-grade undifferentiated pleomorphic spindle-cell sarcoma=1

  • Myxofibrosarcoma=1

  • Angiosarcoma=1

  • Intermediate-grade myofibroblastic sarcoma=1

  • Intimal sarcoma=2

SR=1
SR+RT=3
SR+CT=3
SR+RT+CT=2
None=1
23.6 months7
Yin [4]12 (5 M/7 F)51.2 (21–73)Chest pain: 7 (83)
Asthenia: 7 (58)
Cough: 6 (50)
Syncope: 3 (25)
Haemoptysis: 2 (16)
Headache: 2 (16)
Hoarseness: 1 (8)
  • Undifferentiated pleomorphic sarcoma=3

  • Myofibroblastic differentiation=6

  • Leiomyosarcoma=1

  • Synovial sarcoma=1

  • Rhabdomyosarcoma=1

  • PEA=7

  • PEA+pneumonectomy=3

  • Exploratory thoracotomy=2

  • CT+RT=4

  • CT=2

  • RT: 1

None=5
18 months6
Pu [5]9 (4 M/5 F)46.1±10.9Dyspnoea: 8 (89)
Syncope: 3 (33)
Palpitations: 2 (22)
Chest pain: 2 (22.2)
Haemoptysis: 1 (11)
Fever: 4 (44)
Fatigue: 5 (56)
Weight loss: 5 (56)
  • Intimal sarcoma=4

  • Leiomyosarcoma=3

  • Pleomorphic rhabdomyosarcoma=1

  • Undifferentiated sarcoma=1

PEA=5
PEA+RT=2
PEA+CT=1
PEA+RT+ CT=1
8.4 months7
Deng [6]13 (6 M/7 F)45 (26 –79)Dyspnoea: 13 (100)
Syncope: 6 (46)
Chest pain: 5 (38)
Haemoptysis: 4 (31)
Fever: 3 (23)
  • Intimal sarcoma=9

  • steosarcoma=1

  • Undifferentiated pleomorphic sarcoma=1

  • Atypical borderline cartilage tumour/sarcoma=1

  • Malignant solitary fibrous tumour=1

SR=5
SR+CT=7
SR+RT=1
20.2 months8
Lee [7]20 (10 M/10 F)54 (33–75)Dyspnoea: 13 (65)
Chest pain: 5 (25)
Cough: 4 (20)
Haemoptysis: 3 (15)
Syncope: 2 (10)
Asymptomatic: 2 (10)
  • Spindle cell sarcoma=8

  • Intimal sarcoma=4

  • Pleomorphic sarcoma=3

  • High grade sarcoma=2

  • Chondroid differentiation=1

  • Angiosarcoma=1

  • Smooth muscle differentiation=1

SR=6
SR+QT=3
SR+RT=5
SR+RT+ QT=3
None=3
24 months10
Wong [8]20 (11 M/9 F)57 (27–77)Dyspnoea: 20 (100)
Chest pain/tightness: 7 (35)
Dependent oedema: 5 (25)
Constitutional symptoms: 5 (25)
Cough: 3 (15)
Haemoptysis: 3 (15)
  • Intimal sarcoma=13

  • High-grade sarcoma=6

  • No biopsy=1

  • PEA=14

    • PEA+CT=5

    • PEA+RT=4

  • CT=5

  • RT=5

  • Radiofrequency ablation=1

17 months
Bandyopadhyay [2]10 (8 M/2 F)47±11.3
  • Leiomyosarcoma=1

  • Spindle cell sarcoma=2

  • Angiosarcoma=3

  • Myofibroblastic sarcoma=1

  • Osteosarcoma=2

  • Rhabdomyosarcoma=1

21.6 months

Data are presented as n, median (interquartile range), mean±sd, or n (%). PE: pulmonary embolism; M: male; F: female; SR: surgical resection; RT: radiotherapy; CT: chemotherapy; PEA: pulmonary endarterectomy. #: patients with pulmonary artery sarcoma initially misdiagnosed as PE.