Korean J Pathol.  2011 Apr;45(2):132-138. 10.4132/KoreanJPathol.2011.45.2.132.

Pulmonary Vascular Sarcomas: Clinicopathologic Analysis of 14 Cases

• Kim NR
• Kim J
• Ha SY
• Han J

• KMID: 1443745
• DOI: http://doi.org/10.4132/KoreanJPathol.2011.45.2.132

Abstract

BACKGROUND
Pulmonary vessel sarcomas are rare, and their pathogenesis is still unclear.
METHODS
We focus on the pathologic findings of fourteen pulmonary artery and/or vein sarcomas along with clinical prognosis.
RESULTS
Nine patients were male and five were female, and they ranged in age from 26 to 72 years (mean, 47 years). There were ten cases of pulmonary artery sarcoma, three cases of pulmonary artery and vein sarcoma, and one case of pure pulmonary vein sarcoma. Ten out of the fourteen cases were associated with pulmonary thromboembolism. Microscopically, all the tumors showed an undifferentiated sarcomatous portion. There were leiomyosarcoma portions in 8 cases, malignant fibrous histiocytomatous portions in 7 cases, angiosarcomatous differentiation in 3 cases, and osteosarcomatous portion in 1 case. All but two patients died during the follow up period (range, 1 to 78 months). The mean survival time of the patients who died was 14 months and the longest survival time was 78 months after surgical resection.
CONCLUSIONS
The current study is one of the largest single institutional reviews of pulmonary artery and/or vein sarcoma. Regardless of the histological components and macroscopic growth patterns, these rare tumors have a grave prognosis.

Keyword

Sarcoma; Pulmonary artery; Pulmonary veins

MeSH Terms

Female
Follow-Up Studies
Glycosaminoglycans
Humans
Leiomyosarcoma
Male
Prognosis
Pulmonary Artery
Pulmonary Embolism
Pulmonary Veins
Sarcoma
Survival Rate
Veins
Glycosaminoglycans