Pulmonary artery leiomyosarcoma: an unusual cause of shortness of breath
- 72 Downloads
Primary leiomyosarcomas of the pulmonary artery are very rare tumours with 30 cases reported in the English literature. Herein, we report the first case with evidence of a renal metastasis.
Materials and Methods
The tumour was discovered at autopsy in a 61-year-old woman who had presented with shortness of breath and chest pain. It was located in the pulmonary trunk with extension into the adjacent pericardial cavity. Separate nodules were found in the pulmonary valve, the left pulmonary artery and the left kidney.
On microscopic examination, the tumour was composed of spindle cells with cigar-shaped nuclei immunoreactive for vimentin, desmin, CD68 and α-smooth muscle actin.
Primary pulmonary artery leiomyosarcoma is easily confused clinically with pulmonary thromboembolism and most cases are diagnosed post mortem. Most tumours arise in the pulmonary trunk and metastases are only rarely described. The management of patients with this rare tumour depends on early surgical resection and the prognosis is unfavourable.
KeywordsLeiomyosarcoma Tumours of the pulmonary artery Shortness of breath Renal metastasis
We gratefully acknowledge the excellent technical assistance of Mrs. Frances Devlin and Mrs. Marian Morris of the Department of Histopathology, University College Hospital, Galway. We are grateful to Dr. John Lee, Consultant Physician, University College Hospital, Galway for providing clinical details and to Dr. Jeffrey L. Myers of the Department of Pathology, Mayo Clinic, Rochester, Minnesota, for reviewing our slides.
Conflict of interest statement
- 4.Hoffmeier A, Semik A, Fallenberg EM et al. (2001) Leiomyosarcoma of the pulmonary artery: a diagnostic chameleon. Eur J Cardiothorac Surg 1049–1051. doi: 10.1016/S1010-7940(01)00939-3
- 5.Burke AP, Virmani R (1993) Sarcoma of the great vessels: a clinicopathologic study. Cancer 71(5):1761–1773. doi: 10.1002/1097-0142(19930301)71:5<1761::AID-CNCR2820710510>3.0.CO;2-7 Google Scholar
- 14.Falexxa GC, Rodella S, Guilarte HN et al (1993) Intrapericardial leiomyosarcoma arising from the inferior vena cava: a case report. Radiol Med (Torino) 85(6):847–849Google Scholar
- 15.Shmookler BM, Marsh HB, Roberts WC (1977) Primary sarcoma of the pulmonary trunk and/or right or left main pulmonary artery–a rare cause of obstruction to right ventricular outflow. Report on two patients and analysis of the 35 previously reported cases. Am J Med 73:263–272. doi: 10.1016/0002-9343(77)90241-8 CrossRefGoogle Scholar