Irish Journal of Medical Science

, Volume 180, Issue 1, pp 275–278 | Cite as

Pulmonary artery leiomyosarcoma: an unusual cause of shortness of breath

Case Report
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Abstract

Introduction

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.

Results

On microscopic examination, the tumour was composed of spindle cells with cigar-shaped nuclei immunoreactive for vimentin, desmin, CD68 and α-smooth muscle actin.

Conclusions

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.

Keywords

Leiomyosarcoma Tumours of the pulmonary artery Shortness of breath Renal metastasis 

Notes

Acknowledgments

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

None.

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Copyright information

© Royal Academy of Medicine in Ireland 2009

Authors and Affiliations

  1. 1.Department of MedicineUniversity Hospital, Galway and National University of IrelandGalwayIreland
  2. 2.Department of RadiologyUniversity Hospital, Galway and National University of IrelandGalwayIreland
  3. 3.Department of HistopathologyUniversity Hospital, Galway and National University of IrelandGalwayIreland

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