Successful Resection of a Re-Occurred Pulmonary Myosarcoma in a Patient with Turner Syndrome Mosaic

Autor: Volker F. H. Brauer, Frank Reichenberger, Anke Müller, Matthias Steinert, Ursula G. Froster, Hubert R. W. Wirtz, Joachim Schauer
Jazyk: angličtina
Rok vydání: 2002
Předmět:
Zdroj: Sarcoma, Vol 6, Iss 4, Pp 141-143 (2002)
Druh dokumentu: article
ISSN: 1357-714X
1369-1643
DOI: 10.1080/1357714021000066395
Popis: We describe a patient who underwent thoracic radiation therapy for biopsy-proven pulmonary spindle cell sarcoma in the left lower lobe, 15 months after birth. At the age of 37 she developed shoulder pain, fatigue, and progressive exertion dyspnoea. Chest X-ray revealed a pulmonary mass in the left lower lobe due to a cytology-proven malignant tumour.The patient underwent left pneumonectomy. Histology revealed a myosarcoma of the lung, similar to the previous sarcoma. Furthermore, the patient was diagnosed to have Turner syndrome mosaic and chromosomal analysis revealed a translocation t(1;13) in 3/50 metaphases. However a germline mutation of the p53 tumour suppressor gene was excluded. After 2 years of follow-up the patient is stable and there are no signs of recurrence of the tumour.We conclude a re-occurrence of this very rare malignant disorder of the lung after a 36-year interval in a patient with Turner syndrome mosaic. Following initial curative radiation therapy, with a remission over 36 years, lung resection was now successfully performed.
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