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

[摘要] We describe a patient who underwent thoracic radiation therapy for biopsy-proven pulmonary spindle cell sarcoma in theleft 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 patientunderwent 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 translocationt(1;13) in 3/50 metaphases. However a germline mutation of the p53 tumour suppressor gene was excluded. After 2years of follow-up the patient is stable and there are no signs of recurrence of the tumour.We conclude a re-occurrence ofthis very rare malignant disorder of the lung after a 36-year interval in a patient with Turner syndrome mosaic. Followinginitial curative radiation therapy, with a remission over 36 years, lung resection was now successfully performed.

[学科分类] 肿瘤学