Metastatic endometrial stromal sarcoma masquerading as pulmonary lymphangioleiomyomatosis

A 39 year old female presented with bilateral pneumothoraces and interstiti al shadowing on chest x ray. A diagnosis of lymphangioleiomyomatosis was ma de following an open lung biopsy. Over the next eight years she developed r espiratory failure leading to single lung transplantation but she died in t he immediate postoperative period. Necropsy examination and review of the p revious open lung biopsy revealed multiple pulmonary metastases from a low grade endometrial stromal sarcoma of the uterus. This case highlights the i mportance of an accurate diagnosis before transplantation.