Recurrent pneumothoraces in a 65-year-old female: an unusual case of cystic lung disease

A 65-year-old female presented to the emergency department with a 3-day history of dyspnoea and right-sided chest discomfort. She had been an inpatient 6 weeks earlier with right-sided pneumothorax treated with a computed tomography-guided chest drain. On this admission her chest radiograph demonstrated a recurrence of the pneumothorax (fig. 1). She was a life long nonsmoker.

Figure 1.

a) Chest radiograph showing a loculated right-sided pneumothorax. b) Histology of the lung tissue (from open lung biopsy) showed multiple small deposits, nodular or cystic, of spindle cells. Immunohistochemical markers were performed with negative staining for human melanoma black-45 monoclonal antibody and positive staining for progesterone receptor and CD10 (inset; scale bar=500 μm). Scale bar=1000 μm. c) Computed tomography of the thorax confirmed a large right-sided pneumothorax and a large cystic area in the right upper lobe. There were numerous small-walled cysts in the left lung.

She had a pneumothorax on the left side 16 years earlier. Multiple pulmonary nodules were seen on imaging and she underwent thoracoscopy and a lung biopsy. A diagnosis of metastatic endometrial stromal sarcoma was made and she underwent a total abdominal hysterectomy and bilateral salpingo-oophorectomy. She then began continued treatment with progesterone. 9 years ago she had bilateral pneumothoraces, which was treated by pleurectomy.

Uterine sarcomas are rare forms of uterine malignancy that arise from uterine smooth muscle and connective tissue. There are four main endometrial sarcomas: endometrial stromal sarcomas, leiomyosarcoma, rhabdomyosarcoma and undifferentiated endometrial sarcoma. Our patient had a low grade endometrial stromal sarcomas, which accounts for ∼15% of endometrial sarcomas. Preferred treatment is a total abdominal hysterectomy and bilateral salpingo-oophorectomy and hormonal therapy, with some centres advocating radiotherapy. Recurrences are quite common and patients can develop distant metastases after several years of tumour-free intervals [1]. The most commonly targeted site is the lung, with incidences between 7% and 28% [2]. Pulmonary nodules or cysts can be multiple or solitary. Similarly lymphangioleiomyomatosis may present with recurrent pneumothoraces but can be differentiated on the basis of histopathological appearances, and staining for human melanoma black-45 monoclonal antibody in patients with lymphangioleiomyomatosis is usually positive [3].