An unusual case of cystic interstitial lung disease

摘要

In March, 2011, a 27-year-old woman was admitted to us with persistent headache. MRI of the head showed a contrast-enhanced right parasagittal tumour. The mass was heterogeneous, with areas of haemorrhage causing compression of the surrounding tissue. The findings were consistent with a meningioma. Radiograph of the chest showed a diffuse reticulo-nodular interstitial infiltrate, and left pneumothorax. High-resolution CT showed cystic lesions disseminated in both lungs, predominantly in the lower lobes and near the pleural surface (figure A). The cysts had thin walls and measured a few millimetres to one centimetre and, were often confluent in cystic airspaces. There were also multiple centrilobular and peribronchial micronodules. CT confirmed pneumothorax, which was complete on the left side and apical on the right. Our patient had no respiratory complaints. The radiological findings were initially interpreted as a cystic lung disease associated with an interstitial infiltrative pattern.Serum laboratory tests (including anti-nudear antibody, extractable nuclear antigen, Ig-electrophoresis, HIV) and a CT of the abdomen and pelvis were unremarkable. She had a craniotomy with complete removal of the intracranial tumour. Histopathological examination showed a grade I meningioma with 3% of Ki67/MIB1 labelling index. 3 weeks later, she had surgical lung biopsy through a left video-assisted thoracoscopic approach. Pleural abrasion was done to prevent pneumothorax recurrence. Histology showed several bland-looking' nodular and cystic proliferations of spindle cells with intranuclear inclusions, few mitotic figures and no necrosis (see appendix). These cells diffusely expressed Vimentin, EMA, CD56 and oestrogen/progesterone receptors, and focally expressed S100 but not HMB45, Melan-A, smooth-muscle actin, desmin, CD34, CD68, chromogranin, synaptophysin, cytokeratins (AE1/AE3), and CDla (see appendix). The morphology and immunoprofile of both brain tumour and pulmonary lesions were identical and a diagnosis of pulmonary metastases from meningioma was made. The diagnosis was further confirmed by molecular tests showing a wild-type set-up for mutational events in NF1 and NF2 genes and loss-of-heterozygosity at 22q. Chemotherapy with hydroxyurea was started, according to National Comprehensive Cancer Network guidelines. In June, 2012,1 year after the onset of chemotherapy, she was well, with no signs of relapse of the primary tumour.