Long-term nintedanib treatment for progressive pulmonary fibrosis associated with Hermansky-Pudlak syndrome type 1 followed by lung transplantation.

Hermansky-Pudlak syndrome (HPS) is a rare autosomal recessive disease that often causes progressive pulmonary fibrosis (HPS-PPF) in some genetic types with high mortality rates. No effective treatment for HPS-PPF other than lung transplantation has been established. Herein, we report a case of HPS type 1 with progressive pulmonary fibrosis treated with long-term nintedanib administration followed by lung transplantation. The resected lungs revealed diffuse interstitial lung lesions, including fibroblastic foci, suggesting the potential beneficial effects of anti-fibrotic drugs in HPS-PPF. Together with previous reports, the present case suggests that nintedanib might be a safe and effective drug for HPS-PPF.

A successful trimodality therapy for difficult-to-diagnose primary mediastinal dedifferentiated liposarcoma, which originated from the perihilar fat and invaded the right lungs.

A 40-year-old man with high fever, hemoptysis, and fatigue showed a 10-cm mass in the middle and lower lobes of the right lung on computed tomography. Histological examination of transbronchial biopsy specimens showed sheets of small round tumor cells and mild staining for CD99. Primary Ewing sarcoma was suspected, and a trimodality therapy consisting of chemotherapy, intensity-modulated radiation therapy, and right pneumonectomy with surrounding tissue resection was performed. In surgical specimens, negative outcome of NKX2.2 in immunostaining and EWSR1 rearrangement in fluorescence in situ hybridization did not support the diagnosis of Ewing sarcoma. Positive immunostaining for MDM2 and CDK4 led to a diagnosis of dedifferentiated liposarcoma, which probably originated from an adipose tissue of the right perihilar mediastinum, and then invaded the lungs. The postoperative course was uneventful, without recurrence for more than 16 months.