A Rare Case of Diffuse Pulmonary Lymphangiomatosis in a Middle-Aged Woman
A 52-year-old woman with abnormal chest radiographs was referred to our hospital. She was asymptomatic and denied having any cough, wheezing, or hemoptysis. Her past medical history was unremarkable. The findings of a physical examination were normal. Laboratory examination revealed a hemoglobin level of 14.1 g/dL, a white blood cell count of 8160/µL (61.7% neutrophils, 31.1% lymphocytes, 2.6% eosinophils, 4.4% monocytes, and 0.2% basophils), and a platelet count of 209000/µL. Urine analysis findings, blood chemistry findings, and erythrocyte sedimentation rates were normal.\nPosteroanterior chest radiograph showed increased interstitial markings in both lungs (). Diffuse, smooth, and nodular interlobular septal thickening, and minimal amounts of bilateral pleural effusion were demonstrated on CT scan (). Low-density infiltration of mediastinal fat and lymph node enlargement were noted in the right anterior diaphragmatic area of a mediastinal window (). Although the patient was asymptomatic, these imaging findings persisted on the follow-up CT scan taken one month later. Our primary radiologic impression was that this was a case of lymphangitic carcinomatosis. Pulmonary edema, sarcoidosis, and lymphoma were included in the differential diagnosis.\nA pulmonary function test showed mild restrictive patterns. The patient underwent bronchoscopy, and no endobronchial lesion was found. Bronchoalveolar lavage results were negative for malignant cells. Thick varicose septal veins and intraalveolar macrophages were identified on transbronchial lung biopsy.\nAfter seven months, a follow-up chest CT scan revealed increased interstitial septal thickening (), new peribronchovascular cuffing, and small amounts of bilateral pleural effusion (). Finally, the patient underwent video-assisted thoracoscopic surgery wedge resection of the right, middle, and lower lobes. In the surgical field, abnormal hypervascularity was noted on the lung surface ().\nMicroscopic examination showed proliferation of thinwalled, anastomosing vascular spaces lined by a single layer of endothelial cells lacking cytological atypia (). Theses lesions were located along the lymphatic distribution and were highlighted by D2-40 (1:100, Dako, Glostrup, Denmark) immunohistochemical stain (), characteristic of DPL. Presently, the patient is alive without any symptoms and being observed without specific treatment such as low fat medium chain fat.