A rare case of diffuse pulmonary lymphangiomatosis in a middle-aged woman

Abstract

Diffuse pulmonary lymphangiomatosis (DPL) is a rare lymphatic disorder characterized by lymphatic channel proliferation. It is mostly reported in children and young adults. Here, we report a case involving a 52-year-old asymptomatic woman who presented with increased interstitial markings, as seen on a chest radiograph. Diffuse interstitial septal thickening was found on a serial follow-up chest computed tomography scan, and lymphangitic metastasis was the primary radiologic differential diagnosis. However, histologic sections of wedge resected lung revealed diffuse pleural and interlobular septal lymphatic proliferation characteristic of DPL.

Keywords: Computed tomography; Interstitial; Lung; Lymphangiomatosis.

Fig. 1

52-year-old woman with diffuse pulmonary lymphangiomatosis. Posteroanterior chest radiograph revealing increased interstitial markings in both lungs (A). Initial CT scan demonstrating diffuse interstitial thickening in both lungs (B, C) and lymph node enlargement in right anterior diaphragmatic area (D, arrow). Follow-up CT taken eight months after initial CT scan showing increased interlobular septal thickening (E, F) with more prominent bronchovascular bundle thickening (arrows) and increased bilateral pleural effusion (G). Intra-operative photograph showing hypervascularity of lung surface (H). I. Hematoxylin and eosin-stained section of lesion showing proliferation of thin-walled, anastomosing lymphatic vessels lined by single layer of endothelial cells lacking cytological atypia (arrows, × 200). J. Immunohistochemical staining with D2-40 revealing proliferative lymphatic channels (arrows, × 200).