PMC2799640_01
Lymphangiomatosis in 38-year-old woman.. A. Coronal T2-weighted image shows large lobulated cystic mass with many septations in pelvic cavity. Preserved uterus (U) without tumor involvement is seen.. B. Perfusion lung scan with Tc-99m macroaggregated albumin shows perfusion defect in entire right lung, which was suspected to indicate complete occlusion of right pulmonary artery.. C, D. Transthoracic echocardiographs show heterogeneous echogenic mass with cystic component and incomplete coaptation of tricuspid valve, which resulted in tricuspid regurgitation (arrows in C). Mass extended to inferior vena cava (IVC) (arrows in D) (RA = right atrium, RV = right ventricle).. E, F. CT coronal (E) and axial (F) scans show non-enhanced mass with inferior vena cava and right atrial involvement, and hypoattenuating nodular lesion in right distal pulmonary artery (arrow in E).. G. Gross specimen excised from inferior vena cava, right heart and right pulmonary artery was seen as an elongated, reddish, 29 cm long mass with web-like tumor extension in right pulmonary artery (asterisk).. H. Hematoxylin & Eosin stained section of lesion shows dilated lymphatic channels with variable wall thicknesses. Based on immunohistochemical staining, tumor cells were positive for lymphatic vessel marker D2-40 (x40, insert).
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At that time, pelvic MRI had demonstrated the presence of a large cystic mass with many septations and no involvement of the adjacent organs in the pelvis (Fig. 1A). Tc-99m macro-aggregated albumin identified the absence of perfusion throughout the right lung, indicating occlusion of the right main pulmonary artery (Fig. 1B). Transthoracic echocardiography (TTE) performed for the evaluation of persistent dyspnea demonstrated the presence of a mobile echogenic tumor adhering to chordae and papillary muscles of the tricuspid valve at the right atrium and right ventricle chordae. The tumor extended from the IVC into the right heart and caused tricuspid regurgitation (Fig. 1C, D). Chest CT confirmed the presence of an elongated non-enhanced mass in the right chambers of the heart and in the IVC, with extension to the right distal pulmonary artery (Fig. 1E, F). Despite the presence of a neoplastic thrombus in the IVC, excision was straightforward except for resections of an adhesive chordal portion and a web-like tumor, which occluded the right distal pulmonary artery (Fig. 1G). Grossly, the contiguous 29 cm long mass was a well-circumscribed, gray-white tumor with a soft, rubbery consistency (Fig. 1G). Based on the histology, the tumor consisted of dilated lymphatic channels with variable wall thicknesses (Fig. 1H). Atypical features, such as, endothelial tufting, atypia and mitotic activity of lymphatic endothelium were absent, and based on immunohistochemical staining, tumor cells were positive for lymphatic vessel marker D2-40 (Fig. 1H, insert) and CD31.