Generalized lymphangiomatosis: radiologic findings in three pediatric patients

Abstract

Generalized lymphangiomatosis is a rare disease that is characterized by widespread bony and soft tissue involvement of lymphangioma. Radiological evaluation is crucial because the site and extent of the lymphangioma are important prognostic factors. We reported here on three cases of generalized lymphangiomatosis and all three cases showed similar radiologic findings, but a different clinical course. The CT, US and MR images showed sharply defined, nonenhanced cystic lesions involving the mediastinum, bones, spleen, lung and lower neck. The whole body MR imaging with the short tau inversion recovery (STIR) sequence showed good capability for evaluating the extent of disease.

Fig. 1

Case 1. A 4-year-old boy. A. Contrast enhanced CT shows a mediastinal mass (arrowheads) enveloping the thoracic great vessels and thymus. The mass extends around the trachea and esophagus (arrow). B. The CT image with the lung window setting shows interstitial thickenings (arrowheads) and bilateral pleural effusions (arrows).

Fig. 2

Case 2. An 8-year-old boy. A. Pelvic bone radiograph shows well-defined osteolytic lesions (arrowheads) with thin sclerotic rims in both pubic bones. B. The coronal T2 weighted MR image (TR/TE = 1200/90, flip angle = 90°, slice thickness = 6 mm) shows a homogenous high-signal intensity mass in the mediastinum (arrowheads). C. On ultrasound, the mass is delineated as a multiseptated anechoic mass. Note the multiple septa in the mass (arrowheads). D. The coronal short tau inversion recovery whole body MR image shows multiple high signal intensity lesions involving vertebrae, pelvic bones, both humeri, both femurs, both tibias, the ribs, and calvarium.

Fig. 3

Case 3. An 11-year-old boy. A. The contrast enhanced CT shows a mediastinal mass (arrowheads) enveloping the thoracic great vessels and thymus (arrow). The mass extends to the posterior mediastinum (double arrow). Note the well-defined osteolytic lesion in the left fourth rib (curved arrow). B. Contrast enhanced CT shows multiple low-density lesions (arrowheads) in the spleen. Multiple well-defined osteolytic lesions with sclerotic rims are noted in the thoracic vertebra (arrows). Note the mass extending to the retroperitoneum (double arrow). C. The coronal short tau inversion recovery MR image shows multiple high-signal intensity lesions involving the bilateral lower neck (arrows), the mediastinum and the spleen (arrowheads). Note the mass extending to the posterior mediastinum (curved arrow).