Generalized lymphangiomatosis-A rare manifestation of lymphatic malformation

Abstract

Lymphangiomatosis is a rare benign proliferation and dilation of the lymphatic channels that can involve multiple organs with a variety of clinical presentations. We report a case of generalized lymphangiomatosis in a 16-year-old male who presented with a subcutaneous swelling and pain over his lower back area. The patient was diagnosed of generalized lymphangiomatosis with involvement of soft tissues, pulmonary, thoracic and lumbosacral vertebrae, and spinal canal which subsequently confirmed by cytology. In this case report, we aim to discuss radiological features of the relevant differentials, and the patient's symptoms. We also briefly discuss the treatment options as well as prognosis.

Keywords: Computed tomography; Cytology; Generalized lymphangiomatosis; Magnetic resonance imaging.

Fig. 1

Axial (A) and coronal (B) noncontrast CT images showing multilocular cystic lesions within bilateral psoas muscles, left extensor muscles, and left lower back subcutaneous region (white arrows) with calcified node in the right lesion (black arrow). Osteolytic lesion in the vertebral body and pedicle of L5 (arrowheads)

Fig. 2

Sagittal CT image of the bone window demonstrates sharply defined osteolytic lesions with sclerosis over lumbar vertebrae (white arrows), and a mixed area of osteolysis with and without sclerosis over sacral vertebrae (black arrows)

Fig. 3

Coronal and axial T2W HASTE magnetic resonance (MR) image (A and C) showing markedly hyperintense lesions with septa (white arrows). Coronal T1-weighted postgadolinium MR image (B) showing peripheral capsular and septal enhancement (black arrows)

Fig. 4

Sagittal T2-weighted MR image shows hyperintense lesions over lumbosacral vertebrae, extending into the spinal canal that results in nerve root compression of cauda equina (arrows)

Fig. 5

Lung CT shows interstitial thickening with interstitial nodules (black arrows). Well circumscribed osteolytic lesions of the thoracic vertebrae were also noted (white arrow). No evident of pleural effusion and mediastinum adenopathy

Fig. 6

Cytologic evaluation showed a heterologous population of mature lymphocytes with chylomicrons without suspected malignant cells (Giemsa stain, at 40× magnification)