Generalized cystic lymphangiomatosis incidentally diagnosed in an asymptomatic adult: Imaging findings of a very rare case

Abstract

Lymphangiomas are benign lesions of vascular origin with lymphatic differentiation, most commonly found in the head and neck. Generalized lymphangiomatosis is a very rare condition in adults, which is characterized by a diffuse proliferation of lymphatic vessels. The lymphangioma is composed of lymphatic endothelium-lined cystic spaces. This condition can be histologically differentiated from other vascular disorders such as cavernous or capillary hemangioma. However, many cases of lymphangioma can be confused with other vascular disorders, because of overlapping histologic findings. radiologic examinations, such as CT scan and MR imaging, are useful for assessing the morphologic feature and also the extent of disease, it is important to know the radiologic findings of generalized lymphangiomatosis. In this paper, we report a case of generalized lymphangiomatosis in a 42-year-old male who presented with left flank pain and hematuria. The first differential diagnosis was renal colic; hence he underwent an abdominopelvic computed tomography scan (CT scan). In the performed CT scan multiple cystic lesions were seen in the liver and spleen. Also, lytic lesions were seen in bones. CT-guided biopsy was performed and the result was compatible with generalized lymphangiomatosis, confirmed by cytology. Generalized lymphangiomatosis is a rarely reported disease in children and young adults. Delayed diagnosis in older patients or misdiagnosis is common due to its rarity and nonspecific clinical presentation. Different imaging modalities can incidentally diagnose the disease in asymptomatic patients. So radiologists should be aware of the disease manifestations in imaging modalities to diagnose the disease sooner and help the clinician start the therapy if needed.

Keywords: Computed tomography scan; Generalized lymphangiomatosis; Lymphangioma; Lymphangiomatosis; Magnetic resonance imaging cytology.

Fig. 1

A round heterogeneous lesion which is hypodense in comparison with liver parenchyma, with density more than water measured up to 7 mm in the liver. It may contain internal echo or thin septations on ultrasound.

Fig. 2

Multiple hypodense lesions in spleen with some internal and wall calcifications measured up to 27 mm.

Fig. 3

A lytic lesion in the 12th thoracic vertebral body, without cortical destruction.

Fig. 4

Multilocular lytic lesions without soft tissue component and extraosseous extension, in bilateral iliac bones in the vicinity of sacroiliac joints.

Fig. 5

A large well-defined cystic lesion containing calcification in the anterior superior mediastinum. This lesion caused thoracic aorta encasement. Extension of the lesion to the left paracardiac region is noted.

Fig. 6

A large well-defined cystic lesion containing calcification in the anterior, superior, and inferior mediastinum. This lesion caused thoracic aorta encasement. Extension of the lesion to the left paracardiac region is noted.

Fig. 7

Extension of the mediastinal mass to the left posterior triangle of the left neck is also noted.

Fig. 8

(A–C) In the pelvic MRI, there were 2 well-defined multiseptated cystic lesions in bilateral iliac bones which show high signal intensity on T2 and low signal intensity on T1 sequence. No cortical disruption or soft tissue component was visible. Regarding patient history, findings are compatible with macro cystic lymphangioma.