Clinical History
We present the case of a 34-year-old African woman, to our observation as outpatient, for a soft and palpable neck mass to be investigated with MR in view of an eventual surgical removal. Her clinical past history was irrelevant.
Imaging Findings
A previous neck ultrasound revealed a large fluid mass on left supraclavicular region, and its cytologic fluid analysis resulted in some white blood cells and chyle.
MR imaging of the neck confirmed large multicystic fluid-filled masses, presenting as multiseptated fluid collections without diffusion restriction, on both sides (Fig. 1, 2).
Besides, MR demonstrated multiple and focal bone lesions, in particular on several ribs, sternum and several vertebral bodies. These lesions presented variable signal intensity, appearing mainly hypo- to isointense on T1-weighted and hyperintense on T2-weighted images (Fig. 3).
On the suspicion of systemic lymphangiomatosis, an abdominal and lung ultrasound (US) was performed some days after the MR examination.
US examination revealed multiple cysts of the spleen, the largest about 25 mm, in addition to this no further changes in abdominal organs nor pleural effusion were found.
Discussion
Lymphangiomatosis is a rare condition characterised by a diffuse proliferation of lymphatic vessels. It is most common in children and young adults. Symptoms and prognosis vary greatly between individuals and according to the organs involved [1, 2].
The majority of patients have both visceral and skeletal involvement, most commonly in the spleen, liver, lung, long bones, pelvis, skull, and vertebrae. Although this condition is not generally thought to metastasize, the lesions may be extremely destructive locally.
General radiologic findings may include massive chylothorax, pulmonary interstitial infiltrates, pleural effusion, splenomegaly, splenic cysts, and lytic bone lesions [1, 2].
The skeletal changes frequently consist of multiple, rounded, cyst-like lesions, found particularly in the pelvis and upper femur, however, lesions in the spine are not uncommon [3]. In this context, MR can be helpful in suggesting the correct diagnosis, as in our case.
The diagnosis of lymphangiomatosis is made through clinical suspicion combined with radiologic findings. The coexistence of lytic bone lesions and chylothorax supports the diagnosis of lymphangiomatosis.
Differential Diagnosis List
Systemic lymphangiomatosis
Vertebral haemangioma
Lytic bone lesions
Final Diagnosis
Systemic lymphangiomatosis
