Table 1

Clinical characteristics of lymphangiomas and differential diagnosis

Age at presentationChildhood (90% <2 years-old)
Acquired when in the context of chronic lymphatic obstruction due to chronic infection, radiation therapy or trauma
Sex predilectionNone in early diagnosis, female when diagnosed in adults
Thoracic manifestationsIntrapulmonary mass
Mediastinal mass (equal distribution among compartments)
Chylous pleural or chylous pericardial effusion
Extrathoracic manifestationsHead, neck, axilla, abdomen, bones
ClassificationCapillary or simple, cavernous, cystic
Natural historyNo spontaneous resolution
Secondary infections may occur
Differential diagnosis [9]Lymphangiectasis (primary or secondary)
Lymphatic dysplasia syndromes (e.g. lymphedema, yellow nail syndrome)
Acquired lymphatic injuries (e.g. traumatic)
Other lymphatic abnormalities (e.g. lymphangioleiomyomatosis, lymphangiolipomas, haemangiolymphangiomas)