Background: In 9 out of 10 cases, lymphangiomas are observed during the first years of life, generally located in the neck. Rare lymphangiomas have been reported in adults, usually in an intrathoracic localization, raising the hypothesis of an acquired origin.
Patients and methods: A retrospective analysis of patients undergoing surgery for lymphanioma of the neck or mediastinum was conducted. Pathology reports, clinical data and medical imaging (CT) were reviewed and operative and post-operative findings were analyzed in order to determine whether the cases could be divided into congenital and non-congenital forms.
Results: Data were obtained on 37 patients (23 men and 14 women, mean age 45 years, range 8-77). Four lesions (11%) were located in the neck: 3 in children and one in an elderly subject (77 yrs). Thirty-three tumors (89%) were located in the mediastinum. In 5 cases, the tumor was located in the phrenic nodes of the anterior mediastinum. These lymphangiomas occurred in young adults, had a CT density less than liquid with enhancement after contrast injection, and had a malformative vascular component proven anatomically and histologically (hemolymphangioma). These elements favored a congenital orgin. In 28 cases (76.6%) the tumor involved nodes in the posterior or middle mediastinum. They occurred in older adults and were purely liquid with no vascular component. These lymphangiomas were undoubtedly acquired.
Conclusion: In children, adolescents and young adults, lymphagiomas are found in the neck or anterior mediastinum and have a tissular component. These tumors should be considered as congenital lymphangiomas. In older adults, lymphangiomas are found in the posterior or middle mediastinum and are purely liquid cysts suggesting an acquired origin.