Lymphangioma in the Neurosurgical Axis: A Review and Illustrative Case Report

Lymphangiomas are benign malformations of the lymphatic system that are rarely present in adults. This report describes a rare case of cervical lymphangioma in an adult patient that mimicked a schwannoma and reviews all published cases of histologically confirmed, noncomplex adult lymphangiomas involving neurological structures. A comprehensive literature search of OVID/MEDLINE and Web of Science was conducted from inception to August 29, 2025, to identify all case reports and case series involving adult patients with histologically confirmed, noncomplex lymphangiomas affecting cranial, spinal, or peripheral nerves, either by location or symptomatology. Eighteen articles comprising 18 cases were included. Data collected included lesion location, presenting symptoms, size, imaging performed, histologic findings, treatment, and follow-up. The average patient age was 46.7 years (standard deviation 19.6), with 8 males (44%), 9 females (50%), and 1 patient (6%) whose sex was not reported. The majority of patients experienced neurological symptoms (n=14, 78%), most commonly pain (50%), paresthesia (44%), and weakness (17%); 2 patients (11%) presented with neck swelling and no other symptoms. All patients except 1 underwent surgical resection. Sixteen patients (89%) had no recurrence at the last follow-up (mean follow-up time 15.2 months; standard deviation 12.2), while 2 (11%) experienced recurrence. Although rare in adults, lymphangiomas can mimic other lesions and cause neurological symptoms due to involvement of adjacent nervous system structures. Clinicians should maintain awareness of this entity to avoid misdiagnosis and guide appropriate management. This review emphasizes the importance of recognizing lymphangiomas as a relevant consideration in neurosurgical differential diagnosis.