Cervical Cystic Lymphangiomas in Adults: A Case Series of a Rare Entity with Literature Review

Abstract

Cystic lymphangiomas (CLs) are benign congenital lymphatic malformations that are thought to represent developmental abnormalities rather than true neoplasms. The head and neck region is the most common site of occurrence, although CLs can be present elsewhere in the body including the axilla, mediastinum, groin, and retroperitoneum. More than 80% of CLs present before the age of two. Occurrence in adults is rare with only a few cases reported in the literature. We describe five cases of cervical CLs in adults and discuss imaging modalities used for diagnostics, clinicopathologic features, and therapeutic options.

Keywords: Adults; Cystic lymphangioma; Histopathology; Imaging studies.

Fig. 1

a US grey scale and b color flow Doppler demonstrate a large, well-encapsulated, anechoic, cystic mass with multiple internal septations

Fig. 2

a MGG stained smear of the fluid aspirate shows mature lymphocytes against a proteinaceous background. b Characteristic histologic findings of dilated lymphatic spaces and moderate to dense lymphoid infiltrate in the surrounding fibrocollagenous stroma (H&E × 400)

Fig. 3

CECT of the neck shows a voluminous, well-defined, thin-walled, non-enhancing mass lesion of fluid attenuation posterior to right sternocleidomastoid. It extends cranially to the C4 vertebra and caudally to the level of the thoracic inlet

Fig. 4

Ectatic lymphatic vessels lined by attenuated endothelium. Some contain lymphoid aggregates (H&E × 100)

Fig. 5

a Lymphatic channels of varying caliber (H&E × 100). b High power view shows a lymphoid aggregates in the lymph space and in the septa (H&E × 400)

Fig. 6

a and b CECT of the neck demonstrates an elongated, well-circumscribed, hypodense lesion with enhancing thin internal septations involving the right side of the neck. It is in close proximity to IJV and posteromedial to the sternocleidomastoid muscle

Fig. 7

Histology shows a multi-cystic lesion lined by flattened endothelium along with dense lymphoid infiltrate in the wall (H&E × 100)