Lymphangioma circumscriptum: Clinicopathological spectrum of 29 cases

Objective: To describe the clinicopathological spectrum of Lymphangioma Circumscriptum (LC). Study Design: Observational case series. Place and Duration of Study: Department of Pathology and Microbiology, AKUH, Karachi, from 2002 to 2012. Methodology: All reported cases of LC were retrieved from medical record. Clinical and pathological features were noted. Frequency percentages were determined. Results: There were 29 cases of LC predominantly males (62%). The mean age was 27.17 ± 15.5 years. The commonest sites was anal/perianal region (24%) followed by extremities (17%) and tongue, (14%). Vulval LC was seen in 3 patients. Two cases were described on scrotum. The lesions were most commonly suspected as viral warts, mole or polyp (in anal region). Vesicles with erosions and bleeding and localized growth were the usual clinical presentations. Four of the patients presented with swelling since birth. All were treated with surgical excision. Microscopic examination revealed acanthotic squamous epithelium with papillomatosis. The subepithelial region had collections of lymphatic channelscomposed of ectatic dilated vessels with serum and inflammatory cells in their lumina. The lymphatic channels were seen in deeper layers along with lymphocytic aggregates. Conclusion: Lymphangioma circumscriptum is a malformation of abnormal lymphatic channels with feeding cisterns in subcutaneous tissue. It is a benign lesion usually occurring in anal/perianal region and confused with warts. Surgical excision is preferred mode of treatment.