Potential utilization of lymphoscintigraphy in patients with kaposiform hemangioendothelioma

Kaposiform hemangioendothelioma (KHE) is characterized by the dysregulation of both angiogenic and lymphangiogenic pathways. The application of lymphoscintigraphy in the management of KHE remains poorly documented. This study aims to delineate the lymphoscintigraphic imaging features of KHE and assess the potential utility of this modality in clinical management.

In this retrospective study, we evaluated lymphoscintigraphic features in patients with KHE. The assessed parameters included the extent of dermal backflow, flow patterns, lymphatic stasis, regional lymph node visualization, and collateral vessel formation. Findings were classified into three grades of obstruction severity. In patients with follow-up scans, temporal changes from baseline were systematically characterized.

Twenty-five patients (15 male, 10 female) with KHE underwent lymphoscintigraphy, comprising 24 with extremity and one with truncal involvement. Abnormal findings were detected in 22 of the 24 extremity cases. In all abnormal cases, lymphedema was unilateral and ipsilateral to the lesion. Lymphatic stasis within the involved lesions was a universal finding (100%, 22/22). Regarding flow patterns, 68% (15/22) of these patients demonstrated a restricted pattern, while the remaining 32% (7/22) exhibited a trunk flow pattern. Among the six patients who underwent follow-up lymphoscintigraphy, five showed no significant improvement in lymphatic drainage. However, one patient, who initially presented with partial obstruction, achieved complete resolution with normal drainage on subsequent imaging.

Lymphoscintigraphy effectively characterizes lymphatic dysfunction in KHE, aiding in both initial diagnosis and the long-term management of secondary lymphedema.