Lymphangiomatosis is a rare disorder that affects multiorgan: cyst formation due to lymphatic channel increase and increased permeability causes various symptoms. Its optimal treatment strategy is yet to be determined. Lymphatic vessel proliferation is regulated by the expression of the signal protein Vascular Endothelial Growth Factor (VEGF) and its subtypes and monoclonal antibodies targeting this factor have been used in the treatment of several human tumors. Since lymphangiomatosis is also due to abnormal lymphatic vessel proliferation, we hypothesized that anti-angiogenic agent Bevacizumab may also be effective in this lymphatic disorder. Here, we report a male patient with lymphangiomatosis: Bevacizumab successfully ameliorated generalized edema and multiple massive lymphatic cysts. Although experience of one case does not lead in definitive results, we suggest that Bevacizumab may be an option for intractable cases of lymphangiomatosis.
Sotirios Sachanas*, Gerassimos A. Pangalis, Anastasios Koutsopoulos, Panagiotis Kekis, Dimitrios Tsamis, Chrysothea Zaroulis, Efstathios Koulieris, Maria Moschogiannis, Xanthi Yiakoumis and Ioannis Anastasakis
Journal of Rare Disorders: Diagnosis & Therapy received 241 citations as per google scholar report