Clinical, Cosmetic and Investigational Dermatology (Sep 2023)
Surgical Treatment for Benign Lymphangioendothelioma After Two Incomplete Excisions: A Case Report and Literature Review
Abstract
Wei Lu,1 Yan Cao,2 Fanhua Zeng,1,3 Chun Chen,1,4 Zhenyu Yang,1 Zuoliang Qi,1 Xiaonan Yang1 1The Department of Hemangioma and Vascular Malformation, Plastic Surgery Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, People’s Republic of China; 2The Department of Pathology, Plastic Surgery Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, People’s Republic of China; 3The Department of Burn and Plastic Surgery, Hengyang No.1 People’s Hospital, Hunan, People’s Republic of China; 4E.N.T. Department, Shenzhen Longgang District Third People’s Hospital, Guangdong, People’s Republic of ChinaCorrespondence: Xiaonan Yang, The Department of Hemangioma and Vascular Malformation, Plastic Surgery Hospital, Chinese Academy of Medical Sciences (CAMS) and Peking Union Medical College (PUMC), Beijing, 100144, Tel +86 18810601889, Fax +86 01088964614, Email [email protected]: Benign lymphangioendothelioma (BL) is a rare, poorly identified, slow-growing benign vascular lesion characterized by asymptomatic, solitary, well-demarcated macules, or by mildly infiltrated plaque. We report a case of an atypical BL that arose as a tender, protuberant, flesh-colored mass with cyanotic vesicles, and then progressed to a persistent exudative wound after two incomplete excisions. The patient was also diagnosed with thoracic duct narrowing. Although the stenosis was removed by surgery, the right lower extremity ulceration and exudation did not improve. Thus, we performed a thorough excision and split-thickness skin graft transplant following vacuum sealing drainage, and eventually the patient had a favorable functional and cosmetic outcome. A biopsy revealed irregular, dilated vascular spaces lined with a single layer of flat endothelial cells extending from the superficial dermis to the subcutis that did not reach the striated muscles. Additionally, by reviewing the literature on BL, in this paper we summarize the diverse pathogenic, morphological, and immunohistochemical presentations for this rare disease, as well as the histopathological differential diagnosis of lymphangiomatosis, Kaposi’s sarcoma, and angiosarcoma.Keywords: acquired progressive lymphangioma, angiosarcoma, benign lymphangioendothelioma, Kaposi’s sarcoma, lymphangioma, lymphangiomatosis
