Recent Progress in Lymphangioma

Abstract

Lymphangioma is a common type of congenital vascular disease in children with a broad spectrum of clinical manifestations. The current classification of lymphangioma by International Society for the Study of Vascular Anomalies is largely based on the clinical manifestations and complications and is not sufficient for selection of therapeutic strategies and prognosis prediction. The clinical management and outcome of lymphangioma largely depend on the clinical classification and the location of the disease, ranging from spontaneous regression with no treatment to severe sequelae even with comprehensive treatment. Recently, rapid progression has been made toward elucidating the molecular pathology of lymphangioma and the development of treatments. Several signaling pathways have been revealed to be involved in the progression and development of lymphangioma, and specific inhibitors targeting these pathways have been investigated for clinical applications and clinical trials. Some drugs already currently in clinical use for other diseases were found to be effective for lymphangioma, although the mechanisms underlying the anti-tumor effects remain unclear. Molecular classification based on molecular pathology and investigation of the molecular mechanisms of current clinical drugs is the next step toward developing more effective individualized treatment of children with lymphangioma with reduced side effects.

Cervical lymphangioma in adults: A report of seven cases and review of the literature

“…However, due to their proclivity to infiltrate nearby neurovascular structures and muscles, complete excision of lymphangiomas may be unachievable, and the effectiveness of sclerotherapy is commonly limited to macrocystic areas, with a high recurrence rate after treatment: approximately 22%-27% after surgery and approximately 57% after sclerotherapy. [28][29][30] In this study, all patients presented with a lymphangioma that had infiltrated the nearby carotid sheath. Mean-while，71.43% of the lymphangiomas located medial to the sternocleidomastoid muscle.…”

“…Meanwhile，71.43% of the lymphangiomas located medial to the sternocleidomastoid muscle. All these situations posed a challenge for the surgeons, as they had to sufficiently expose and excise tumors to avoid missing a hidden or residual portion of the tumor that might lead to a serious recurrence problem 30,31 . The successful excision of tumors infiltrated deep into the interstitial space or adjacent to vital structures requires a highly skilled surgeon.…”

“…However, an effective response (shrinkage ≥60%) to sclerotherapy mainly occurs in macrocystic lesions, and the disease recurrence rate is ~9%. Moreover, microcystic tumors have shown poor response to sclerotherapy 30,39 . Another limitation of sclerotherapy is that nearly all the relevant studies were performed in pediatric patients, and convincing evidence supporting the use of sclerotherapy for treating lymphangiomas in adult patients is still lacking.…”

“…All these situations posed a challenge for the surgeons, as they had to sufficiently expose and excise tumors to avoid missing a hidden or residual portion of the tumor that might lead to a serious recurrence problem. 30 , 31 The successful excision of tumors infiltrated deep into the interstitial space or adjacent to vital structures requires a highly skilled surgeon. In addition, the precise excision of tumors in the neck may inadvertently cause injury to the surrounding structures and result in lethal comorbidities.…”

Lymphangioma of the Lower Lip—A Diagnostic Dilemma: Report of a Rare Case with a Brief Literature Review

“…Sclerotherapy with 25% dextrose, hypertonic saline, bleomycin, and picibanil (OK-432) is considered deemed for recurrent, unresectable/surgically demanding lesions [ 37 ]. Sclerosing agents have shown superior efficacy in the management of macrocystic lymphangiomas, as compared to the microcystic lyphangiomas [ 38 ]. Senthilnathan et al treated a soft tissue lymphangioma by ultrasonographic-guided bleomycin sclerotherapy.…”