A case of microcystic lymphatic malformation successfully treated with topical sirolimus

Abstract

Microcystic lymphatic malformation (MLM), also known as "lymphangioma circumscriptum," is a lymphatic malformation which may involve the skin and subcutaneous tissues. Progressive growth of lesions may cause pressure to the surrounding organs. Lesions frequently reoccur after treatment with surgery, sclerotherapy, radiotherapy, and laser therapy. In the last decades, oral sirolimus has been successfully used in lymphatic malformations. Since systemic treatment with sirolimus is associated with various side effects, topical form of the drug has been used with satisfying results, especially for the angiofibromas. Promisingly, few case studies indicate topical sirolimus as a potential treatment for the lymphatic malformations. Here, we report an 8-year-old girl with MLM on left trunk whose lesions recurred after the surgery that has been performed at 1 year of age. We administered twice daily topical sirolimus at 0.75 mg/ml concentration. After 2 weeks, a local irritation occurred and the dose was decreased to once daily. The treatment was stopped at the end of 3 months as the lesions were almost cleared and the patient did not want to continue to therapy. There were no new lesions after 8 months of follow up.

Treatment With Topical Sirolimus for Recurrent Lymphatic Malformation of the External Urethral Meatus

“…However, oral sirolimus would be appropriate when the malformation is situated deeply into the urethra, or in any area where topical sirolimus is washed away rapidly. It is necessary to continue treatment with topical sirolimus, considering a recurrence risk . In this case, we proposed a maintenance therapy of 4-week cycles during which the patient used topical sirolimus during the first and second weeks and took a break during the third and fourth weeks because we observed the recurrence took approximately 2 to 3 weeks until it grew large enough to affect the patient’s quality of life.…”

Dermoscopic Assessment of Lymphangioma Circumscriptum Treated With Topical Sirolimus Ointment: A Case Report

“…In addition to cosmetic concerns, many patients often complain of pruritus, bleeding, pain, and exudation. 5…”

Topical sirolimus 0.1% for treating cutaneous microcystic lymphatic malformations in children and adults (TOPICAL): protocol for a multicenter phase 2, within-person, randomized, double-blind, vehicle-controlled clinical trial

“…Topical sirolimus is known to be efficient and well tolerated in cutaneous angiofibromas linked to tuberous sclerosis complex: several RCTs have been performed and the drug is currently used in general practice [15,16]. Recently, topical sirolimus was reported as effective in CMLMs, decreasing the number of vesicles, bleeding, oozing, and pain, in one retrospective study involving 11 patients and in three case reports [17][18][19][20]. In the reported cases of CMLMs and angiofibromas, only local side effects were reported, mostly irritative.…”