Result of using Sirolimus in a child with lymphangioma of the thigh. Case report
CLINICAL OBSERVATION
Abstract
Lymphangioma is a benign tumor, often representing a congenital anomaly of the lymphatic system, most commonly manifesting as soft, nodular reddish or bluish lesions on the skin and mucous membranes. While typically asymptomatic and merely a cosmetic concern, larger forms may cause complications. The pathology arises from disrupted embryogenesis, where isolated lymphatic cavities fail to connect with the main vascular network, leading to their dilation, fibrosis of surrounding tissues, and secondary skin changes. Conservative treatments are largely ineffective, making surgical excision or minimally invasive procedures the mainstay of management, depending on the lesion's size and location.
The case we describe is a 15-year-old female patient with an extensive lymphangioma of the right thigh, who had been observed at the Pediatric University since childhood. Despite years of treatment (staged excisions, sclerotherapy), recurrent papillomatous growths and lymphorrhea persisted. MRI revealed multiple cystic cavities up to 1 cm in the subcutaneous tissue.
Histologically confirmed lymphangioma with chronic inflammation and weak expression of vascular endothelial growth factor (VEGF).
Despite minimal VEGF expression, sirolimus demonstrated clinical efficacy. The patient responded favorably to this treatment, whereas surgical approaches had proven less effective. Significant local improvement was documented, including reduction of lymphatic vesicles and soft tissue swelling in the thigh.
References
Ключарева С.В., Нечаева О.С., Белова Е.А., Гусева С.Н., Хаббус А.Г., Пономарев И.В. Лечение лимфангиомы лазером на парах меди. Российский журнал кожных и венерических болезней. 2016;19(6):365–369. https://doi.org/10.18821/1560-9588-2016-19-6-365-369.
Корбут И.А., Захаренкова Т.Н., Накамура Т., Хирома Т. Лимфангиома в практике акушера-гинеколога. Проблемы здоровья и экологии. 2016;(4):105–110. https://doi.org/10.51523/2708-6011.2016-13-4-23.
Соколов Ю.Ю., Донской Д.В., Вилесов А.В., Шувалов М.Э., Дзядчик А.В., Самсиков Г.А. Хирургические вмешательства у детей с интраабдоминальными лимфангиомами. Российский вестник детской хирургии, анестезиологии и реаниматологии. 2014;4(1):20–24.
Acevedo J.L., Shah R.K., Brietzke S.E. Nonsurgical therapies for lymphangiomas: a systematic review. Otolaryngol Head Neck Surg. 2008;138(4):418–424. https://doi.org/10.1016/j.otohns.2007.11.018.
Bouwman F.C.M., Kooijman S.S., Verhoeven B.H., Schultze Kool L.J., van der Vleuten C.J.M., Botden S.M.B.I., de Blaauw I. Lymphatic malformations in children: treatment outcomes of sclerotherapy in a large cohort. Eur J Pediatr. 2021;180(3):959–966. https://doi.org/10.1007/s00431-020-03811-4.
Ha J., Yu Y.C., Lannigan F. A review of the management of lymphangiomas. Curr Pediatr Rev. 2014;10(3):238–248. https://doi.org/10.2174/1573396309666131209210751.
Kumar N., Yadav P., Ansari M.S., Lal H. Surgical management of giant retroperitoneal lymphangioma in a child. BMJ Case Rep. 2020;13(2):e234447. https://doi.org/10.1136/bcr-2020-234447.
Sun J., Wang C., Song D., Wu C., Guo L. Efficacy of OK-432 sclerotherapy for different types of lymphangiomas: a review and meta-analysis. Braz J Otorhinolaryngol. 2023;89(4):101270. https://doi.org/10.1016/j.bjorl.2023.03.007.
Zhou Q., Zheng J.W., Mai H.M., Luo Q.F., Fan X.D., Su L.X., Wang Y.A., Qin Z.P. Treatment guidelines of lymphatic malformations of the head and neck. Oral Oncol. 2011;47(12):1105–1109. https://doi.org/10.1016/j.oraloncology.2011.08.001.
