🏠︎ » 2022 » Volume 80 - Number 3 » Sirolimus in the treatment of cystic lymphangioma in a pediatric patient
Inês Fidalgo Martins 1, Ana Cordeiro 2, Maria J. Paiva-Lopes 3
1 Área de Pediatria Médica, EPE, Rua Jacinta Marto, Lisboa, Portugal; 2 Centro Hospitalar Lisboa Central (CHLC)???Hospital Dona Estefânia, Hospital da Luz da Lisboa, Portugal, Lisboa, Portugal; 3 Department of Dermatology and Venereology, Hospital de Santo António dos Capuchos, Unidade Local de Saúde São José; Department of Dermatology and Venereology, NOVA Medical School, Faculdade de Ciências Médicas; Centro Clínico Académico de Lisboa. Lisboa, Portugal
Inês Fidalgo Martins, Ana Cordeiro, Maria J. Paiva-Lopes
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*Correspondence: Ana Cordeiro, Email not available
Cystic lymphangioma (CL) is a rare benign tumor, which occurs typically during childhood, with craniofacial, cervical or axillary being the most common locations. Lymphangiomas management can be challenging due to their permeative growth throughout tissue layers. Sirolimus is an immunosuppressive and antitumor agent that can inhibit abnormal vascular proliferation by blocking the mTOR/PI3K pathway. It is typically well-tolerated, with nausea, cytopenias, and metabolic imbalances as the most significant adverse effects. We present the case of a pediatric patient in which sirolimus was used to treat a macrocytic lymphangioma, highlighting its effectiveness and safety.