Neoadjuvant Everolimus for Adult Giant Mesenteric Cystic Lymphangioma with mTOR Pathway Activation

Abstract

Cystic lymphangioma are rare benign vascular or lymphatic tumors, diagnosed mostly in newborns or children, that may become life-threatening because of local invasiveness. Surgical "en-bloc" resection with negative margins is the only curative treatment, but some patients are diagnosed with unresectable tumors. We describe the case of a young adult with giant unresectable mesenteric lymphangioma. Extensive pathological characterization as well as whole exome and transcriptome sequencing enabled us to identify

Nonpolypous Hamartomas of the Gastrointestinal Tract: An Updated Review on Classification, Denominations, and Clinical Management

“…Based on the size of the dilated vessels, lymphangiomas are classified in capillary, cavernous and cystic lymphangiomas [ 4 , 21 ]. If capillary and cavernous lymphangiomas are mainly developed in the submucosa of the GI tract, cystic lymphangioma is rather a mesenteric lesion developed as result of the mTOR pathway activation, which is therapeutically inhibited by everolimus [ 21 , 22 ].…”