Rosa Álvarez, Agra Pujol Carolina, Arregui Valles Marta, Francisco Alijo Serrano, Fernández Gonzalo Adriana, Gutiérrez Natalia, Pablo Lozano, Cristina Mata Fernández, Mediavilla Santos Lidia, Novo Ulrike, Marina Santos, H. Peña Guillermo, Carpintero García Henar, Ana Gutiérrez
Desmoid tumors (DTs), also known as aggressive fibromatosis, are rare neoplasms characterized by local invasiveness and a high risk of recurrence, despite their lack of metastatic potential. The management of these tumors remains challenging due to their unpredictable behavior and heterogeneous presentations. In this two-part study, we first provide a comprehensive review of the scientific evidence on diagnosis and emerging therapeutic strategies for DT. In the second part, we will present a retrospective analysis of our experience at a national reference center for sarcoma treatment, focusing on diagnostic strategies, therapeutic interventions, and clinical outcomes.