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A Case Study of a Rare Undifferentiated Spindle Cell Sarcoma of the Penis: Establishment and Characterization of Patient-Derived Models.

Ariane Cavalcante Dos Santos SousaBruno Leonardo Nascimento Correa FernandesJeronimo Paulo Assis da SilvaPaulo Roberto Stevanato FilhoLuiza Bitencourt de Carvalho Terci CoimbraAdriano de Oliveira BeserraAna Luiza AlvarengaGiovanna MaidaCamila Tokumoto GuimaraesIngrid Martinez NakamutaFabio Albuquerque MarchiCamila AlvesMartina LichtenfelsCaroline Brunetto de FariasBruna Elisa Catin KupperFelipe D'almeida CostaCelso Abdon Lopes de MelloDirce Maria CarraroGiovana Tardin TorrezanAdemar LopesTiago Goss Dos Santos
Published in: Genes (2024)
Rare sarcomas present significant treatment challenges compared to more prevalent soft tissue sarcomas due to limited treatment options and a poor understanding of their biology. This study investigates a unique case of penile sarcoma, providing a comprehensive morphological and molecular analysis. Through the creation of experimental patient-derived models-including patient-derived xenograft (PDX), 3D, and monolayer primary cultures-we successfully replicated crucial molecular traits observed in the patient's tumor, such as smooth muscle actin and CD99 expression, along with specific mutations in genes like TSC2 and FGFR4 . These models are helpful in assessing the potential for an in-depth exploration of this tumor's biology. This comprehensive approach holds promise in identifying potential therapeutic avenues for managing this exceedingly rare soft tissue sarcoma.
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