학술논문
A Case Study of a Rare Undifferentiated Spindle Cell Sarcoma of the Penis: Establishment and Characterization of Patient-Derived Models
Document Type
article
Author
Ariane Cavalcante dos Santos Sousa; Bruno Leonardo Nascimento Correa Fernandes; Jeronimo Paulo Assis da Silva; Paulo Roberto Stevanato Filho; Luiza Bitencourt de Carvalho Terci Coimbra; Adriano de Oliveira Beserra; Ana Luiza Alvarenga; Giovanna Maida; Camila Tokumoto Guimaraes; Ingrid Martinez Nakamuta; Fabio Albuquerque Marchi; Camila Alves; Martina Lichtenfels; Caroline Brunetto de Farias; Bruna Elisa Catin Kupper; Felipe D’Almeida Costa; Celso Abdon Lopes de Mello; Dirce Maria Carraro; Giovana Tardin Torrezan; Ademar Lopes; Tiago Goss dos Santos
Source
Genes, Vol 15, Iss 4, p 424 (2024)
Subject
Language
English
ISSN
2073-4425
Abstract
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.