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Metastatic Primary Brain Rhabdomyosarcoma In A Pediatric Patient: Illustrative Case, Michel Gustavo Mondragón-Soto, Luis Del Valle, José Alfredo González-Soto, Roberto Alfonso De Leo-Vargas
Metastatic Primary Brain Rhabdomyosarcoma In A Pediatric Patient: Illustrative Case, Michel Gustavo Mondragón-Soto, Luis Del Valle, José Alfredo González-Soto, Roberto Alfonso De Leo-Vargas
School of Medicine Faculty Publications
BACKGROUND: Primary intracranial rhabdomyosarcoma is an extraordinarily rare malignant tumor, with even fewer presenting with distant metastasis. To date, only five cases, including the one presented here, have been reported to present metastatic activity. OBSERVATIONS: A 12-year-old boy presented with a few days of headache, nausea, vomiting, but no neurological deficit. Brain computed tomography and magnetic resonance imaging demonstrated hydrocephalus and a cystic lesion with left parieto-occipital extension. After resection, pathology reported primary rhabdomyosarcoma, with positive desmin and myogenin on immunohistochemistry. The patient presented with pulmonary metastasis. The patient had an overall survival of 21 months after diagnosis with optimal …
Met Inhibition Sensitizes Rhabdomyosarcoma Cells To Notch Signaling Suppression, Clara Perrone, Silvia Pomella, Matteo Cassandri, Michele Pezzella, Giuseppe Maria Milano, Marta Colletti, Cristina Cossetti, Giulia Pericoli, Angela Di Giannatale, Emmanuel De Billy, Maria Vinci, Stefania Petrini, Francesco Marampon, Concetta Quintarelli, Riccardo Taulli, Josep Roma, Soledad Gallego, Simona Camero, Paolo Mariottini, Manuela Cervelli, Roberta Maestro, Lucio Miele, Biagio De Angelis, Franco Locatelli, Rossella Rota
Met Inhibition Sensitizes Rhabdomyosarcoma Cells To Notch Signaling Suppression, Clara Perrone, Silvia Pomella, Matteo Cassandri, Michele Pezzella, Giuseppe Maria Milano, Marta Colletti, Cristina Cossetti, Giulia Pericoli, Angela Di Giannatale, Emmanuel De Billy, Maria Vinci, Stefania Petrini, Francesco Marampon, Concetta Quintarelli, Riccardo Taulli, Josep Roma, Soledad Gallego, Simona Camero, Paolo Mariottini, Manuela Cervelli, Roberta Maestro, Lucio Miele, Biagio De Angelis, Franco Locatelli, Rossella Rota
School of Medicine Faculty Publications
Rhabdomyosarcoma (RMS) is a pediatric myogenic soft tissue sarcoma. The Fusion-Positive (FP) subtype expresses the chimeric protein PAX3-FOXO1 (P3F) while the Fusion-Negative (FN) is devoid of any gene translocation. FP-RMS and metastatic FN-RMS are often unresponsive to conventional therapy. Therefore, novel therapeutic approaches are needed to halt tumor progression. NOTCH signaling has oncogenic functions in RMS and its pharmacologic inhibition through γ-secretase inhibitors blocks tumor growth in vitro and in vivo. Here, we show that NOTCH signaling blockade resulted in the up-regulation and phosphorylation of the MET oncogene in both RH30 (FP-RMS) and RD (FN-RMS) cell lines. Pharmacologic inhibition of …