Clinical Sarcoma Research

1. Overexpressed PRAME is a potential immunotherapy target in sarcoma subtypes

   PRAME (preferentially expressed antigen in melanoma), a member of the cancer-testis antigen family, has been shown to have increased expression in solid tumors, including sarcoma, and PRAME-specific therapies...

2. Disseminated and late metastatic disease from nasal pit leiomyosarcoma after radical surgical resection. Case report of a singular presentation of a rare disease

   Leiomyosarcoma of the head and neck is a rare cancer with high local aggressiveness. Radical surgery and adjuvant treatment offer the best chance for cure, nonetheless 5-years recurrence rate remains high. Des...

3. Antiangiogenic effects in patients with progressive desmoplastic small round cell tumor: data from the French national registry dedicated to the use of off-labeled targeted therapy in sarcoma (OUTC’s)

   Desmoplastic small round cell tumor (DSRCT) is a very rare mesenchymal tumor that mainly affects teenagers and young adults with a mean age at diagnosis around 20–25 years. Although initial management still ne...

4. IDH1 or -2 mutations do not predict outcome and do not cause loss of 5-hydroxymethylcytosine or altered histone modifications in central chondrosarcomas

   Mutations in isocitrate dehydrogenase (IDH)1 or -2 are found in ~50% of conventional central chondrosarcomas and in up to 87% of their assumed benign precursors enchondromas. The mutant enzyme acquires the activi...

5. Histone 3.3 hotspot mutations in conventional osteosarcomas: a comprehensive clinical and molecular characterization of six H3F3A mutated cases

   Histone 3.3 (H3.3) hotspot mutations in bone tumors occur in the vast majority of giant cell tumors of bone (GCTBs; 96%), chondroblastomas (95%) and in a few cases of osteosarcomas. However, clinical presentat...