Clinical characteristics and outcomes for children, adolescents and young adults with “CIC‐fused” or “BCOR‐rearranged” soft tissue sarcomas: A multi‐institutional European retrospective analysis

Sparber‐Sauer, Monika; Corradini, Nadège; Affinita, Maria Carmen; Milano, Giuseppe Maria; Pierron, Gaëlle; Carton, Matthieu; Tirode, Franck; Pissaloux, Daniel; Alaggio, Rita; Vokuhl, Christian; Bisogno, Gianni; Berlanga, Pablo; Ferrari, Andrea; Orbach, Daniel

doi:10.1002/cam4.6113

Cited by 4 publications

(1 citation statement)

References 41 publications

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“…This classification is based on the IUCC proposal, but for the R2 margins, an interesting subdivision has been proposed that more accurately reflects the surgical situation. The R2A category reflects the situation in which the residual tumour can be further resected, R2B when radical surgery cannot be performed without mutilating the patient, and R2C when resection has not been performed due to the advanced stage [ 30 ]. The Toronto Margin Context Classification (TMCC) introduced a scale based on the clinical situation.…”

Section: Definitionsmentioning

confidence: 99%

What is the Optimal Treatment Strategy after Sarcoma R2 Surgery?

Chmiel,

Rutkowski,

Spałek

et al. 2024

Curr. Treat. Options in Oncol.

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Opinion statementSoft tissue sarcomas (STS) are rare tumours of mesenchymal origin, most commonly occurring in the extremity but also in the retroperitoneum. The curative treatment for STS is radical surgery with wide margins, in some cases in combination with perioperative radiotherapy and chemotherapy. Nonradical resection (R2) of STS has been an emerging issue in recent decades, as optimal subsequent management remains debatable. Similarly, there is still no consensus on optimal surgical margins. Combining multiple treatment modalities in adjuvant therapy can achieve local and distant control in patients following surgery with positive margins. Patients who have undergone nonradical resection therefore require additional surgical interventions, and adjuvant radiotherapy resulting in a better prognosis but a higher number of complications. Following non-radical treatment, patients with limb and trunk wall sarcomas and retroperitoneal sarcomas should also undergo increased oncological surveillance. Given the potential issues that may emerge in such clinical situations, it is crucial to up-date the current guidelines to enhance the long-term prognosis of these patients.

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Section: Definitionsmentioning

confidence: 99%