Hormonal contraception and pregnancy and risk of progression or relapse in desmoid-type fibromatosis (DF)

Debaudringhien, et al.


Pregnancy and exposure to hormonal contraception within 24 months did not influence DF outcome.



Initial active surveillance strategy for patients with peripheral sporadic desmoids: a multicentre phase 2 observational trial.

Bonvalot, et al.


Active surveillance of newly diagnosed DT is an effective strategy to better select indications for treatment and spare patients from aggressive treatments when unnecessary. The onset of the decrease of the ratio of median tumor size/baseline size may take 9 months.



Patterns of care and outcomes of 64 CIC-rearranged sarcoma: a retrospective multicentre case-series within the French Sarcoma Group (FSG)

Mehdi, et al.


FSG experience confirms the aggressive clinical course of CDS patients regardless of chemotherapy regimen, even if this study suggests that metastatic patients should not benefit from a therapeutic de-escalation. Molecular studies are urgently needed to improve understanding and treatment of this orphan disease.



Phase I/II study of MAK683 in patients (pts) with advanced malignancies including epithelioid sarcoma

Bahleda, et al.


MAK683 was generally well-tolerated and there were preliminary signs of activity in pts with advanced epithelioid sarcoma.



GEMMK: A phase I study of gemcitabine (gem) and pembrolizumab (pem) in patients (pts) with leiomyosarcoma (LMS) and undifferentiated pleomorphic sarcoma UPS).

Smrke, et al.


Gem-pem is safe and efficacy continues to be evaluated in the expansion cohort. A MTD dose expansion cohort is fully enrolled and follow-up is ongoing.



Biomarkers of response and hyperprogression in patients with sarcoma treated with checkpoint blockade

N.D. Klemen, et al.


This study of patients with sarcoma treated with PD-1 blockade demonstrates an ORR of 16%. Low disease burden was associated with response. Patients with liver metastases were less likely to respond while those with nodal metastases were more likely. The incidence of HPD in sarcoma is comparable to other solid tumors, but we did not detect clinical or biological differences between PD and HPD tumors except disease burden at baseline. HPD does occur in sarcoma but its clinical significance remains uncertain.