ASPS is a rare sarcoma subtype with clinical specificities, such as an indolent behavior, brain metastasis and resistance to doxorubicin. AAT have shown clinical activity in this setting, but little is known on the optimal therapeutic strategy, and the management of brain metastasis (BM).
We retrospectively analyzed patients (pts) treated in 3 referral centers of the French Sarcoma Group. Factors associated with BM development and overall survival (OS) were analyzed. In addition, progression-free survivals (PFS) under AAT in patients with and without BM were reported.
We identified 75 pts [median age at diagnosis: 23 (5-96 years), 61% females]. Among those, 31 (41%) pts had documented synchronous lung metastasis (LM), and none had BM. Median OS in pts with localized and metastatic disease were 279 months, (95% CI, 279-NR) and 74 months (95% CI, 62-144) (Log-rank, p = 0.002), respectively. Only surgical complete resection (R0) was associated with better OS in localized disease (HR = 4.3; (95% CI, 1-19.3), p = 0.056). Fifty-two (69%) pts had documented LM in the course of the disease; among those, 13 (17%) pts developed BM within a median interval of 35 months (95% CI, 17-48) from LM. Initial tumor size was associated with BM-free-survival (≥5cm vs
These data highlight the indolent course of the disease leading to BM, which turned a shift in the course of the disease, along with limited efficacy of AAT in this setting. Furthermore, they suggest that the appropriate timing for AAT introduction has to be discussed in an individual basis considering the PFS/OS ratio in pts with metastatic disease.
Clinical trial identification
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Institut Gustave Roussy
G. Malouf: Consulting: Pfizer, BMS, Novartis Research grant: Pfizer, Novartis. O. Mir: Speaker: Lilly, Roche Consulting: Amgen, Astra-Zeneca, Bayer, Blueprint, BMS, Lilly, Novartis, Pfizer, Roche, Servier. J-P. Spano: conflict of interest with the following companies: Pfizer, BMS, MSD, Roche. J-Y. Blay: Research support: Novartis, Roche, GSK, Bayer, Pfizer. A. Lecesne: Honoraria: Amgen, Novartis, Pharmamar, Pfizer, Lilly. All other authors have declared no conflicts of interest.