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Systemic treatments in MDM2 positive intimal sarcoma: A multicentre experience with anthracycline, gemcitabine, and pazopanib within the World Sarcoma Network
Author(s) -
Frezza Anna Maria,
Assi Tarek,
Lo Vullo Salvatore,
BenAmi Eytan,
Dufresne Armelle,
Yonemori Kan,
Noguchi Emi,
Siontis Brittany,
Ferraro Richard,
Teterycz Pawel,
Duffaud Florence,
Ravi Vinod,
Vincenzi Bruno,
Gelderblom Hans,
Pantaleo Maria A.,
Baldi Giacomo G.,
Desar Ingrid,
Fedenko Alexander,
Maki Robert G.,
Jones Robin L.,
Benjamin Robert S.,
Blay Jean Yves,
Kawai Akira,
Gounder Mrinal,
Gronchi Alessandro,
Le Cesne Axel,
Mir Olivier,
Czarnecka Anna M.,
Schuetze Scott,
Wagner Andrew J.,
Adam Julien,
Barisella Marta,
Sbaraglia Marta,
Hornick Jason L.,
Meurgey Alexandra,
Mariani Luigi,
Casali Paolo G.,
Thornton Katherine,
Stacchiotti Silvia
Publication year - 2020
Publication title -
cancer
Language(s) - English
Resource type - Journals
SCImago Journal Rank - 3.052
H-Index - 304
eISSN - 1097-0142
pISSN - 0008-543X
DOI - 10.1002/cncr.32508
Subject(s) - pazopanib , medicine , gemcitabine , anthracycline , oncology , regimen , sarcoma , retrospective cohort study , surgery , chemotherapy , cancer , pathology , breast cancer , sunitinib
Background Intimal sarcoma (InS) is an exceedingly rare neoplasm with an unfavorable prognosis, for which new potentially active treatments are under development. We report on the activity of anthracycline‐based regimens, gemcitabine‐based regimens, and pazopanib in patients with InS. Methods Seventeen sarcoma reference centers in Europe, the United States, and Japan contributed data to this retrospective analysis. Patients with MDM2‐positive InS who were treated with anthracycline‐based regimens, gemcitabine‐based regimens, or pazopanib between October 2001 and January 2018 were selected. Local pathological review was performed to confirm diagnosis. Response was assessed by RECIST1.1. Recurrence‐free survival (RFS), progression‐free survival (PFS) and overall survival were computed by Kaplan‐Meier method. Results Seventy‐two patients were included (66 anthracycline‐based regimens; 26 gemcitabine‐based regimens; 12 pazopanib). In the anthracycline‐based group, 24 (36%) patients were treated for localized disease, and 42 (64%) patients were treated for advanced disease. The real‐world overall response rate (rwORR) was 38%. For patients with localized disease, the median RFS was 14.6 months. For patients with advanced disease, the median PFS was 7.7 months. No anthracycline‐related cardiac toxicity was reported in patients with cardiac InS (n = 26). For gemcitabine and pazopanib, the rwORR was 8%, and the median PFS was 3.2 and 3.7 months, respectively. Conclusion This retrospective series shows the activity of anthracycline‐based regimens in InS. Of note, anthracyclines were used in patients with cardiac InS with no significant cardiac toxicity. The prognosis in patients with InS remains poor, and new active drugs and treatment strategies are needed.