CStone submits new drug application for the targeted therapy avapritinib in Taiwan for the treatment of adults with advanced PDGFRA exon 18 mutant gastrointestinal stromal tumor

On March 27, 2020 CStone Pharmaceuticals ("CStone" or the "Company", HKEX: 2616) reported that the Company has submitted a New Drug Application (NDA) to the Taiwan Food and Drug Administration (TFDA) for avapritinib, a precision therapy being developed for the treatment of gastrointestinal stromal tumor (GIST), and had received priority review designation from the TFDA on March 9, 2020 (Press release, CStone Pharmaceauticals, MAR 27, 2020, View Source [SID1234555949]). This NDA is for the indication of adult patients with unresectable or metastatic GIST harboring a platelet-derived growth factor receptor alpha (PDGFRA) exon 18 mutation, including PDGFRA D842V mutations. Discovered by CStone’s partner, Blueprint Medicines, avapritinib is the second drug candidate for which CStone has submitted an NDA.

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"Within just one year, CStone has submitted NDAs for two first-in-class targeted-therapies, demonstrating our commitment to addressing urgent clinical needs and the Company’s rapid transformation toward commercialization," said Dr. Frank Jiang, Chairman and CEO of CStone. "GIST is a rare tumor type, and PDGFRA D842V mutant GIST is resistant to currently approved therapies in Greater China. In addition to Taiwan, we plan to submit an NDA for the same indication in Mainland China in the first half of this year with the goal of benefitting more patients suffering from this devastating condition."

"Avapritinib is an investigational, orally available, potent, and selective inhibitor of KIT and PDGFRA. In January this year, avapritinib received an approval from the U.S. FDA for the treatment of adults with unresectable or metastatic PDGFRA exon 18 mutant GIST and became the first and only U.S. FDA-approved targeted therapy for this indication," said Dr. Jason Yang, Chief Medical Officer of CStone. "Clinical data submitted to the TFDA show unprecedented antitumor activity of avapritinib in advanced GIST patients with a PDGFRA exon 18 mutation, including PDGFRA D842V mutations, with an overall response rate (ORR) of 84% and most adverse events reported as Grade 1 or 2. In addition, the median duration of response (DOR) was not reached and 61% of these patients had a DOR ≥6 months. "

GIST is the most common mesenchymal tumor of the GI tract, and it is most prevalent in patients aged 50 to 80. Around 90% of all GIST cases are associated with dysregulated cell growth due to mutations in KIT and PDGFRA tyrosine kinases. As GIST patients do not respond well to chemotherapy and radiotherapy, current treatment for advanced GIST is primarily based on sequential treatment with tyrosine kinase inhibitors (TKIs). However, GIST patients harboring PDGFRA D842V mutations are not sensitive to existing approved TKIs in Greater China, with studies showing an ORR of 0%, a median progression-free survival of just three to five months, and a median overall survival of about 15 months1. Similarly, these approved therapies have shown limited effects against other rare PDGFRA exon 18 mutations.

CStone Pharmaceuticals and Blueprint Medicines have an exclusive collaboration and license agreement for the development and commercialization of avapritinib and certain other drug candidates in Mainland China, Hong Kong, Macau and Taiwan. Blueprint Medicines retains development and commercial rights for these licensed products in the rest of the world.

About Avapritinib

Avapritinib is an investigational, selective and potent inhibitor of KIT and PDGFRA mutant kinases. It is a type 1 inhibitor that works by directly binding to the active kinase conformation from which mutant KIT and PDGFRA signal. Avapritinib has demonstrated inhibition of a broad range of KIT and PDGFRA mutations associated with GIST, including potent clinical activity against activation loop mutations that are associated with resistance to currently approved therapies in Greater China.

Blueprint Medicines is pursuing a broad clinical development program for avapritinib across multiple lines of GIST treatment, as well as for advanced, smoldering and indolent systemic mastocytosis.

Avapritinib is a kinase inhibitor approved by the U.S. FDA under the brand name AYVAKIT for the treatment of adults with unresectable or metastatic GIST harboring a PDGFRA exon 18 mutation, including PDGFRA D842V mutations.

Avapritinib is not approved for the treatment of any other indication in the U.S. or for the treatment of any indication by the TFDA in Taiwan, by the National Medical Products Administration in China or by any other health authority in any other jurisdiction.