Senhwa’s Silmitasertib Receives US FDA Orphan Drug Designation for the Treatment of Medulloblastoma

On December 17, 2021 Senhwa Biosciences, Inc. (TPEx: 6492), a drug development company focusing on first-in-class therapeutics for oncology, rare diseases, and novel coronaviruses, reported that the US Food and Drug Administration (FDA) has granted Orphan Drug Designation (ODD) for Silmitasertib, a highly selective inhibitor of casein kinase 2 (CK2) to treat patients with Medulloblastoma (Press release, Senhwa Biosciences, DEC 17, 2021, View Source [SID1234597382]).

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The FDA grants ODD status to drugs and biologics that are intended to treat, prevent or diagnose a rare disease or condition with a prevalence of fewer than 200,000 people in the U.S. ODD affords certain financial incentives to support clinical development, exemption from user fees and the potential for up to seven years of market exclusivity in the U.S. upon marketing approval.

"We are pleased to receive ODD for Silmitasertib for Medulloblastoma, a rare, severe pediatric disease for which there are no approved targeted therapies. ODD represents an important regulatory milestone that has the potential to expedite the clinical development of Silmitasertib, a potent and selective CK2 inhibitor," said Dr. John Soong, Chief Medical Officer of Senhwa Biosciences.

Senhwa’s clinical partner, the Pediatric Brain Tumor Consortium (PBTC, www.pbtc.org), is currently conducting a Phase I/II and Surgical Study of Silmitasertib in both children and adults with recurrent sonic hedgehog (SHH) () driven Medulloblastoma. This study is taking place at the PBTC’s participating member academic medical centers and children’s hospitals across the United States. The PBTC is sponsoring this clinical trial and is funded through the Consortium grant awarded by the US National Institute of Health – Cancer Therapy Evaluation Program (CTEP).

Medulloblastoma is the most common cancerous brain tumor in children, but no targeted therapy is currently available. In addition to ODD, Silmitasertib was granted Fast Track Designation and Rare Pediatric Disease (RPD) Designation from the US FDA on Aug 17, 2021 and July 6, 2020, respectively. If certain criteria are met with the RPD Designation, Senhwa would be eligible for a transferrable Priority Review Voucher (PRV). The PRV allows its recipient an expedited review process of any one of its new drug products from a ten-month to a six-month timeframe.

About Silmitasertib

Silmitasertib is a first-in-class small molecule drug that targets the CK2 pathway and acts as a CK2-inhibitor. Clinical studies thus far have shown CX-4945 to be well-tolerated in humans. In addition to COVID-19, Silmitasertib is currently under development in several oncology programs in adults and children with recurrent/advanced or metastatic cancer. To date, three Phase I trials and one Phase II trial of Silmitasertib in cancer patients have been completed; currently, there are two ongoing Phase II studies of Silmitasertib. The US FDA granted Silmitasertib key drug designations: Orphan Drug Designation for the treatment of Cholangiocarcinoma in December 2016, Rare Pediatric Disease Designation in July 2020 for the treatment of Medulloblastoma, Fast Track Designation in August 2021 for the treatment of recurrent Sonic Hedgehog driven Medulloblastoma and an eIND for the treatment of a patient with severe COVID-19 in August 2020.

Antengene Announces NDA Approval by NMPA for XPOVIO®, China’s First XPO1 inhibitor, for the Treatment of Adults with Relapsed or Refractory Multiple Myeloma

On December 17, 2021 Antengene Corporation Limited (the "Company" or "Antengene") reported that ATG-010 (selinexor, brand name: XPOVIO), a first-in-class, oral Selective Inhibitor of Nuclear Export (SINE) compound has received conditional approval for marketing by the National Medical Products Administration (the "NMPA"), applicable in combination with dexamethasone for the treatment of adults with relapsed or refractory multiple myeloma (RRMM) who have received prior therapy including a proteasome inhibitor, an immunomodulatory agent and an anti-CD38 monoclonal antibody. XPOVIO is China’s first approved XPO1 inhibitor (Press release, Antengene, DEC 17, 2021, View Source [SID1234597380]). The conditional approval of XPOVIO was based on the global Phase 2 STORM trial as well as the positive results from the Phase 2 MARCH trial. The ongoing, randomized Phase 3 BENCH study in China, evaluating selinexor in combination with bortezomib and low-dose dexamethasone for patients with multiple myeloma as early as first relapse, will serve as the confirmatory trial.

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Antengene will host conference calls for investors on December 20, 2021 (China Standard Time).

Dr. Jay Mei, M.D., Ph.D., Chairman and Chief Executive Officer of Antengene commented, "Our mission is to bring first-in-class/best-in-class medicines to the market for patients with cancer and other life-threatening diseases. I am pleased that XPOVIO is Antengene’s first product to be approved in China and the first and only XPO1 inhibitor on the market in China."

Dr. Mei continued, "We believe the approval of XPOVIO will bring important clinical benefits to Chinese patients with refractory or relapsed multiple myeloma. XPOVIO is approved in three indications in the U.S., including in second line multiple myeloma, and the product has been widely adopted into practice guidelines by major oncology networks including the Chinese Society of Clinical Oncology (CSCO) in China, the National Comprehensive Cancer Network (NCCN) in the U.S. and the European Society for Medical Oncology (ESMO) (Free ESMO Whitepaper) in the EU. These are very positive steps to enabling product acceptance and adoption."

Dr. Kevin Lynch, M.D., Chief Medical Officer of Antengene commented, "With XPOVIO’s approval in China, Antengene can offer a new, much needed therapeutic option to Chinese patients with RRMM. We believe selinexor has broad potential in oncology and are committed to advance further development of the product though our program of 10 clinical studies in China. These include the confirmatory Phase 3 BENCH study for multiple myeloma patients after at least one prior therapy, as well as studies in other oncology indications that have strong relevance for patients in China."

Dr. Lynch continued, "Antengene would like to thank all of the patients and investigators involved in the clinical study and the NMPA for their support during the priority review. Together, we are aiming to improve the care and lives of people with cancer in China and around the world."

Antengene Conference Call Details

Antengene management will hold conference calls on Monday, December 20, 2021 to discuss the approval in China of ATG-010/XPOVIO:

Pivotal STORM and MARCH Trials

The conditional approval of XPOVIO was based on results from the global Phase 2 STORM trial as well as the Phase 2 MARCH trial in China evaluating the efficacy and safety of selinexor plus dexamethasone in 82 patients with relapsed/refractory multiple myeloma (RRMM).

Results of the STORM trial showed that the overall response rate (ORR), the primary endpoint, as assessed by an Independent Review Committee (IRC), based on the International Myeloma Working Group (IMWG) Uniform Response Criteria, was 25.3% for the prespecified subgroup of 83 patients whose disease was refractory to bortezomib, carfilzomib, lenalidomide, pomalidomide, and daratumumab.

Results of the MARCH trial showed that the efficacy and safety in Chinese patients whose disease was refractory to both lenalidomide and bortezomib, as well as the last line of therapy (with some also refractory to anti-CD38 monoclonal antibody), were generally consistent with that seen in the global study. The overall response rate, the primary endpoint, as assessed by an Independent Review Committee (IRC), was 29.3%, for all treated patients in the MARCH trial and 25% for patients refractory to at least a proteasome inhibitor, an immunomodulatory agent and an anti-CD38 antibody.

The ongoing, randomized Phase 3 BENCH study evaluating selinexor in combination with bortezomib and low-dose dexamethasone will serve as the confirmatory trial.

About the SINE Compounds

SINE (Selective Inhibitor of Nuclear Export) compounds are inhibitors of the major nuclear export protein Exportin 1 (XPO1). Currently, there are three oral SINE compounds, ATG-010 (selinexor), ATG-016 (eltanexor), and ATG-527 (verdinexor), under clinical development. Antengene has an exclusive license from Karyopharm Therapeutics Inc. ("Karyopharm") to these compounds in certain APAC markets.

About ATG-010/Selinexor/ XPOVIO

Selinexor is the first and only oral XPO1 inhibitor approved by the U.S. Food and Drug Administration (FDA). By blocking the nuclear export protein XPO1, selinexor can promote the intranuclear accumulation and activation of tumor suppressor proteins and growth regulating proteins, and down-regulate the levels of multiple oncogenic proteins. This induces apoptosis without affecting normal cells. Due to its novel mechanism of action, selinexor is being evaluated for use in multiple combination regimens to improve treatment efficacy.

Selinexor is approved by the U.S. FDA for the treatment of relapsed/refractory multiple myeloma (RRMM), second line multiple myeloma and relapsed/refractory diffuse large B–cell lymphoma.

Antengene obtained approval of selinexor in South Korea through a priority review process prior to the current approval by NMPA in China. Antengene is conducting 10 studies with selinexor in mainland China (3 in collaboration with Karyopharm) for relapsed/refractory/advanced hematological malignancies and advanced solid tumors.

Transgene Announces Investor Meetings for January 2022

On December 17, 2021 TRANSGENE (Paris:TNG) reported that Management will participate in the upcoming investor events set out below (Press release, Transgene, DEC 17, 2021, View Source [SID1234597379]).

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Transgene will meet institutional investors at the 11th Annual LifeSci Advisors Corporate Access Event (virtual event) from January 5th to 7th, 2022.

The Company will also attend:

25th ODDO BHF Digital Forum (virtual): January 6, 7, 10 and 11, 2022
Biotech Showcase Investor Conference (virtual):
January 10 to 12, 2022, in conjunction with the J.P. Morgan Healthcare conference
January 17 to 19, 2022
Biomed Event (Paris): January 26, 2022

TEPMETKO® (tepotinib) Receives Positive CHMP Opinion for Patients with Advanced NSCLC with METex14 Skipping Alterations

On December 17, 2021 EMD Serono, the Healthcare business sector of Merck KGaA, Darmstadt, Germany in the US and Canada, reported that the Committee for Medicinal Products for Human Use (CHMP) of the European Medicines Agency (EMA) adopted a positive opinion recommending approval of once-daily oral TEPMETKO (tepotinib) as monotherapy for the treatment of adult patients with advanced non-small cell lung cancer (NSCLC) harboring alterations leading to mesenchymal-epithelial transition factor gene exon 14 (METex14) skipping, who require systemic therapy following prior treatment with immunotherapy and/or platinum-based chemotherapy (Press release, EMD Serono, DEC 17, 2021, View Source [SID1234597378]). The CHMP positive opinion will now be reviewed by the European Commission (EC), with a decision expected in the first quarter of 2022.

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"TEPMETKO has demonstrated important clinical benefits and a manageable safety profile in the treatment of this aggressive form of lung cancer, and has the potential to advance the treatment of this type of tumor," said Danny Bar-Zohar, M.D., Global Head of Development for the Healthcare business of Merck KGaA, Darmstadt, Germany. "We look forward to the European Commission decision, and bringing the first oral MET inhibitor in NSCLC to patients in Europe."

The positive opinion is based on results from the pivotal Phase II VISION study evaluating TEPMETKO as a once-daily oral monotherapy treatment for patients with advanced NSCLC with METex14 skipping alterations. Data from the primary analysis of the VISION study were previously published in The New England Journal of Medicine, and showed TEPMETKO demonstrated consistent and durable responses in both treatment naïve and previously treated adult patients with advanced NSCLC harboring alterations leading to METex14 skipping. Additional data from the VISION study presented at IASLC 2021 World Conference on Lung Cancer include results for 123 patients from Cohort C with follow-up of at least 3 months from the start of treatment and provide additional evidence supporting the clinically meaningful responses demonstrated in the primary analysis.1

In Europe, lung cancer is estimated to be the second most common cancer, and the leading cause of cancer-related mortality, responsible for 388,000 deaths in 2018.2 Alterations of the MET signaling pathway, including METex14 skipping alterations, are found in 3% to 4% of NSCLC cases, and are associated with having advanced disease and poor prognosis.1,3-6

TEPMETKO was the first oral MET inhibitor to receive a regulatory approval anywhere in the world for the treatment of advanced NSCLC harboring MET gene alterations, with its approval in Japan in March 2020. In February 2021, the U.S. Food and Drug Administration granted accelerated approval to TEPMETKO, making it the first and only once-daily oral MET inhibitor approved for patients in the U.S. with metastatic NSCLC with METex14 skipping alterations. This indication is approved under accelerated approval based on overall response rate and duration of response. Continued approval for this indication may be contingent upon verification and description of clinical benefit in confirmatory trials. Tepotinib is available in a number of countries, and under review by various other regulatory authorities globally. To meet an urgent clinical need, tepotinib is also available in a pilot zone of China in line with the government policy to drive early access for innovative medicines approved outside of China.

About VISION Study
VISION (NCT02864992) is an ongoing pivotal Phase II, multicenter, multi-cohort, single-arm, non-randomized, open-label study investigating tepotinib as monotherapy in 275 patients with a median age of 72.6 years with advanced or metastatic NSCLC with METex14 skipping alterations.

About TEPMETKO (tepotinib)
TEPMETKO is an oral MET inhibitor that inhibits the oncogenic MET receptor signaling caused by MET (gene) alterations. Discovered and developed in-house at Merck KGaA, Darmstadt, Germany, TEPMETKO has a highly selective mechanism of action, with the potential to improve outcomes in aggressive tumors that have a poor prognosis and harbor these specific alterations.

Merck KGaA, Darmstadt, Germany is also investigating the potential role of tepotinib in treating patients with NSCLC and acquired resistance due to MET amplification in the Phase II INSIGHT 2 study of tepotinib in combination with osimertinib in MET amplified, advanced or metastatic NSCLC harboring activating EGFR mutations that has progressed following first-line treatment with osimertinib.

Important Safety Information from the US FDA-Approved Label
TEPMETKO can cause interstitial lung disease (ILD)/pneumonitis, which can be fatal. Monitor patients for new or worsening pulmonary symptoms indicative of ILD/pneumonitis (eg, dyspnea, cough, fever). Immediately withhold TEPMETKO in patients with suspected ILD/pneumonitis and permanently discontinue if no other potential causes of ILD/pneumonitis are identified. ILD/pneumonitis occurred in 2.2% of patients treated with TEPMETKO, with one patient experiencing a Grade 3 or higher event; this event resulted in death.

TEPMETKO can cause hepatotoxicity, which can be fatal. Monitor liver function tests (including ALT, AST, and total bilirubin) prior to the start of TEPMETKO, every 2 weeks during the first 3 months of treatment, then once a month or as clinically indicated, with more frequent testing in patients who develop increased transaminases or total bilirubin. Based on the severity of the adverse reaction, withhold, dose reduce, or permanently discontinue TEPMETKO. Increased alanine aminotransferase (ALT)/increased aspartate aminotransferase (AST) occurred in 13% of patients treated with TEPMETKO. Grade 3 or 4 increased ALT/AST occurred in 4.2% of patients. A fatal adverse reaction of hepatic failure occurred in one patient (0.2%). The median time-to-onset of Grade 3 or higher increased ALT/AST was 30 days (range 1 to 178).

TEPMETKO can cause embryo-fetal toxicity. Based on findings in animal studies and its mechanism of action, TEPMETKO can cause fetal harm when administered to a pregnant woman. Advise pregnant women of the potential risk to a fetus. Advise females of reproductive potential or males with female partners of reproductive potential to use effective contraception during treatment with TEPMETKO and for one week after the final dose.

Avoid concomitant use of TEPMETKO with dual strong CYP3A inhibitors and P-gp inhibitors and strong CYP3A inducers. Avoid concomitant use of TEPMETKO with certain P-gp substrates where minimal concentration changes may lead to serious or life-threatening toxicities. If concomitant use is unavoidable, reduce the P-gp substrate dosage if recommended in its approved product labeling.

Fatal adverse reactions occurred in one patient (0.4%) due to pneumonitis, one patient (0.4%) due to hepatic failure, and one patient (0.4%) due to dyspnea from fluid overload.

Serious adverse reactions occurred in 45% of patients who received TEPMETKO. Serious adverse reactions in >2% of patients included pleural effusion (7%), pneumonia (5%), edema (3.9%), dyspnea (3.9%), general health deterioration (3.5%), pulmonary embolism (2%), and musculoskeletal pain (2%).

The most common adverse reactions (≥20%) in patients who received TEPMETKO were edema, fatigue, nausea, diarrhea, musculoskeletal pain, and dyspnea.

Clinically relevant adverse reactions in <10% of patients who received TEPMETKO included ILD/pneumonitis, rash, fever, dizziness, pruritus, and headache.

Selected laboratory abnormalities (≥20%) from baseline in patients receiving TEPMETKO in descending order were: decreased albumin (76%), increased creatinine (55%), increased alkaline phosphatase (ALP) (50%), decreased lymphocytes (48%), increased alanine aminotransferase (ALT) (44%), increased aspartate aminotransferase (AST) (35%), decreased sodium (31%), decreased hemoglobin (27%), increased potassium (25%), increased gamma-glutamyltransferase (GGT) (24%), increased amylase (23%), and decreased leukocytes (23%).

The most common Grade 3 to 4 laboratory abnormalities (≥2%) in descending order were: decreased lymphocytes (11%), decreased albumin (9%), decreased sodium (8%), increased GGT (5%), increased amylase (4.6%), increased ALT (4.1%), increased AST (2.5%), and decreased hemoglobin (2%).

A clinically relevant laboratory abnormality in <20% of patients who received TEPMETKO was increased lipase in 18% of patients, including 3.7% Grades 3 to 4.

For more information about TEPMETKO, please see full Prescribing Information, and visit www.TEPMETKO.com.

Commitment to Cancer
EMD Serono is a science-led organization dedicated to delivering transformative medicines with the goal of making a meaningful difference in the lives of people affected by cancer. Our oncology research efforts aim to leverage our synergistic portfolio in oncogenic pathways, immuno-oncology, and DNA Damage Response (DDR) to tackle challenging tumor types in gastrointestinal, genitourinary, and thoracic cancers. Our curiosity drives our pursuit of treatments for even the most complex cancers, as we work to illuminate a path to scientific breakthroughs that transform patient outcomes. Learn more at www.emdseronooncology.com. Follow us on Twitter: @EMDOncologyUS and LinkedIn: EMD Serono, Inc.

References
1. Felip E, et al. WCLC 2021. Poster 170.
2. Ferlay J, et al. Eur J Cancer. 2018;103:356–387.
3. Reungwetwattana T, et al. Lung Cancer 2017;103:27-37.
4. Wolf J, et al. EORTC/NCI/AACR 2018. Poster 403.
5. Schrock AB, et al. J Thorac Oncol. 2016;11:1493–1502.
6. Tong JH, et al. Clin Cancer Res. 2016;22:3048–3056.8.

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Vincerx Pharma Announces First Patient Dosed in Phase 1 Dose-Escalation Study of VIP152 in Relapsed or Refractory Chronic Lymphocytic Leukemia or Richter Syndrome

On December 17, 2021 Vincerx Pharma, Inc. (Nasdaq: VINC), a biopharmaceutical company aspiring to address the unmet medical needs of patients with cancer through paradigm-shifting therapeutics, reported that the first patient has been dosed in the Company’s Phase 1 dose-escalation study of VIP152 in relapsed or refractory (R/R) chronic lymphocytic leukemia (CLL) or Richter Syndrome (RS) (Press release, Vincerx Pharma, DEC 17, 2021, View Source [SID1234597377]).

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"The dosing of the first patient in Vincerx’s Phase 1 dose-escalation study of VIP152 in R/R CLL or RS marks the initiation of the second Vincerx-sponsored clinical trial this year, less than one year after becoming a publicly-listed company," said Ahmed Hamdy M.D., Chief Executive Officer of Vincerx. "Our data recently disclosed through an oral presentation at ASH (Free ASH Whitepaper) demonstrated increased selectivity and potency of VIP152 when compared with other CDK9 inhibitors currently in development, leading to cytotoxic activity in primary CLL samples as well as improved survival in a mouse model of CLL. With this compelling preclinical proof-of-concept data in hand, we are focused on advancing VIP152 across challenging indications like R/R CLL and RS, where targeted CDK9 inhibition has the potential to bring meaningful patient benefit. We are continuing our momentum in the clinic and remain on-track to initiate Phase 2 studies of VIP152 in the second half of 2022."

The Phase 1 study will evaluate VIP152 in patients with relapsed/refractory CLL who have failed a Bruton tyrosine kinase inhibitor (BTKi) and venetoclax and in patients with RS who have relapsed after, or been refractory to, at least one prior line of therapy for DLBCL and have MYC overexpression/amplification/translocation. A dose-escalation arm will be performed in R/R CLL before enrolling 20 additional patients in each of the CLL and RS cohorts.

The Phase 1 dose-escalation in CLL and RS builds upon Vincerx’s ongoing first-in-human (FIH) study (NCT04978779) in patients with advanced cancer, which consists of two expansion arms. Arm 1 will enroll up to 40 patients with relapsed/refractory aggressive lymphoma, including DLBCL, transformed follicular lymphoma, patients with mantle cell lymphoma who have failed a BTKi, as well as patients with any other type of lymphoma characterized by a MYC aberration. Arm 2 will enroll up to 40 patients with advanced solid tumors, including patients with ovarian cancer, triple negative breast cancer, castration-resistant neuroendocrine prostate cancer, and any other solid tumor with MYC aberration. All patients must have confirmed MYC overexpression or translocation.