ESMO Virtual Congress 2020: Phase 2 Study of the Oral HIF-2α Inhibitor MK-6482 for Von Hippel-Lind Disease–Associated Clear Cell Renal Cell Carcinoma

(UroToday.com) Von-Hippel Lindau (VHL) disease is an autosomal dominant hereditary disorder, which harbors an increased risk of developing renal cell carcinoma (RCC), CNS, and retinal hemangioblastomas, pancreatic neuroendocrine tumors, and other lesions. RCC occurs in 25-60% of individuals and is a key cause of morbidity and mortality. Surgery is generally considered when the largest renal tumor reaches approximately 3 cm. The goal of surgery is to decrease the risk of metastases. Unfortunately, tumors will continue to recur, and repeated surgeries are required to control other VHL disease manifestations. Despite aggressive treatment, life expectancy is shortened in patients with VHL.


VHL inactivation results in aberrant stabilization and accumulation of HIF-2α (Figure 1). This leads to subsequent constitutive activation of genes that drive tumor growth. MK-6482 is a potent, selective, small molecule, which is a HIF-2α inhibitor and has shown favorable safety and antitumor activity in advanced RCC.1

Figure 1 – HIF-2α drives tumor growth in VHL-RCC:

ESMO_HIF-2α.png



In the presented study (figure 2), patients with a confirmed diagnosis of VHL disease, with at least one RCC non-metastatic tumor and no previous systemic or anticancer treatments, were treated with 120 mg once daily of MK-6482 for a duration of at least 12 weeks. Tumor evaluation was performed 12 weeks thereafter. The primary objective was to evaluate the efficacy of MK-6482 for the treatment of VHL disease-associated RCC, and the primary endpoint included objective response rates (ORR).

Figure 2 – Study design:

ESMO_Study_design.png



A total of 61 patients were accrued to the study with their baseline characteristics shown in figure 3. Out of the 61 patients, 56 (91.8%) patients are still undergoing treatment with a minimum of 60 weeks’ follow-up.

Figure 3 – Patient baseline characteristics:

ESMO_VHL.png

The overall response rate in target RCC lesions was 36.1% (95% CI 24.2-49.4), with 91.8% of patients demonstrating a decrease in the size of the target lesion (Figure 4).

Figure 4 – Response in target RCC lesions by independent central review:

ESMO_RCC_lesions.png

The responses were durable with a median time to response of 31.1 weeks (range 11.9-62.3 weeks). The median duration of response has not been reached up until the time of this presentation. The progression-free survival (PFS) rate at 52 weeks was 98.3% (figure 5).

Figure 5 – Duration of treatment:

ESMO_Duration_of_treatment.png

Response rates were also seen in pancreatic lesions (63.9%, 95% CI 50.6-75.8), and CNS hemangioblastoma (30.2%, 95% CI 17.2-46.1). Improvement in retinal lesions was also evident by the central committee, with 93.8% (15 patients) showing improved and stable retinal lesions.

The adverse events profile is shown in figure 6, with 60 patients (98.4%) having a treatment-related adverse event (TRAEs). A total of 8 patients (13.1%) had a grade 3 TRAE, and no grade 4 or 5 events were reported. One patient (1.6%) discontinued treatment due to a treatment-related adverse event (grade 1 dizziness). Anemia was the most common adverse effect (83.6% grade 1 or 2) but was amenable to medical management.

Figure 6 – Treatment-related adverse events:

ESMO_TRAEs.png

Concluding his presentation, Dr. Ramaprasad stated that promising clinical activity was observed with MK-6482 in treatment-naïve patients with VHL-associated RCC. Importantly, clinical activity was also observed in non-RCC lesions. MK-6482 was shown to be well-tolerated and had a favorable safety profile. MK-6482 had demonstrated durable efficacy in patients with VHL disease-associated with RCC and non-RCC lesions. 

Presented by: Ramaprasad Srinivasan MD, PhD, Head, Molecular Cancer Therapeutics Section, National Cancer Institute, Center for Cancer Research, Bethesda, MD

Written by: Hanan Goldberg, MD, MSc., Assistant Professor of Urology, SUNY Upstate Medical University, Syracuse, NY, USA @GoldbergHanan at the 2020 European Society for Medical Oncology Virtual Congress (#ESMO20), September 19th-September 21st, 2020.

References:

  1. Choueiri TK, Plimack ER, Bauer TM, et al. Phase I/II study of the oral HIF-2 α inhibitor MK-6482 in patients with advanced clear cell renal cell carcinoma (RCC). Journal of Clinical Oncology 2020; 38(6_suppl): 611-.
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