WHA
 

Welireg (belzutifan)

Indications for Prior Authorization

Welireg (belzutifan)
  • For diagnosis of Von Hippel-Lindau (VHL) disease
    Indicated for treatment of adult patients with von Hippel-Lindau (VHL) disease who require therapy for associated renal cell carcinoma (RCC), central nervous system (CNS) hemangioblastomas, or pancreatic neuroendocrine tumors (pNET), not requiring immediate surgery

  • For diagnosis of Renal Cell Carcinoma with a Clear Cell Component (ccRCC)
    1. Indicated as monotherapy for treatment of adult patients with advanced ccRCC following a programmed death receptor-1 (PD-1) or programmed death ligand 1 (PD-L1) inhibitor and a vascular endothelial growth factor tyrosine kinase inhibitor (VEGF-TKI); 2. Indicated in combination with pembrolizumab or pembrolizumab and berahyaluronidase alfa-pmph, for the adjuvant treatment of adult patients with ccRCC at intermediate-high or high risk of recurrence following nephrectomy, or following nephrectomy and resection of metastatic lesions.

  • For diagnosis of Pheochromocytoma or Paraganglioma (PPGL)
    Indicated for the treatment of adult and pediatric patients 12 years and older with locally advanced, unresectable, or metastatic pheochromocytoma or paraganglioma (PPGL)

Criteria

Welireg

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of von Hippel-Lindau (VHL) disease

  • Diagnosis of von Hippel-Lindau (VHL) disease [A, 1]
    • AND
  • Patient requires therapy for one of the following [A, 1] :
    • Renal cell carcinoma (RCC)
    • Central nervous system (CNS) hemangioblastoma
    • Pancreatic neuroendocrine tumor (pNET)
    AND
  • Patient does not require immediate surgery [A,1]
Welireg

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of von Hippel-Lindau (VHL) disease

  • Patient does not show evidence of progressive disease while on therapy [A, 1]
Welireg

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Renal Cell Carcinoma with a Clear Cell Component (ccRCC)

  • Diagnosis of renal cell carcinoma with a clear cell component (ccRCC)
    • AND
  • One of the following:
    • Both of the following:
      • Disease is advanced
        • AND
      • Disease has progressed after treatment with both of the following:
        • One of the following:
          • Programmed death receptor-1 (PD-1) inhibitor [e.g., Keytruda (pembrolizumab), Opdivo (nivolumab)]
          • Programmed death-ligand 1 (PD-L1) inhibitor [e.g., Bavencio (avelumab), Imfinzi (durvalumab)]
          AND
        • Vascular endothelial growth factor tyrosine kinase inhibitor (VEGF-TKI) [e.g., Votrient (pazopanib), Inlyta (axitinib)]
      OR
    • Both of the following:
      • Used in combination with one of the following:
        • pembrolizumab
        • pembrolizumab and berahyaluronidase alfa-pmph
        AND
      • One of the following:
        • Patient is at intermediate-high or high risk of recurrence following nephrectomy
        • Used following nephrectomy and resection of metastatic lesions
Welireg

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Renal Cell Carcinoma with a Clear Cell Component (ccRCC)

  • Patient does not show evidence of progressive disease while on therapy
Welireg

Prior Authorization (Initial Authorization)

Length of Approval: 12 Month(s)
For diagnosis of Pheochromocytoma or Paraganglioma (PPGL)

  • Diagnosis of Pheochromocytoma or Paraganglioma (PPGL)
    • AND
  • Disease is one of the following:
    • Locally advanced
    • Unresectable
    • Metastatic
    AND
  • Patient is 12 years of age or older
Welireg

Prior Authorization (Reauthorization)

Length of Approval: 12 Month(s)
For diagnosis of Pheochromocytoma or Paraganglioma (PPGL)

  • Patient does not show evidence of progressive disease while on therapy

  • Guideline ID
    GL-658188

  • Formulary
    Premium

  • Effective date
    Oct 1, 2026

  • P&T approval date
    Nov 18, 2021

P & T Revisions

2026-07-17, 2025-12-02, 2025-06-04, 2024-10-01, 2024-01-26, 2023-09-11, 2023-07-03, 2022-09-11, 2021-10-09

  1. Welireg [Prescribing Information]. Merck & Co, Inc. Rathway, NJ. June 2026.
  2. NCCN Clinical Practice Guidelines in Oncology. Kidney Cancer V1.2026. Available at https://www.nccn.org/professionals/physician_gls/pdf/kidney.pdf. Accessed July 17, 2026.
  1. The efficacy of WELIREG was evaluated in Study 004 (NCT03401788), an open-label clinical trial in 61 patients with VHL-associated RCC diagnosed based on a VHL germline alteration and with at least one measurable solid tumor localized to the kidney as defined by response evaluation criteria in solid tumors (RECIST) v1.1. Enrolled patients had other VHL-associated tumors including CNS hemangioblastomas and pNET. CNS hemangioblastomas and pNET in these patients were diagnosed based on the presence of at least one measurable solid tumor in brain/spine or pancreas, respectively, as defined by RECIST v1.1 and identified by IRC. The study excluded patients with metastatic disease. Patients received WELIREG 120 mg once daily until progression of disease or unacceptable toxicity. The study population characteristics were: median age 41 years [range 19-66 years], 3.3% age 65 or older; 53% male; 90% were White, 3.3% were Black or AfricanAmerican, 1.6% were Asian, and 1.6% were Native Hawaiian or other Pacific Islander; 82% had an ECOG PS of 0, 16% had an ECOG PS of 1, and 1.6% had an ECOG PS of 2; and 84% had VHL Type I Disease. The median diameter of RCC target lesions per central independent review committee (IRC) was 2.2 cm (range 1-6.1). Median time from initial radiographic diagnosis of VHL-associated RCC tumors that led to enrollment on Study 004 to the time of treatment with WELIREG was 17.9 months (range 2.8-96.7). Seventy- seven percent of patients had prior surgical procedures for RCC. The major efficacy endpoint for the treatment of VHL-associated RCC was overall response rate (ORR) measured by radiology assessment using RECIST v1.1 as assessed by IRC. Additional efficacy endpoints included duration of response (DoR), and time to response (TTR).
  • 2026-07-17: Added PA criteria for new indication in ccRCC as adjuvant therapy.
  • 2025-12-02: Updated indication for RCC. Updated criteria verbiage to align with indication.
  • 2025-06-04: Added criteria for new indication of Pheochromocytoma or Paraganglioma (PPGL)
  • 2024-10-01: 2024 Annual Review. Background updates only.
  • 2024-01-26: update guideline
  • 2023-09-11: 2023 Annual Review
  • 2023-07-03: Removed specialist requirement
  • 2022-09-11: 2022 Annual Review
  • 2021-10-09: New UM PA criteria