VIJOICE (alpelisib)

VIJOICE (alpelisib)

Self-Administration – oral

Diagnosis considered for coverage:

• Indicated for the treatment of adult and pediatric patients 2 years of age and older with severe manifestations of PIK3CA-Related Overgrowth Spectrum (PROS) who require systemic therapy
  • This indication is approved under accelerated approval based on response rate and duration of response. Continued approval for this indication may be contingent upon verification and description of clinical benefit in a confirmatory trial(s)

Coverage Criteria:

For diagnosis of PROS:

• Dose does not exceed Food and Drug Administration (FDA) labeled maximum dosing:
  • Patients 2-5 years of age: 50 mg once daily
  • Patients 6-17 years of age: 125 mg once daily
  • Adults: 250 mg once daily; AND
• Patient is 2 years of age or older; AND
• Diagnosis of PIK3CA-Related Overgrowth Spectrum (PROS); AND
• Documentation of mutation in the PIK3CA gene; AND
• Documentation of severe clinical manifestations (e.g., Congenital Lipomatous Overgrowth, Vascular malformations, Epidermal nevi, Scoliosis/skeletal and spinal [CLOVES], Facial Infiltrating Lipomatosis [FIL], Klippel-Trenaunay Syndrome [KTS], Megalencephaly-Capillary Malformation Polymicrogyria [MCAP]); AND
• Prescribed by or in consultation with a physician who specializes in the treatment of PROS 

Reauthorization Criteria:

For diagnosis of PROS:

• Dose does not exceed Food and Drug Administration (FDA) labeled maximum dosing:
  • Patients 2-5 years of age: 50 mg once daily
  • Patients 6-17 years of age: 125 mg once daily
  • Adults: 250 mg once daily; AND
• Documentation of positive clinical response to therapy (e.g., radiological response defined as a ≥ 20% reduction from baseline in the sum of target lesion volume); AND
• Prescribed by or in consultation with a physician who specializes in the treatment of PROS

Coverage Duration: 

• Initial: 6 months
• Reauthorization: 1 year

Authorization is not covered for the following:

The use of this drug for indications not listed in this policy does not meet the coverage criteria established by the Western Health Advantage (WHA) Pharmacy and Therapeutics (P&T) Committee.

Additional Information: 

• Recommended dose: 
  • Adults: 250 mg once daily
  • Pediatric patients (2 to less than 18 years of age): initial dose is 50 mg once daily. Consider a dose increase to 125 mg once daily in pediatric patients ≥ 6 years old for response optimization (clinical/radiological) after 24 weeks of treatment with Vijoice at 50 mg once daily. When a pediatric patient turns 18 years old, consider a gradual dose increase up to 250 mg
• Patients without any response assessment at Week 24 were considered non-responders
• Conditions associated with PIK3CA mutations include the following:
  • Capillary malformation of the lower lip, lymphatic malformation of the face and neck, asymmetry of the face and limbs, and partial or generalized overgrowth (CLAPO) syndrome
  • Congenital lipomatous overgrowth, vascular malformations, epidermal nevi, scoliosis/skeletal and spinal (CLOVES) syndrome
  • Epidermal nevus (EN), benign lichenoid keratosis (BLK), or seborrheic keratosis (SK)
  • Fibroadipose hyperplasia or overgrowth (FAO)
  • Fibroadipose vascular anomaly (FAVA)
  • Facial (fibroadipose) infiltrating lipomatosis (FIL)
  • Hemihyperplasia multiple lipomatosis (HHML)
  • Hemimegalencephaly (HME)/ Dysplastic megalencephaly (DMEG) / focal cortical dysplasia (FCD) type II
  • Isolated lymphatic malformation (ILM)
  • Klippel-Trenaunay Syndrome (KTS)
  • Macrodactyly
  • Megalencephaly-capillary malformation (MCAP or M-CM) 
  • Muscular hemihyperplasia (HH)

Policy Updates:

• 8/16/2022 – New policy approved by P&T

References:

• Vijoice Prescribing Information. Novartis Pharmaceuticals Corporation. East Hanover, New Jersey. April 2022.