CurrentNeighborhood Health Plan of Rhode IslandPolicy N/A

Xermelo (telotristat ethyl) usage for carcinoid syndrome in neuroendocrine tumors

Defines accepted indications, continuation and exclusion criteria, contraindications, coding, and applicable lines of business for Xermelo (telotristat ethyl). Applies to medication requests processed by Evolent for Neighborhood Health Plan of Rhode Island across listed lines of business.

Policy Summary

PayerNeighborhood Health Plan of Rhode Island

PolicyXermelo (telotristat ethyl) usage for carcinoid syndrome in neuroendocrine tumors

Policy CodePolicy N/A

Change TypeRevised (template conversion; verbiage updates)

Effective Date

Next Review Date

Key ActionUse must be supported by FDA labeling, CMS-recognized compendia, NCCN or ASCO guidelines, or peer-reviewed literature meeting CMS Chapter 15 requirements and include documentation addressing representativeness of patient characteristics, therapy regimens, outcomes, and study design.

POLICY UPDATE CHANGES

Converted to new Evolent guideline template in June 2025; replaced UM ONC 1303 Xermelo policy.

June 2024 update: Updated NCH verbiage to Evolent.

1Covered Indications (neuroendocrine tumor with carcinoid syndrome)

2Hard dosing limits (single dose, monthly)

1Contraindications listed

Coverage Summary

Coverage stance: covered_with_criteria for Xermelo (telotristat ethyl) for the treatment of metastatic neuroendocrine tumor with carcinoid syndrome when all policy criteria are met, including use in combination with a somatostatin analog and evidence of intractable diarrhea despite appropriate somatostatin analog therapy.

Requests for Xermelo are processed by Evolent and must meet the policy's authorization criteria or be otherwise authorized by Evolent; medications not authorized by Evolent may be deemed not approvable and not reimbursable. Applicable lines of business include CHIP, Commercial, Exchange/Marketplace, Medicaid, and Medicare Advantage.

Initial / Continued Use for Neuroendocrine Tumors (Carcinoid Syndrome)

Covered when ALL of the following are met:

ALL of the following

Metastatic neuroendocrine tumor/carcinoma: Member has metastatic neuroendocrine tumor/carcinoma
Use with somatostatin analog: Xermelo (telotristat ethyl) will be used in combination with a somatostatin analog (e.g., Sandostatin LAR (octreotide) or Somatuline Depot (lanreotide))
Intractable diarrhea: Member has intractable diarrhea associated with carcinoid syndrome
Minimum somatostatin dosing: Member is currently receiving the minimum dose of Octreotide LAR 30 mg every 4 weeks or Lanreotide Depot 120 mg every 4 weeksOctreotide LAR 30 mg q4 weeks; Lanreotide Depot 120 mg q4 weeks
Inadequate control with somatostatin analog: Member has experienced inadequate control of his/her diarrhea with somatostatin analog therapy

Continuation Therapy Criteria

Continuation Requests Exemption from Not-Approvable Medication Policy

Continuation requests for a not-approvable medication shall be exempt provided ALL of the following are met:

ALL of the following

No disease progression: Member has not experienced disease progression on the requested medication
Recent use without lapse: Requested medication was used within the last year without a lapse of more than 30 days of having an active authorizationno lapse > 30 days within last year
No additional medications: Additional medication(s) are not being added to the continuation request

Contraindications / Warnings

Treatment is not appropriate when the following contraindication is present:

Hypersensitivity to telotristat ethyl or components: Hypersensitivity (e.g., angioedema, rash, pruritus) to telotristat ethyl or any component of the formulation

Exclusions, Contraindications, and Dosing Limits

Exclusion Criteria (Not Approvable)

Treatment is excluded / not approvable when ANY of the following are present:

ANY of the following

Disease/symptom progression after trial: Disease/symptom progression defined as a lack of improvement in symptoms of diarrhea associated with carcinoid syndrome, following 3 months on the maximum tolerated dose of Xermelo (telotristat ethyl)3 months on maximum tolerated dose
Single dose exceeds limit: Dosing exceeds single dose limit of Xermelo (telotristat ethyl) 250 mgMaximum single dose 250 mg
Single dose limit specified
Monthly quantity exceeds limit: Treatment exceeds the maximum limit of 84 (250 mg) tablets/monthMaximum monthly quantity 84 tablets (250 mg) per month
Monthly maximum specified
Investigational/off-label evidence insufficiency
- Representativeness of patient and cancer characteristics: Whether the clinical characteristics of the patient and the cancer are adequately represented in the published evidence
- Representativeness of therapies in evidence: Whether the administered chemotherapy/biologic therapy/immune therapy/targeted therapy/other oncologic therapy regimen is adequately represented in the published evidence
- Clinically meaningful outcomes: Whether the reported study outcomes represent clinically meaningful outcomes experienced by patients (ASCO recommended: e.g., Hazard Ratio < 0.80; OS/PFS benefit at least 3 months)OS/PFS benefit at least 3 months; HR < 0.80
- Appropriate experimental design: Whether the experimental design is appropriate to address the investigative question

Numeric Thresholds

Maximum single dose250 mg

Maximum monthly quantity84 tablets (250 mg) per month

Trial for progression assessment3 months on maximum tolerated dose

Minimum somatostatin analog dosingOctreotide LAR 30 mg q4 weeks or Lanreotide Depot 120 mg q4 weeks

Continuation lapse allowanceno lapse > 30 days within last year

Provider Actions & Authorization Requirements

Prior Authorization

Medication requests processed by Evolent

All medication requests for Xermelo (telotristat ethyl) must be processed by Evolent. Medications not authorized by Evolent may be deemed not approvable and therefore not reimbursable.

J8499

Documentation Required

Supportive evidence required for off-label uses

Off-label uses must be supported by FDA-approved product labeling, CMS-recognized compendia, NCCN or ASCO clinical guidelines, or peer-reviewed literature that meets the requirements of the CMS Medicare Benefit Policy Manual Chapter 15. Documentation should address whether the patient and cancer characteristics are adequately represented in the evidence, whether the therapy regimens are represented, whether reported study outcomes are clinically meaningful, and whether the experimental design is appropriate.

Denial Risk

Exclusion criteria will lead to non-approval

Claims will be non-approvable when exclusion criteria are met, including exceeding dosing limits, disease progression after an assessment period, or insufficient evidence for off-label use.

Single-dose limit: 250 mg
Monthly maximum: 84 tablets (250 mg) per month
Disease progression defined as lack of improvement after 3 months on maximum tolerated dose

Background & Evidence

This policy defines accepted indications for Xermelo (telotristat ethyl), including FDA‑approved and acceptable off‑label uses when supported by CMS‑recognized compendia, NCCN or ASCO guidelines, or peer‑reviewed literature meeting CMS Medicare Benefit Policy Manual Chapter 15 requirements.

Evolent is responsible for processing all medication requests from network ordering providers and enforces authorization requirements; medications not authorized by Evolent may be deemed not approvable and not reimbursable.

Continuation requests for a not‑approvable medication may be exempt if the member has not experienced disease progression on the medication, the medication was used within the last year without a lapse of more than 30 days in active authorization, and no additional medications are being added to the continuation request.

This policy applies to medication requests processed by Evolent for Neighborhood Health Plan of Rhode Island across the listed lines of business.

Primary prescribing information: Xermelo prescribing information, TerSera Therapeutics LLC, 2022.

Guideline compendia cited: NCCN, ASCO, CMS‑recognized compendia, Clinical Pharmacology, Micromedex.

Definitions

Intractable diarrhea associated with carcinoid syndrome: Diarrhea related to carcinoid syndrome not adequately controlled with somatostatin analog therapy (as specified in the policy criteria).

Continuation exemption: Continuation requests for not‑approvable medications may be exempt if the member has no disease progression on the medication, the medication was used within the last year without a lapse of more than 30 days in active authorization, and no additional medications are being added to the continuation request.