Overview

This document addresses the use of Fyarro (sirolimus protein bound) for use in the treatment of adults with locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor (PEComa). Fyarro is a nanoparticle albumin-bound mTOR inhibitor that is given intravenously. It is the first agent to received FDA approval for PEComa. Sirolimus oral, everolimus oral, and temsirolimus intravenous are mTOR inhibitors recommended by the National Comprehensive Cancer Network (NCCN) as single-agent therapies for the treatment of PEComa. These agents use is supported by case studies and retrospective analyses and is considered off-label.

Definitions and Measures

• Malignant: Cancerous. Malignant cells can invade and destroy nearby tissue and spread to other parts of the body.
• Metastasis: The spread of cancer from one part of the body to another; a metastatic tumor contains cells that are like those in the original (primary) tumor and have spread.
• Unresectable: Unable to be removed with surgery.

Clinical Criteria

When a drug is being reviewed for coverage under a member’s medical benefit plan or is otherwise subject to prior authorization, the review will determine whether the drug meets any applicable medical necessity requirements for the intended/prescribed purpose.

Fyarro (sirolimus protein bound)

Requests for Fyarro (sirolimus protein bound) may be approved when the following criteria are met:

1. Individual is 18 years of age or older; AND
2. Using as a single agent; AND
3. Individual is using in one of the following ways:
   • A. In soft tissue sarcoma for the treatment of locally advanced unresectable or metastatic malignant perivascular epithelioid cell tumor (PEComa) (Label, NCCN 2A); OR
   • B. In uterine sarcoma for the treatment of advanced, recurrent/metastatic or inoperable PEComa (NCCN 2A).

Requests for Fyarro may not be approved for any of the following:

• Individual has severe hepatic impairment; OR
• Individual has a history of severe hypersensitivity to sirolimus, other rapamycin derivatives, or albumin; OR
• When the above criteria are not met and for all other indications.

Coding

The following codes for treatments and procedures applicable to this document are included below for informational purposes. Inclusion or exclusion of a procedure, diagnosis or device code(s) does not constitute or imply member coverage or provider reimbursement policy. Please refer to the member's contract benefits in effect at the time of service to determine coverage or non-coverage of these services as it applies to an individual member.

1. HCPCS
   • J9331
2. ICD-10 Diagnosis
   • Injection, sirolimus protein-bound particles, 1 mg C49.4-C49.9 Malignant neoplasm of connective and soft tissue of abdomen

Document History

Revised: 11/19/2023