Navigating Insurance

Getting an EHE diagnosis is overwhelming enough without also worrying about insurance coverage or getting access to the care you need. Because EHE is an ultra-rare cancer, health plans are often unfamiliar with the disease. That unfamiliarity may delay diagnostics, access to expert care, and treatment.

To help you navigate, we have gathered general information about insurance approvals, what to do if you are denied coverage, and how to advocate for your care. This information is based primarily on the United States insurance system. If you are outside the US, refer to your national health system for approvals and appeals processes.

This information is intended for general purposes and is not provided as healthcare, legal, or financial advice. Refer to your insurance or healthcare coverage provider for information, rules, and guidelines specific to your situation.

Understanding the Basics

Three foundational concepts are worth understanding before a coverage question arises.

1. How you get insurance shapes everything that follows. In the US, most people are covered through an employer-sponsored plan, a plan purchased through the state or federal Health Insurance Marketplace, or a government program such as Medicare, Medicaid, or TRICARE. Each of these has different requirements.
2. "Prior authorization" is a common hurdle. Many scans, procedures, and treatments require insurance approval before your doctor can move forward. Talk with your care team to understand which tests, procedures, or treatments may require prior authorization.
3. Get familiar with your insurance plan before you need it. Understand your deductibles, your out-of-pocket costs, and how your plan considers out-of-network care.

Helpful tools from Triage Cancer, a nonprofit that provides free education on the legal and financial issues that follow a cancer diagnosis:

• Quick Guide to Health Insurance Basics
• Medicare Materials & Resources and Medicaid Materials & Resources
• Checklist: Using Health Insurance, a plain-language walkthrough of networks, prior authorization, formulary exceptions, and appeals
• Cancer Finances, an interactive toolkit for navigating insurance and finances after a cancer diagnosis

Navigating Prior Authorization

Here's generally how the process works:

1. You or your care team submits a request for a test, procedure, or treatment, along with supporting clinical documentation.
2. If the request fits your insurance provider’s guidelines, it is approved, and you may not need to do anything further.
3. If the request needs more information, it may be pended rather than decided. A pended request is not a denial. It usually means the insurer wants additional documentation from your care team.
4. If the request does not clearly fit coverage guidelines, it may be denied.

The guidelines insurers rely on are built around common cancers. Because EHE is ultra-rare, those guidelines may say nothing about the care you need. That does not mean your requested care is inappropriate. It usually means more documentation and explanation are required.

Three things will help you before you ever face a denial:

1. Know the treatment guidelines that apply to you. The National Comprehensive Cancer Network (NCCN) Guidelines for Patients: Soft Tissue Sarcoma cover sarcomas including EHE. Your care team can draw on the clinician version of the NCCN Guidelines and the NCCN Drugs & Biologics Compendium, which insurers frequently reference in coverage decisions. The Experts’ Consensus Paper on EHE Management is the international consensus document specific to this disease, and citing it is often the strongest support available for an EHE-specific request.
2. Understand the prior authorization rules for your specific plan. Requirements differ meaningfully between individual and employer-sponsored coverage, and again for Medicare and Medicaid. Resources from Triage Cancer: Quick Guide to Pre-Authorizations for Individual & Employer-Sponsored Health Insurance Quick Guide to Which Insurance Plan Pays First, useful if you have more than one type of coverage
3. Keep records from the very first request. This is the single most valuable thing you can do. For every test or treatment request, track:

• The date your care team ordered it
• The date prior authorization was requested
• The date it was approved, pended, or denied
• Every phone call: the date, who you spoke with, and the reference or call ID number they give you
• Any delay to your scheduled care, and how long it lasted

If you ever need to appeal, or to escalate beyond your insurer, this record is what makes your case. Patterns of delay are far more persuasive than a single frustrating phone call, and you cannot reconstruct a pattern after the fact.

How to Appeal

A denial can feel frightening and discouraging, but it is often not the final word, and many denials are successfully overturned.

Know your deadlines first.

For most plans that comply with the Affordable Care Act, the following timelines usually apply. Medicare, Medicaid, TRICARE, and grandfathered or short-term plans may have different rules. Your timelines will depend on your specific plan. Check with your insurance provider to confirm the deadlines that apply to your coverage.

File an internal appeal Deadline: Within 180 days of receiving the denial notice

Insurer decision, care not yet received Deadline: Within 30 days

Insurer decision, care already received Deadline: Within 60 days

Insurer decision, urgent care Deadline: As quickly as your condition requires, and no later than 4 business days after your request

File for external review Deadline: Within 4 months of the final internal denial

External review decision Deadline: Within 45 days, or 72 hours or less if expedited

If a delay in care could jeopardize your health, say so explicitly in writing and ask for an expedited appeal.

Step 1: Read the denial notice closely.

Your denial notice is the roadmap for moving forward with an appeal. For most insurance plans, the insurer must give you in writing:

• Specific reason your claim or request was denied
• Notice of your right to file an internal appeal
• Instructions for how to submit an appeal
• Deadlines that apply

If any of that is missing or unclear, call your insurance provider and ask for it in writing.

Step 2: Ask your care team to request a peer-to-peer review.

Your oncologist can ask to speak directly with the physician who reviewed your case. Many denials are reversed in this single conversation, because it is often the first time anyone at the insurer hears the clinical reasoning from someone who understands the disease.

This step is worth pushing for, because the insurer's reviewing physician is frequently not an oncologist and is unlikely to have sarcoma or EHE experience.

Step 3: Ask for a copy of the independent physician expert review if one was performed.

You have a right to know who decided your case. Under the federal claims and appeals rules that govern most health coverage in the US, your plan must give you two things when you ask for them:

• The identification of any medical experts whose advice the plan obtained in connection with your denial. This applies whether or not the plan actually relied on that advice. The Department of Labor has stated explicitly that a plan cannot satisfy this by giving you only the reviewer's qualifications or the name of the company that employs them.
• Reasonable access to, and free copies of, all documents, records, and other information relevant to your claim. This is often called your claim file, where you’ll find the reviewer’s notes and the criteria applied to your case.

These rules apply to employer-sponsored group health plans and, with minor differences, to individual and Marketplace plans. Medicare, Medicaid, and TRICARE run their own appeal systems with their own disclosure rules, and plans sponsored by churches or by state and local governments may be exempt. If you are not sure which applies to you, the “Who Can Help” section explains how to find out.

How to ask. Call your insurance provider to make the request verbally. Then put the request in writing, because a written request creates a record and is harder to deflect. Ask for the rationale supporting the denial, and ask whether the medical reviewer has experience or expertise relevant to EHE. If your insurance provider provides only limited information, ask for additional details about the reviewer, including their name and the basis for the decision. Keep a copy of all correspondence for your records, and continue with the appeal process.

What to do with the answer. If you have reason to believe a physician reviewer lacked appropriate expertise to review your appeal, document your concerns and discuss them with your treating physician. In some cases, it may be appropriate to file a complaint with the state’s medical board in the state where the reviewer is licensed.

Step 4: Lean on your care team.

Ask your care team to review your claim with you and to help with your appeal. Your doctor may be your best support.

Step 5: Ask your insurer about a rare disease care coordinator.

Some plans assign a care coordinator or case manager to members with complex or rare conditions. If yours does, that person may become your ally, someone inside the system whose job is to make your care happen. Ask whether your plan offers someone who helps people with rare diseases.

Step 6: Request an external review.

If your internal appeal is denied, you may have the right to an independent external review. An independent medical expert or review organization outside your insurer reviews your case and assigns reviewers who were not involved in the original decision. The insurer must abide by the outcome. Expedited external review is available when your condition is urgent and, in some cases, can be requested at the same time as your internal appeal rather than after it.

If a Medication or Treatment is Denied as Off-label:

There are no approved treatments specifically for EHE. This causes many patients and their doctors to seek use of a treatment that is approved for another disease for use as an “off-label” treatment for EHE.

An off-label denial may not be the end of the road. Your doctor can request insurance coverage based on medical necessity. The request can be supported by published literature, the ESMO consensus guidelines, or listings in recognized drug compendia such as the NCCN Drugs & Biologics Compendium. An alternate approach may be for your doctor to ask the drug manufacturer about its patient assistance program, which may provide the drug at reduced or no cost.

For drugs in clinical trials that have not yet been approved for use, expanded access, also called compassionate use, is a pathway your doctor can consider if it is appropriate for your situation.

Helpful tools

1. ProPublica: Find Out Why Your Health Insurance Claim Was Denied
2. Triage Cancer: Appeals Resources

Who Can Help

If a denial is not resolved through your care team, your insurer, or the appeals process, other parties can apply pressure on your behalf. Your records are what make these routes work, so bring your documentation with you.

Your Employer

If your coverage comes through an employer, ask your Human Resources department or benefits manager to raise concerns directly with the insurer.

Your Plan's Regulator

Who oversees your plan depends on how you are covered.

• If you have an individual or Marketplace plan, or a fully insured employer plan, your state Department of Insurance regulates your insurer and can investigate unfair or unlawful coverage practices.
• If you have a self-funded employer plan, your state Department of Insurance has no authority over it. Federal law governs these plans, and the agency to contact is the US Department of Labor's Employee Benefits Security Administration. You can reach a benefits advisor at Ask EBSA or 1-866-444-3272.
• If you have Medicare or Medicaid, each program has its own appeal system, deadlines, and ombudsman.

If you are not sure which category you fall into, look for information in your plan documents.

Organizations That Can Work Your Case With You

Two nonprofits offer free, one-on-one help, and neither requires a common diagnosis. The EHE Foundation does not partner with or endorse these services.

• Triage Cancer Legal & Financial Navigation Program, free one-on-one help with insurance and related legal and financial questions
• Patient Advocate Foundation, case management for insurance denials and appeals, reachable at 800-532-5274. In 2025, they served people across more than 750 distinct diagnoses, so an ultra-rare cancer is not a barrier to getting help.

Helpful tool:

• Charts of State Laws Related to Health Insurance (Triage Cancer), because state law sometimes offers more protection than federal law

If you would like to read more widely, Triage Cancer's Cancer & Health Insurance hub and its full library of quick guides and checklists are the source of most of the tools linked on this page.

Understanding your coverage, keeping careful records, and knowing who to escalate to will not guarantee that every request is approved, but it can make a real difference in the outcome.

Downloadable Resources

As you navigate medical insurance and a potential appeal, it may help to use templates designed specifically for cases involving EHE. While these will not guarantee a successful appeal, they are a good starting point for your specific case. They are designed to be customized with your personal information, date of service, etc.