Neurofibroma

January 12, 2026

This page was reviewed under our medical and editorial policy by Rosalind Munoz, P.A., Department of Medical Oncology & Therapeutics Research, City of Hope^® Cancer Center Duarte.

Neurofibroma is the most common type of tumor that develops in the peripheral nerve sheath – the protective tissue that surrounds the body’s nerves. Neurofibromas are common, almost always benign and respond well to treatment.

What Is Neurofibroma?

Neurofibroma is a kind of benign tumor that can develop in the body’s peripheral nerve sheath tissue. The peripheral nerve sheath forms a barrier that surrounds and protects nerves inside the body.

Unlike some other tumor types, neurofibroma develops in several different types of cells at the same time. It is called neurofibroma because it affects both neuron (nerve) tissue and fibrous (connective) tissue. In about 10% of cases, people who develop neurofibroma may also have a rare genetic condition called neurofibromatosis type 1.

Schwannoma vs. Neurofibroma

Schwannoma and neurofibroma are both types of tumors that can affect the body’s central nervous system. Both Schwannoma and neurofibroma can affect nerves in the brain, spinal cord or elsewhere in the body. They are both usually benign and slow-growing.

The main difference between Schwannoma and neurofibroma is that Schwannoma only develops in a type of cell called Schwann cells, while neurofibroma develops in Schwann cells and other cell types, including mast cells, perineurial cells and fibroblasts. Another difference is that neurofibroma may be caused by neurofibromatosis type 1, while Schwannoma has no clear causes.

Neurofibroma vs. Skin Tag

One possible sign of neurofibroma is a small lump or swelling on the skin that can sometimes be mistaken for a skin tag. A skin tag is a small, benign, skin-colored bump While a skin tag does not usually change color or texture when it is touched, a bump caused by neurofibroma may turn “inside-out” and create an indentation like a buttonhole if it’s pressed on.

Types

There are several types of neurofibroma. These types are classified according to where they tend to develop in the body and how the tumor grows.

Cutaneous Neurofibroma

Cutaneous neurofibroma (sometimes called localized neurofibroma) refers to neurofibroma tumors that develop in nerve tissue that is just under the surface of the skin. This type of neurofibromas usually appears as a fleshy bump or lump on the skin. Often, cutaneous neurofibroma does not require treatment.

Diffuse cutaneous neurofibroma is a rare subtype of cutaneous neurofibroma that has grown or spread more significantly. It usually occurs on the head or neck.

Plexiform Neurofibroma

Plexiform neurofibroma is a type of neurofibroma that affects up to half of all people with neurofibromatosis type 1. Though it can cause bumps or swellings of any size, it tends to result in larger lesions or bumps than cutaneous neurofibroma. These lesions can have poorly-defined edges, look different in color than the surrounding skin and feel “knotty” when pressed on.

Neurofibroma Symptoms

Many people with neurofibromas experience no symptoms. For those who do have symptoms related to the condition, these usually depend on what type of neurofibroma they have.

Some common symptoms of cutaneous neurofibroma are:

• Small, round lumps on the skin that are flesh-colored and painless Small skin bumps that “retract” into the surface of the skin when pressed on, then “pop” back out when pressure is taken away
• Larger lesions or bumps on the head or neck that may also feel rough to the touch, in the case of diffuse cutaneous neurofibroma

Some common symptoms of plexiform neurofibroma include:

• Swellings in the arms or legs, on the head and neck or on the torso that are larger in size
• Larger lesions that feel “knotty” and fibrous to the touch
• Rough-skinned swellings that may be numb or uncomfortable

Causes and Risk Factors

Neurofibroma is caused by a genetic change to a gene called NF1. When someone has a mutation in the NF1 gene, this can cause the body to develop cutaneous (localized) or plexiform neurofibromas. Some risk factors that may cause people to develop neurofibroma include:

• Having a rare genetic condition called neurofibromatosis type 1: This genetic syndrome causes a permanent change to how the body’s NF1 gene works, which can lead to the development of neurofibroma of any type.
• Being under age 5: Almost all cases of plexiform neurofibroma first appear in children aged 5 and under.
• Being an adult aged 20 to 40: The most common age for cutaneous (localized) neurofibroma to appear is between the ages of 20 and 40. It affects men and women equally.

Diagnosis and Testing

The first step in diagnosing neurofibroma is usually a physical exam performed by a doctor who is specialty-trained in skin health conditions. A definitive diagnosis can be made by removing a neurofibroma lesion and examining it under a microscope. For patients with diffuse or plexiform neurofibroma, doctors may also recommend imaging tests, such as magnetic resonance imaging, to help determine a tumor’s size and extent.

Neurofibroma Treatment

In many cases, cutaneous (localized) neurofibroma may require no treatment other than regular check-ups with a dermatologist or skin health specialist. Some patients may choose to have neurofibroma tumors removed surgically for cosmetic reasons. For patients with more widespread neurofibroma or with plexiform neurofibroma, treatment may involve surgery to remove or shrink a tumor. Medications called MEK inhibitors are another treatment option. They work by blocking how the body produces certain proteins, which can help shrink tumors.

Who Treats Neurofibroma?

Patients with benign or malignant neurofibroma may receive care from a wide range of health specialists, depending on their unique diagnosis. This multidisciplinary team may include:

• Dermatologists, who are doctors who specialize in diagnosing and treating skin disorders.
• Pathologists, who may help diagnose neurofibroma if a tumor is removed during a surgical procedure.
• Cancer doctors with specialist training in treating tumors affecting the areas of the body affected by neurofibroma.
• Surgeons with expertise in removing tumors from various sites in the body.
• Medical oncologists with experience using medications such as chemotherapy and targeted therapies to treat cancer.
• Supportive medicine providers, who help patients manage the physical, emotional and social side effects of cancer and its treatments.

Complications

Cutaneous (localized) neurofibroma rarely causes any complications. However, patients with plexiform neurofibroma can experience health issues related to the condition. These include:

• Itching or numbness at the site of a neurofibroma tumor
• Pain, tingling, numbness caused by a tumor pressing on nerves or organs inside the body
• Problems with organ function, bowel movements or other bodily functions caused by pressure from a tumor inside the body
• An increased risk of a benign neurofibroma becoming cancerous, although this only occurs in about 10% of cases