What Is Perineurioma and How Is It Treated?

Written by Medicover Team and Medically Reviewed by Dr Krishna Haskar Dhanyamraju , Neurologists

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1. Causes 2. Find a Doctor 3. Diagnosis 4. Treatment 5. FAQs

Perineurioma is a rare, typically benign tumor arising from perineurial cells, which form part of the protective covering around peripheral nerves. It can develop in different locations where peripheral nerves are present and may grow slowly over time.

Perineuriomas are generally classified into intraneural and soft tissue forms. Intraneural perineurioma affects a peripheral nerve and may cause progressive weakness or other nerve-related symptoms, while soft tissue perineurioma usually presents as a slow-growing, painless mass. The exact cause of perineurioma is not fully understood.

What are the Types of Perineurioma?

Perineuriomas are broadly classified according to whether they arise within a nerve or in soft tissue. Their clinical features and management can differ depending on the location and extent of the tumor.

Intraneural perineurioma: A benign peripheral nerve tumor that develops within a nerve and can cause progressive weakness, sensory changes, or loss of nerve function.
Soft tissue perineurioma: Usually presents as a well-circumscribed, slow-growing, painless nodule in the soft tissues.
Sclerosing perineurioma: A rare subtype characterized by perineurial cells within a prominent collagen-rich, sclerotic background.

What are the Symptoms of Perineurioma?

The symptoms of perineurioma depend on its type, location, size, and whether it affects a nerve. Soft tissue perineuriomas may present as a painless, slowly growing lump, while intraneural perineuriomas can cause progressive nerve dysfunction.

Painless lump or swelling: A slow-growing, palpable mass may occur in the soft tissues, commonly involving the extremities.
Numbness or tingling: Intraneural perineurioma may cause altered sensation along the affected nerve.
Muscle weakness: Progressive weakness may occur when a perineurioma affects motor nerve function.
Pain or discomfort: Some individuals may experience pain or discomfort in the affected area, although many tumors are painless.
Reduced function: Nerve involvement may lead to difficulty moving or using the affected limb or body part.

What are the Causes of Perineurioma?

The exact cause of perineurioma is not fully understood. These tumors arise from perineurial cells, and specific genetic alterations have been identified in some cases. Most perineuriomas occur sporadically, and a clear environmental or lifestyle-related cause has not been established.

Genetic alterations: Certain molecular and chromosomal changes may contribute to the development of perineurioma.
Age: Perineuriomas can occur in children and adults, depending on the tumor subtype.
Previous radiation exposure: Radiation-associated perineurioma has been reported but appears to be uncommon.
Trauma or chronic irritation: These have been proposed as possible contributing factors, although a definite causal relationship has not been established.

When to See a Doctor?

Consult a neurologist or healthcare provider if you notice an unusual lump or develop symptoms that may indicate a perineurioma or another nerve-related condition.

Painless or gradually enlarging lump in the soft tissues or limbs
Numbness, tingling, or altered sensation in an affected area
Progressive weakness or difficulty moving a limb

Get medical help promptly if:

There is a rapid increase in the size of a lump
Significant or rapidly worsening weakness or loss of function develops
New severe pain or other rapidly progressive neurological symptoms occur

Early evaluation can help determine the cause of a lump or nerve-related symptoms and guide appropriate monitoring or treatment.

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How Is Perineurioma Diagnosed?

Diagnosing perineurioma involves a combination of clinical assessment, imaging, and examination of tumor tissue. The diagnostic approach depends on whether the tumor appears to involve a peripheral nerve or soft tissue.

Physical and neurological examination: The healthcare provider evaluates the size and location of a mass and checks for sensory changes, weakness, reflex abnormalities, or other signs of nerve dysfunction.
Imaging studies: MRI is particularly useful for assessing the location and extent of the tumor and its relationship to surrounding nerves and tissues. Ultrasound or CT may also be used in selected cases.
Biopsy and histopathological examination: A biopsy may be performed when necessary to examine the tumor under a microscope and establish the diagnosis.
Immunohistochemistry: Specialized staining, including markers such as epithelial membrane antigen (EMA), can help confirm perineurial differentiation and distinguish perineurioma from other nerve sheath tumors.
Electrodiagnostic testing: Nerve conduction studies and electromyography may be used in intraneural perineurioma to assess the extent of nerve dysfunction.

What are the Treatment Options for Perineurioma?

Treatment depends on the type, size, location, symptoms, growth pattern, and effect on nerve function. Because most perineuriomas are benign, management is individualized with the goal of controlling symptoms while preserving nerve function.

Observation and monitoring: Small, asymptomatic soft tissue perineuriomas may be monitored through regular clinical examinations and imaging.
Surgical excision: Complete surgical removal may be considered for symptomatic, enlarging, or accessible soft tissue tumors. The approach is planned to minimize damage to surrounding nerves and tissues.
Nerve-sparing surgery: When a tumor is associated with a peripheral nerve, specialized surgical techniques may be considered when removal is appropriate and feasible.
Supportive rehabilitation: Physical therapy, occupational therapy, or other rehabilitation may help improve strength and function when nerve dysfunction is present.

Radiation therapy and chemotherapy are generally not standard treatments for benign perineuriomas. Regular follow-up may be recommended to monitor symptoms, tumor growth, and nerve function.

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What are the Risk Factors of Perineurioma?

There are no well-established lifestyle or environmental risk factors for most perineuriomas. The majority occur sporadically, and many patients have no identifiable risk factor.

Age: Different forms of perineurioma can occur at different ages, with intraneural perineurioma often recognized in younger individuals.
Genetic or molecular changes: Specific genetic alterations may contribute to tumor development.
Previous radiation exposure: Rare radiation-associated cases have been described.