Solitary neurofibromas are are well-circumscribed, non-encapsulated, benign tumors of neuromesenchymal tissue (Schwann cells, perineural cells, fibroblasts, and mast cells) with nerve axons. They present as asymptomatic, skin-colored or violaceous, rubbery papules or nodules. They can occur anywhere on the body. Solitary neurofibromas occur primarily in adulthood and late adolescence and are not an indication of neurofibromatosis. There is no sex predilection.
Codes
ICD10CM: D21.9 – Benign neoplasm of connective and other soft tissue, unspecified
SNOMEDCT: 404030000 – Solitary neurofibroma
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Diagnostic Pearls
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Differential Diagnosis & Pitfalls
Neurofibromatosis – Should be ruled out with a complete cutaneous examination.
Solitary neurofibroma is a type of non-cancerous growth on the skin that is caused by the cells around nerves. Imagine these supportive cells wrapping around the nerve like a tight blanket. This blanket covers the nerve to help it work properly and to help keep its shape. But if a blanket is not woven properly, the threads can sometimes lump together and form knots in certain places. This is what happens in solitary neurofibroma. The supportive cells surrounding the nerve do not grow properly and can form an abnormal lump of cells, and although they are not cancer, this is sometimes called a tumor. It is not known why or where these will occur, but when this happens at one location it is called solitary neurofibroma. If tumors are found in many locations it is called multiple neurofibromas and is associated with a medical syndrome called neurofibromatosis type I, also called Von Recklinghausen's disease. Most neurofibromas (90%) are solitary and can happen in nerves in the skin or in nerves found deep within the body.
Who’s At Risk
Solitary neurofibroma is a common non-cancerous growth, or tumor, that can happen to both men and women. Usually they are found in young people around the ages of 20 to 30.
Signs & Symptoms
A solitary neurofibroma can form in any nerve in the body but it usually shows up on the skin. It is usually painless, but sometimes it can cause weakness, numbness, or pain depending on where the growth is. When the growth forms on a nerve that is close to the skin, it can appear as a small and sometimes painful lump. When a doctor presses down on the lump it can feel like a sharp, electric pain is shooting down the body, arm, or leg. When a doctor examines the lump, they may check for "button-holing," which is when the lump folds back into the skin when pressed.
Self-Care Guidelines
If a solitary neurofibroma is painless and has no other signs or symptoms, then treatment is not necessary. It is important to remain physically active and to pay attention for numbness, tingling, pain, or weakness and to tell your doctor if you notice any of these symptoms.
When to Seek Medical Care
Seek medical care if you notice bleeding or pain, or if the growth changes size or shape. Numbness, tingling, or weakness in your muscles is also a sign to seek medical care.
Treatments
Treatment is not required. If requested, the only treatment for solitary neurofibroma is surgery. For small neurofibromas on the skin, dermatologists or other doctors can easily remove them in the office. Recovery from the surgery is generally immediate.
Patient Information for Solitary neurofibroma in Child