Is Peripheral Nerve Sheath Tumor A Serious Condition?

Is Peripheral Nerve Sheath Tumor A Serious Condition?

Nerve sheath tumors or peripheral nerve sheath tumors (PNST) are tumors that arise from the myelin sheath which covers the nerves. There are two types of peripheral nerve sheath tumors benign and malignant tumors. The benign tumors are the schwannoma, schwannoma variants, neurofibroma, and neurofibromas variants; even though the names are different all tumors arise from the myelin sheath.

Malignant peripheral nerve sheath tumors (MPNST) is a rare sarcoma, the exact reason is unknown some believe that neurofibroma increase the risk of getting malignant peripheral nerve sheath tumors and some tumors appear without any cause. A malignant peripheral nerve sheath tumor originates from Schwann cells, perineural cells or from fibroblasts. So, there is a possibility that the benign peripheral nerve sheath tumors turning into malignant. Therefore, having a nerve sheath tumor can be a serious condition.

Is Peripheral Nerve Sheath Tumor A Serious Condition?

What Are Malignant Peripheral Nerve Sheath Tumors?

As, we mentioned above it is a very rare tumor the incidence is about 1:100,000 per year and it counts for about 5-10% of all soft tissue sarcomas. It is a very aggressive tumor.

A malignant peripheral nerve sheath tumor commonly occurs in the age group of 20-50 years and it is seen in both males and females equally. 40% of tumors occur in the extremities, 38% are in the trunk and the retroperitoneal area, and 21% of the tumors arise from the head and neck regions. On diagnosis most of the malignant peripheral nerve sheath tumors are high grade very aggressive tumors and these tumors recur 40% to 65% even after surgical removal. Malignant peripheral nerve sheath tumors metastasize 40% to 80%, it metastasize mainly by blood and most of the secondary tumors are seen in the lungs.

Can Benign Peripheral Nerve Sheath Tumors Change To Malignant Peripheral Nerve Sheath Tumors?

Well, no one knows the exact answer to this question and no one can say with 100% guarantee that peripheral peripheral nerve sheath tumors will not turn into a malignant one. It is unclear whether all benign peripheral nerve sheath tumors can turn into malignant peripheral nerve sheath tumors but there is evidence that neurofibromatosis type 1 and previous radiation exposure can increase the risk and these patients have a 10% life time risk of developing malignant peripheral nerve sheath tumors. 25-50% of malignant peripheral nerve sheath tumors are associated with neurofibromatosis 1. Although, there is not much evidence about the association of malignant peripheral nerve sheath tumors with schwannoma.

If you have schwannoma or neurofibroma any sudden increase in the tumor, rapid growth of the tumor, pain or tingling sensation on the area of the tumor can be symptoms of Malignant PNST. But, just by clinical examination we cannot say exactly whether it is benign or malignant. Therefore, if the above symptoms occur consult a doctor, the doctor will tell you what to do after evaluating your case. The only way to accurately distinguish benign and malignant tumors is by doing a needle biopsy.

Prognosis Of Malignant Peripheral Nerve Sheath Tumors

Prognosis depends on many factors the size and the location of the tumor, patients who have small tumor and/ or tumor in the arms or legs has an increase survival rate. If Malignant PNST is diagnosed at an early stage the prognosis is good. Tumors that have recurred and/or metastasized have poor prognosis.

Summary

Nerve sheath tumors or peripheral nerve sheath tumors (PNST) are tumors that arise from the myelin sheath which covers the nerves. There are two types of peripheral nerve sheath tumors benign and malignant tumors. The benign tumors are the schwannoma, neurofibromas, and the tumor variants. Malignant peripheral nerve sheath tumors (MPNST) is a rare sarcoma the exact reason is unknown. It’s unclear whether all benign peripheral nerve sheath tumors can turn into a malignant one, but there is evidence that neurofibromatosis type 1 and previous radiation exposure can increase the risk and these patients have a 10% life time risk of developing malignant peripheral nerve sheath tumors. 25-50% of malignant peripheral nerve sheath tumors are associated with neurofibromatosis 1. Although, there is not much evidence about the association of malignant peripheral nerve sheath tumors with schwannoma and hemangiopericytoma. Sudden increase in the tumor, rapid growth of the tumor, pain or tingling sensation on the area of the tumor can be symptoms of malignant peripheral nerve sheath tumors and the only way to diagnose accurately is by doing a needle biopsy.

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