Malignant peripheral nerve sheath tumors

Malignant peripheral nerve sheath tumors

overview

Malignant peripheral nerve sheath tumors are a rare type of cancer that occur in the lining of the nerves that extend from the spinal cord into the body. Previously, malignant peripheral nerve sheath tumors were called neurofibrosarcomas.

Malignant peripheral nerve sheath tumors can occur anywhere in the body, but are most common in the deep tissues of the arms, legs, and trunk. They tend to cause pain and weakness in the affected area and can also cause a growing lump or mass.

Malignant peripheral nerve sheath tumors are typically treated surgically. In certain cases, radiation therapy and chemotherapy may also be recommended.

Symptoms

Signs and symptoms of malignant peripheral nerve sheath tumors include:

• Schmerzen im betroffenen Bereich
• Schwäche beim Versuch, den betroffenen Körperteil zu bewegen
• Ein wachsender Gewebeklumpen unter der Haut

When to go to the doctor?

Make an appointment with your doctor if you have persistent signs and symptoms that worry you. Malignant peripheral nerve sheath tumors are rare, so your doctor may investigate more common causes of your symptoms.

Causes

It is not clear what causes most malignant peripheral nerve sheath tumors.

Doctors know that these cancers begin when a cell in the protective layer around a nerve develops a mistake (mutation) in its DNA. The mutation instructs the cell to multiply quickly and continue living when other cells would normally die. The accumulating cells form a tumor that can grow to invade nearby tissue or spread to other areas of the body.

Risk factors

Factors that increase the risk of malignant peripheral nerve sheath tumors include:

• Frühere Strahlentherapie bei Krebs. 10 bis 20 Jahre nach der Behandlung kann sich im bestrahlten Bereich ein bösartiger peripherer Nervenscheidentumor entwickeln.
• Gutartige Nerventumore. Bösartige periphere Nervenscheidentumoren können sich aus gutartigen (gutartigen) Nerventumoren wie Neurofibromen entwickeln.
• Eine Erbkrankheit, die das Risiko von Nerventumoren erhöht. Bösartige periphere Nervenscheidentumoren treten häufiger bei Menschen mit Neurofibromatose 1 auf.

Treatment of malignant peripheral nerve sheath tumors

Sources:

1. Gilchrist JM, et al. Tumore der peripheren Nerven. https://www.uptodate.com/contents/search. Abgerufen am 20. Juli 2017.
2. Winn HR, Hrsg. Gutartige und bösartige Tumoren des peripheren Nervs. In: Youmans Neurologische Chirurgie. 7. Aufl. Philadelphia, Pennsylvania: Elsevier; 2017. https://www.clinicalkey.com. Abgerufen am 20. Juli 2017.
3. Stucky CH, et al. Maligne periphere Nervenscheidentumoren (MPNST): Die Erfahrung der Mayo Clinic. Annalen der chirurgischen Onkologie. 2012;19:878.
4. Warner KJ. Allscripts EPSi. Mayo Clinic, Rochester, Minnesota, 4. April 2017.