| TERRY HAMBlIN |
These can be either benign or malignant.
This is what you get on Google: The malignant peripheral nerve sheath
tumor (MPNST) is the malignant counterpart to benign soft tissue tumors
such as neurofibromas and schwannomas. This term is preferred to older
designations such as malignant schwannomas and neurofibrosarcomas. It is
most common in the deep soft tissue, usually in close
proximity of a nerve trunk. The most common sites include the sciatic
nerve, brachial plexus, and sarcal plexus. The most common symptom is
pain which usually prompts a biopsy. Most cases average more than 5 cm
in diameter. On sections, it may have a white surface with areas of
necrosis or hemorrhage. It is the most common sarcoma arising in the
setting of von Recklinghausen's
disease.
Von Recklinghausen's syndrome is a congenital disease that manifests as
soft fleshy growths in many situations on the body. A minor form has no
growths, but 'cafe au lait' spots on the skin. In my series of 600 local
patients with CLL seen in Bournemouth over 30
years, I saw two patients with Von Recklinghausen's syndrome.
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