Sarcomas

Malignant Peripheral Nerve Sheath Tumors (MPNST)

Malignant peripheral nerve sheath tumors (MPNSTs) are sarcomas which originate from peripheral nerves or from cells associated with the nerve sheath, such as Schwann cells, perineural cells, or fibroblasts. Because MPNSTs can arise from multiple cell types, the overall appearance can very greatly from one case to the next. This can make diagnosis and classification somewhat difficult. In general, a sarcoma arising from a peripheral nerve or a neurofibroma is considered to be a MPNST. The term MPNST replaces a number of previously used names including malignant schwannoma, neurofibrosarcoma, and neurogenic sarcoma.

A sarcoma is defined as a MPNST when at least one of the following criteria is met:

1. It arises from a peripheral nerve

2. It arises from a preexisting benign nerve sheath tumor (neurofibroma)

3. It demonstrates Schwann cell differentiation on histologic examination

Dr. David Geller at the and Harvard Orthopaedic Surgery Program and Dr. Gebhardt, Frederick W. and Jane M. Ilfeld Professor of Orthopedic Surgery at Harvard Medical School, have written and in-depth article about Malignant Peripheral Nerve Sheath Tumors (MPNST) for the Electronic Sarcoma Update Newsletter (ESUN). Their article gives an extensive set of references to articles dealing with this disease. We are grateful to them for writing this informative article. Click on the above link to view it.

There are over 50 subtypes of sarcoma. The American Cancer Society's 2005 Key Statistics About Sarcoma states that the most common types of soft tissue sarcoma are: MFH or Malignant Fibrous Histiocytoma (28%), liposarcoma (15%), LMS or leiomyosarcoma (12%), synovial sarcoma (10%), malignant peripheral nerve sheath tumors (6%), and rhabdomyosarcoma (5%). All of the other types of soft tissue sarcoma occur at percentages of 3% or less.

Where else can you learn about MPNST? Steve Dunn's CancerGuide provides a very useful starting point for undertaking investigations into cancer and cancer-related issues on the Internet. Among other things, he discusses how to research the medical literature and how to use and access medical databases and online resources. He explains the medical research cycle, where to get medical references and describes the various types of papers in the medical literature, and how to find and use a medical library. A word of caution. The Internet is a wonderful tool to access to information. However, simply because information appears on the Internet does not necessarily indicate that it is accurate or truthful. Cancer-related information that you obtain on the internet that you believe might be relevant to your situation, should be reviewed with your physician for its accuracy and relevance. Interested readers are encouraged to continue their investigations into malignant peripheral nerve sheath tumors (MPNSTs) by examining the resources at the websites listed below: The Doctor's Doctor MPNST webpage Malignant Peripheral Nerve Sheath Tumor by Dr. Peter Hrehorovich et al