The cutaneous neurofibromas in neurofibromatosis type 1 (NF1) are benign. Other tumors, however, occur and include optic pathway tumors, leukemia, malignant peripheral nerve sheath tumors, rhabdomyosarcomas, pheochromocytomas, and various brain tumors. The brain tumors sometimes can be managed more conservatively than similar presentations in the general population (they usually have a more indolent course), but malignant peripheral nerve sheath tumors tend to be more aggressive. [Korf: 2000] Treatment of the various tumors in NF1 is an area of ongoing research. If a patient develops unexplained pain and/or rapid tumor growth, one should be suspicious for a neoplasm. Referral to a tertiary care center familiar with NF1 and sarcoma management is recommended for concerns of tumors other than the benign neurofibromas.
The benign dermal neurofibromas generally cause no significant problems. They can be removed for cosmetic reasons, pain, or if they are in an area where the tumor catches onto clothing etc. The mainstay of treatment is surgical excision (Rosser and Packer, 2002). Excision does not mean the neurofibroma will not return, as it is difficult to excise the entire tumor. The neurofibromas usually present after the pigmentary lesions and increase with age with a more rapid increase during puberty and pregnancy. Various modalities of surgical excision have been adopted at different centers, but presently there is not an accepted protocol for medical management or prevention.
|Author:||David A. Stevenson, MD - 11/2004|
|Content Last Updated:||2/2009|
Malignancy in neurofibromatosis type 1.
Oncologist. 2000;5(6):477-85. PubMed abstract
Excellent review of the malignancies in NF1 with particular focus on malignant peripheral nerve sheath tumors.