Last Updated: 2001-07-27 13:07:56 EDT (Reuters Health)
מתוך מדיקונטקסט
WESTPORT, CT (Reuters Health) – Pain and enlarging neurofibromas are the first signs of malignant peripheral nerve sheath tumors in patients with neurofibromatosis type 1 (NF1), French researchers report in the July issue of the Archives of Dermatology.
Dr. Pierre Wolkenstein, of Henri-Modor Hospital, Creteil, and colleagues note that such tumors are the main cause of mortality in adults with NF1. However, "symptoms are usually present for months or even years before the malignant neoplasm is recognized."
To investigate potential prognostic factors and criteria that might help in early detection, the researchers conducted a retrospective review of a cohort of 395 NF1 patients.
Of these, 17 had malignant peripheral nerve sheath tumors. In 12 patients the tumors were high-grade. All tumors, but one, developed on pre-existing nodular neurofibromas and pain and enlarging mass were the first and predominant symptoms.
Analysis showed no significant p53 tumor suppressor gene staining or mutation in benign tumors. However, 6 of 12 malignant tumors significantly overexpressed p53 and 4 of 6 showed missense mutations, suggesting that "p53 alterations play a role."
The researchers, who note that median survival was 18 months in patients with these "highly aggressive" tumors, recommend that to shorten the diagnosis delay "repeated deep biopsies of painful and enlarging nodular or plexiform neurofibromas should be considered in patients with NF1."
Arch Dermatol 2001;137:908-913.
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