Del Río IJ, Hernández JMK, Montes CJG, Torales RU, Guadarrama OP

Language: Spanish
References: 18
Page: 72-76
PDF size: 445.45 Kb.

ABSTRACT

Neurofibromatosis type 1 (NF1) is an autosomal dominant disease that causes the development of tumors called neurofibromas, its prevalence is one case per 3000 people approximately. Its diagnosis is mainly clinical. A plexiform neurofibroma (PN) is a neoplasm that affects multiple fascicles of a nerve and that grows along them, causing the compression of adjacent structures. Malignant transformation occurs in up to 17% of cases and is suspected when there is fast growth of PN, difficult-to-manage pain, hard tumor consistency, and new onset neurological deficit. Metastases to the lungs and bones are the most common. The surgical management of these lesions is often difficult due to the anatomical complexity of the tumors and affected structures. Until now, the best treatment for inoperable PN remains controversial. New drugs have been proposed to achieve this goal; gene therapy is expected to be able to offer an etiologic solution in the near future. This review provides updated information on new leaflets drugs for treatment of pn of patients diagnosed with NF1.

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