A Rare Association Between Neurofibromatosis Type 1 and Thigh Leiomyosarcoma

Abstract

Neurofibromatosis type 1 (NF1) is a rare genetic disorder sporadic or genetically transmitted in autosomal dominant mode with an estimated incidence of 1 :3000-4000 live births [1]. Clinical diagnostic criteria are well established. About that there are seven criteria for NF I according to the National Institutes of Health (Table 1) [2] and two or more of which must be met to make a diagnosis of the disease. During NF I, the susceptibility to neoplastic malignant transformation is the main feature of the disease with a probability from 3 to 4 times greater than the general population [3-5]. On the other hand, few cases of NF I associated with sarcoma including pleomorphic sarcoma [6], rhabdomyosarcoma [7], leiomyosarcoma [8] and Ewing sarcoma [9], have been reported.