NF1 diagnosis criteria and associated sarcomatous tumor review of the literature and case report

Abstract

Few cases of association of neurofibromatosis with other types of soft tissue sarcomas have been reported. Undifferentiated pleomorphic sarcomas are the most common of these tumors. We intend to call attention to the importance of patient follow-up and counseling.

Free Flap Reconstruction of Extensive Defects Following Resection of Large Neurofibromatosis

“…When a neurofibroma involves the orbitotemporal region, problems can occur, from a spectrum of isolated orbital tumors to extensive infiltration of the periorbita and eyelids and temporal deformities. 21,22 Progression of these lesions may result in severe proptosis, displacement of the globe, and compromised visual acuity.…”

“…9,14 -17 Most reports about NF-1 primarily focus on conservative methods and staged resections. [5][6][7]20,21 However, complete removal of the nidus is essential for possible local control and should be attempted during the resection. Severe, complex neurofibromas of the head and neck are rare, and thus, most surgeons have limited experience with them.…”

“…[5][6][7]20,21 Patients with severe, complex neurofibromas of the head and neck frequently undergo multiple procedures, often with poor functional and aesthetic outcomes. The severity of orbital involvement and the condition of the eye are key factors in orbitotemporal NF.…”

Epithelioid sarcoma associated with neurofibromatosis type I

“…Also, malignant tumors unrelated to the nervous system rarely develop in patients with neurofibromatosis. However, few cases of NF I associated with sarcoma have been reported, including pleo-morphic sarcoma [4], rhabdomyosarcoma [5], leiomyosarcoma [6], and Ewing sarcoma [7]. Here, we describe an unusual case of epithelioid sarcoma in a patient with NF I.…”

A Sublingual Neurofibroma: A Case Report

“…Since NF1 was classified as a tumor predisposition syndrome individuals with this syndrome harbour the elevated risk of development malign and benign tumors on peripheral nerves. 9,10 Besides that features, oral manifestations can be seen up to 92% of NF1 patients. 5 The tongue, buccal mucosa, alveolar crest, gingiva, lips, the floor of mouth, palatal and pharyngeal regions are the common involved sites in oral cavity.…”