Giant Plexiform Neurofibroma of the Pelvic Region: Unusual Presentation in an Uncommon Location (About a Case)

Plexiform neurofibroma is a rare benign tumor of the peripheral tissue cells developed in the perineurium. Often considered pathognomonic of neurofibromatosis type 1 (NF1 or von Recklinghausen disease), it can be solitary, especially in children [1]. These entities consist of multiple, twisted masses that grow along the axis of a large nerve, infiltrating and separating normal nerve fascicles [2]. It rarely grows to be larger than 5 cm; however, neurofibromas can undergo continuous enlargement and eventually become giant lesions [3]. Thus, it constitutes a significant source of structural and functional morbidity [4]. The diagnosis is essentially pathological [1] and malignant transformation is the main associated complication [2]. Surgery is frequently reserved until PNs have progressed to the point of causing functional compromise, esthetic deformity or pain [2].