Management of large dorsal diffuse plexiform neurofibroma

Abstract

Neurofibromas are considered one of the hallmark signs of neurofibromatosis type 1(NF1). Diffuse plexiform neurofibroma is a congenital NF1-associated tumor, characterized by overgrowth and interference with function of the affected area. The rich vascular plexus associated with neurofibromas, together with their infiltrative pattern, makes them difficult to eradicate. Complications of neurofibromas are rare but include malignant transformation and potentially life-threatening hemorrhages. The use of the term “giant” to define a neurofibroma is controversial because there is no clear consensus and descriptions are limited to few case reports. We report a case of a large dorsal diffuse plexiform neurofibroma responsible for significant worsening of a patient’s quality of life. These features make us believe that the mass can be defined as “giant.” Comprehensive perioperative management, including arterial embolization of the feeding vessels and stabilization of the patient’s conditions, is required to perform a safe surgical procedure in such a complicated case.