Abdominal plexiform neurofibroma. Case report
Keywords:
neurofibromatosis, Von Recklinghausen's disease, abdominal plexiform neurofibromaAbstract
Introduction: Plexiform neurofibroma is a subtype of type 1 neurofibromatosis that is characterized by affecting multiple bundles of a nerve, being recurrent, and having a high risk of malignant transformation.
Case Report: A 2-year-old female patient, her mother noticed increase in volume in the lower abdominal region. On physical examination an increase in volume was palpated in the right iliac fossa. The abdominal computed tomography revealed a tumor image of variable density, predominantly hypodense. Ultrasound-guided fine-needle aspiration cytology revealed plexiform neurofibroma. During the intraoperative period, an abdominal tumor adhered to the posterior face of the bladder and the sacrum was identified. The tumor was completely resected. Histopathological examination confirmed the diagnosis. In the postoperative period, the magnetic resonance imaging concluded as a tumor recurrence of possible neurogenic etiology with malignant behavior. It was evaluated in a multidisciplinary consultation and pharmacological treatment was decided.
Conclusions: Abdominal plexiform neurofibroma is a rare condition with a high risk of tumor recurrence and malignant transformation. Although there is no curative treatment for this disease, surgical resection of the tumor together with the use of drugs that are still in the clinical trial phase have managed to reduce tumor volume and improve quality and life expectancy.
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15 junio 2025
