Surgical management of a plexiform neurofibroma in neurofibromatosis type 1: The haemostatic challenge.

BACKGROUND

Plexiform neurofibromas (PNFs) are benign tumours associated with neurofibromatosis type 1 (NF1) that may cause significant functional and cosmetic impairment. Surgical management is challenging because of diffuse infiltration and marked vascularity, increasing the risk of severe intraoperative bleeding.

CASE REPORT: A 70-year-old woman with NF1 presented with a progressively enlarging mass of the left flank causing contour deformity with functional impairment. Previous resection 20 years earlier had been complicated by major haemorrhage requiring transfusion of red blood cells. MRI demonstrated a 40 × 25 cm subcutaneous infiltrative PNF without neither muscular nor neurological involvement. Following multidisciplinary discussion, partial surgical debulking was performed using a hemicircular dermolipectomy approach. Intraoperative haemostasis was complicated because of diffuse vascular abnormalities and extensive infiltrative growing pattern of the tumour. Visible bleeding was mainly controlled using vascular clips. Postoperatively, tranexamic acid and incisional negative pressure wound therapy were used. Symptomatic postoperative anaemia required transfusion of red blood cells. Recovery was otherwise uneventful, and histopathology confirmed benign neurofibroma without atypia. At 9 months, functional and cosmetic outcomes were satisfactory without recurrence.

CONCLUSION

PNFs in NF1 patients remain surgically challenging because of their infiltrative growth and abnormal vascularity. Careful perioperative planning and meticulous haemostatic strategies are essential to reduce bleeding-related morbidity and optimize outcomes.