Total Resection of Pediatric Desmoid Tumor of the Left Neck with Utilization of 3D Virtual Surgical Planning

Abstract

Summary: A 13-year-old girl with a painful left neck mass was referred to our institution due to suspicions of malignancy. The patient reported pain that accompanied her frequent neck spasms. Computed tomography revealed a large, soft-tissue mass in the left neck, deep to the sternocleidomastoid. The lesion anteriorly displaced the internal carotid artery and both displaced and crushed the internal left jugular vein. Uniquely, a three-dimensional virtual reality model combining magnetic resonance imaging and computed tomography data was used to determine the lesion’s resectability and visualize which structures would be encountered or require protection while ensuring total resection. During operation, we confirmed that the mass also laterally displaced the brachial plexus, cranial nerves X and XI, and spinal nerves C3–C5 (including the phrenic) of the cervical plexus. Postsurgical pathological analysis confirmed a diagnosis of desmoid tumor, also known as aggressive fibromatosis, whereas DNA sequencing revealed a CTNNB1 mutation, a somatic genetic marker found in approximately 90% of desmoid tumor cases. When possible, the most widely used method for the treatment of desmoid tumors has been gross resection. Chemotherapy, radiotherapy, and local excision are also used in the treatment of fibromatoses when complete resection is judged infeasible. In this case, a complete surgical resection with tumor-free surgical margins was performed. A standard cervical approach with a modified posterolateral incision site was implemented to avoid a conspicuous anterior neck scar. No flap, nerve repair, or reconstruction was warranted. At 1 year of postsurgical follow-up, the patient showed minimal scarring and no signs of recurrence.