J Neurol Surg B Skull Base 2015; 76 - P034
DOI: 10.1055/s-0035-1546662

Endonasal Management of a Pediatric Pterygopalatine Fossa and Skull Base Myofibroma

Jeffrey C. Rastatter 1, Shaun D. Rodgers 1, Santino Cervantes 1, Tord D. Alden 1
  • 1Ann and Robert H Lurie Childrens Hospital of Chicago, Chicago, New York, United States

Introduction: Fibromatoses of childhood are a group of heterogenic conditions that involve abnormal proliferation of fibroblasts and myofibroblasts with up to one-third presenting in the head or neck. Myofibromas can be multicentric but more often present as an isolated lesion, showing rapid growth and local invasion. Myofibroma is a rare lesion and, more rarely, reported in the skull base.

Case Description: A 14-year-old otherwise healthy female patient presented with a 1 to 2 week history of progressive numbness of the right cheek associated with R > L nasal congestion, right orbital pressure, and mild-to-moderate headaches. On examination, she was afebrile and well appearing with normal facial contour. She had numbness in the right V2 distribution with an otherwise normal neurological examination. Nasendoscopy showed a pink to red-colored lesion under the right middle turbinate causing nasal airway obstruction and a mild septal deviation.

MR imaging demonstrated a mass lesion with intense enhancement involving the right pterygopalatine fossa, nasopharynx, and posterior nasal cavity with lateral extension to the pterygoid musculature. There was posterosuperior extension to involve the sphenoid sinus and skull base. The lesion abutted the temporal lobe of the brain superiorly and petrous genu of the internal carotid artery posteriorly. CT angiogram demonstrated erosion of the posterior nasal septum, right sphenoid floor, pterygoid root, pterygoid plates, lateral clivus, and greater wing of the sphenoid. There was invasion of the foramen rotundum and vidian canal, and extension through the posterior maxillary sinus.

Biopsy demonstrated a myofibroma with spindle cells and numerous vessels. Surgical management was recommended via an endoscopic endonasal approach (EEA). The lesion was embolized preoperatively. CTA and MR images were used for surgical navigation. Intraoperatively, the tumor was quite vascular despite the embolization. The lesion was dissected from the carotid artery at the petrous genu posteriorly and the dura of the temporal lobe superiorly. Gross total resection was achieved. The patient was discharged home on postoperative day 3.

Discussion: Pediatric myofibroma is a rare entity that is pathologically benign, yet clinically can demonstrate rapid local growth and invasiveness. Skull base presentations of these lesions are very rare in the current literature. This clinical case demonstrates the usefulness of an endoscopic endonasal approach for gross total resection of a locally aggressive myofibroma of the pterygopalatine fossa and skull base in a teenager.