Resection of a Giant Craniofacial Chondrosarcoma: A Case Report

Background: Extremely large and disfiguring skull base and facial tumors are becoming a rare entity in developed countries. They always represent surgical and medical challenge. In this case report we present a 26-year-old gentleman from Jamaica who was referred to our institution with a very large facial, skull base and neck chondrosarcoma, which required staged surgery and multidisciplinary approach.

Clinical Presentation: A 26-year-old Jamaican male was brought to the hospital with a disfiguring and progressively growing facial mass with severe proptosis and near total blindness of the left eye. The left eye was pushed out from the orbital cavity due to mass effect from the tumor. He was biopsied and diagnosed in Jamaica with chondrosarcoma and had treatment with chemotherapy and radiation only. Further imaging with CTA and MRI showed a 10 × 12 × 13 cm partially calcified enhancing mass arising from the left nasal cavity with intracranial extension, left orbit, infratemporal and neck invasion. The first surgery consisted of trans-facial approach via Weber-Ferguson incision and maxillectomy with large debulking of the tumor. In the second stage, the intracranial portion was resected with left orbit exenteration and resection of the infratemporal fossa and neck component and dissection from the internal carotid artery. During the second procedure, reconstruction of the cranial base was performed with pericranial flap and left temporalis muscle flap for the orbit. In the third surgery, a vastus lateralis musculocutaneous free flap was harvested and used to fill the surgical defect separating the nasal cavity from the oral cavity. A multidisciplinary team formed by cranial base neurosurgeons, ENT, ophthalmologists, and plastic surgeons were involved. The tumor margins achieved were negative for tumor.

Conclusion: This case report presents a rare case of giant facial and skull base chondrosarcoma, which represents a challenge for resection and reconstruction by a multidisciplinary skull base team.