Recurrent Multicompartment Chondrosarcoma Treated with Staged Multidisciplinary Resection and Adjuvant Proton Therapy: A Case Report

Introduction: Chondrosarcomas (CSA) of the head and neck represent a difficult insidious disease that is locally destructive and adherent, readily follows natural foramina across compartments, and encases critical structures. Surgical management is thus potentially extremely challenging in large or recurrent cases, often requiring combined approaches and multidisciplinary effort and limited to subtotal resection. Proton therapy (PT) has emerged as a potential but unproven adjuvant treatment with decreased rates of local recurrence but risks toxicity to healthy structures, particular in the optic and vestibulocholear apparatuses. We describe a case of extensive grade-II recurrent multicompartment chondrosarcoma managed with staged subtotal resection and separation of from sensitive structures prior to PT.

Case Report: A 51-year-old female with history of reported meningioma status post orbitozygomatic craniotomy 17 years prior after which she underwent no follow-up presented to our center with symptomatic significant recurrence. On examination, the patient had preserved hearing and multiple left sided cranial nerve (CN) palsies including a fixed and dilated pupil with no light sensation, oculoplegia, decreased sensation throughout all three trigeminal dermatomes and recently progressive ptosis. Imaging showed a large expansile extra-axial mass of the left middle fossa, petrous apex, and posterior fossa with internal carotid artery and lower CN encasement and jugular bulb occlusion. The decision was made to undergo staged resection, with the first procedure being a middle fossa transtentorial approach to the supratentorial and cranial aspects of the infralabyrinthine compartments. Extensive resection of the lesion and invaded structures including adjacent bone and the lateral wall and roof of the cavernous sinus was performed as the lesion was traced back along the ventral pons. A high-speed burr was used to resect involved bone as far down as the upper clivus during which the sphenoid sinus was entered and reconstructed on site, as well as in a second procedure via the endonasal corridor. The postoperative course was complicated by persistent rhinorrhea requiring revised endonasal reconstruction, and wound dehiscence requiring repair. Histopathology returned as grade 2 CSA. Stage two involved resection of extracranial components to their origin as the lesion extended through the jugular foramen. A multidisciplinary procedure with otolaryngology included auditory sparring transmastoid resection of high cervical and inferior temporal components, and an infralabyrinthine approach to involved areas of the inferior temporal bone up to the posterior fossa dura. The patient had experienced no complications postoperatively and elected for further management of residual intradural tumor via outpatient PT. Follow-up imaging 3 months later status post-PT showed virtually total resolution of remaining tumor without adverse effect.

Conclusion: We report a highly successful case of PT adjuvant treatment in residual infratentorial recurrent CSA following staged subtotal gross resection on short-term follow-up. A preservative presigmoid approach was selected to maintain hearing, particularly critical given total unilateral vision loss. We advocate for PT to effectively eliminate residual visualized tumor following open resection and spacing of the target from radiosensitive structures.

Publication History

Article published online:
12 February 2021