Leveraging Imaging for Targeted Treatment

Skull Base Chordoma and Chondrosarcoma: Neuroradiologist’s Guide to Diagnosis, Surgical Management, and Proton Beam Therapy

Published Online:Sep 19 2024https://doi.org/10.1148/rg.240036

Abstract

Skull base chordoma and chondrosarcoma are distinct tumors that share important tenets of multimodality management, and treatment decisions should be undertaken in a multidisciplinary tumor board setting where radiologists, skull base surgeons, pathologists, and oncologists individualize recommendations.

Skull base chordomas and chondrosarcomas are distinct types of rare, locally aggressive mesenchymal tumors that share key principles of imaging investigation and multidisciplinary care. Maximal safe surgical resection is the treatment choice for each, often via an expanded endoscopic endonasal approach, with or without multilayer skull base repair. Postoperative adjuvant radiation therapy is frequently administered, usually with particle therapy such as proton beam therapy (PBT). Compared with photon therapy, PBT enables dose escalation while limiting damage to dose-limiting neurologic structures, particularly the brainstem and optic apparatus, due to energy deposition being delivered at a high maximum with a rapid decrease at the end of the penetration range (Bragg peak phenomenon). Essential requirements for PBT following gross total or maximal safe resection are tissue diagnosis, minimal residual tumor after resection, and adequate clearance from PBT dose-limiting structures. The radiologist should understand surgical approaches and surgical techniques, including multilayer skull base repair, and be aware of evolution of postsurgical imaging appearances over time. Accurate radiologic review of all relevant preoperative imaging examinations and of intraoperative and postoperative MRI examinations plays a key role in management. The radiology report should reflect what the skull base surgeon and radiation oncologist need to know, including distance between the tumor and PBT dose-limiting structures, tumor sites that may be difficult to access via the endoscopic endonasal route, the relationship between intradural tumor and neurovascular structures, and tumor sites with implications for postresection stability.

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Article History

Received: Feb 8 2024
Revision requested: Mar 27 2024
Revision received: June 19 2024
Accepted: July 10 2024
Published online: Sept 19 2024

Vol. 44, No. 10

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Abbreviations

Abbreviations:
ADC	apparent diffusion coefficient
ANCT	atypical notochordal tumor
BNCT	benign notochordal tumor
CSF	cerebrospinal fluid
EEA	endoscopic endonasal approach
ESBR	endoscopic skull base reconstruction
FLAIR	fluid-attenuated inversion recovery
ICA	internal carotid artery
MTB	multidisciplinary tumor board
PBT	proton beam therapy

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