Advances in the management of primary bone sarcomas of the skull base
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TOPIC REVIEW
Advances in the management of primary bone sarcomas of the skull base Idara Edem1 · Franco DeMonte1 · Shaan M. Raza1,2 Received: 19 February 2020 / Accepted: 10 April 2020 © Springer Science+Business Media, LLC, part of Springer Nature 2020
Abstract Skull base primary malignancies represent a heterogeneous group of histologic diagnoses and sarcomas of the skull base are specific malignant tumors that arise from mesenchymal cells and can be classified by site of origin into bony and soft tissue sarcomas. The most common bony sarcomas include: chondrosarcoma, osteosarcoma, chordoma, Ewing’s sarcoma. Given the relative rarity of each histologic diagnosis, especially in the skull base, there is limited published data to guide the management of patients with skull base sarcomas. An electronic search of the literature was performed to obtain key publications in the management of bony sarcomas of the skull base published within the last decade. This article is thus a review of the multi-disciplinary management principles of primary bony sarcomas of the skull base. Of note, there have been several recent advancements in the realm of skull base sarcoma management that have resulted in improved survival. These include advances in: imaging and diagnostic techniques, surgical techniques that incorporate oncologic surgical principles, conformal radiation paradigms and targeted systemic therapies. Early access to coordinated multi-disciplinary subspecialty care immediately at suspicion of diagnosis has further improved outcomes. There are several ongoing trials in the realms of radiation therapy and systemic therapy that will hopefully provide further insight about the optimal management of bony sarcomas of the skull base. Keywords Sarcoma · Skull base · Cancer · Surgery · Management
Introduction Primary bone sarcomas of the skull base reflect a heterogeneous group of diagnoses spanning the spectrum with regards to biologic behavior, local invasiveness and site of origin in the skull base. There are over 100 subtypes of sarcomas but they can be generally classified by site of origin, into bony and soft tissue sarcomas. According to the World Health Organization (WHO) Classification [1], there are numerous subtypes of bony sarcomas and these are shown in Table 1. The overall treatment of bony sarcomas of the skull base has undergone significant transformation over time, resulting in incremental improvements * Shaan M. Raza [email protected] 1
Departments of Neurosurgery, The University of Texas MD Anderson Cancer Center, Houston, TX, USA
Departments of Neurosurgery, The University of Texas MD Anderson Cancer Center, 1400 Holcombe Blvd, Rm FC7.2000, Unit 442, Houston, TX 77030, USA
2
in survival. Advances in imaging and surgical technique, in conjunction with an improved understanding for methods of disease spread have increased the application of evidencebased oncologic surgical principles for these lesions that were once thought only amenable to intralesional curettage. Surgical innovation has bee
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