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  • Updated 11.23.2021
  • Released 09.07.1994
  • Expires For CME 11.23.2024

Chordoma

Introduction

Overview

In this article, the author provides an in-depth review of the pathology, biology, clinical presentation, and treatment options for chordoma. These tumors derive from notochordal remnants and are locally invasive. Chordomas usually present at the sacrum and skull base but can arise anywhere along the spinal axis. Aggressive surgical resection is the initial approach to treatment. However, in many cases the tumor cannot be completely removed. For residual tumors, radiotherapy is the most important treatment option. Chemotherapy and immunotherapy continue to be investigated for therapeutic potential.

Key points

• Chordomas are locally invasive bone cancers with metastatic potential.


• MRI typically shows a locally invasive, enhancing mass within the skull base, sacrum, and mobile spine.


• Identification of the brachyury gene is the standard for histological diagnosis.


• Some cases can progress to systemic metastases.


• Gross total resection should be attempted in all cases and is correlated with improved local control and overall survival.


• Postoperative proton beam radiotherapy should be considered in all cases.


• Immunotherapies are being actively investigated for use against chordoma.


• Current trials involving the use of vaccines against brachyury are ongoing.

Historical note and terminology

A chordoma of the clivus was first noted by both Virchow and Luschka in 1856 (202; 119). Virchow described the tumor as "ecchondrosis physaliphora spheno-occipitalis" and believed it was of cartilaginous origin. He used the term "physaliphora," meaning “bubble-bearing” because there were prominent cytoplasmic vacuoles. Muller suggested in 1858 that the origin of this tumor was the primitive notochord, the "chorda dorsalis" (136). The first description of a symptomatic chordoma was made in 1864 by Klebs in a patient with a tumor of the spheno-occipital region (101). In 1894 Ribbert was the first to use the term "chordoma," and further characterized Muller's theory by producing experimental chordomas after releasing tissue of notochordal origin from the nucleus pulposus of rabbits (160; 161). The tumors produced in these experiments were histologically similar to de novo chordomas. The experiments of Ribbert were replicated by Congdon in 1952, using a similar rabbit model (34).

A "chondroid chordoma" subtype, which has lacunae of hyaline cartilage, was described in 1973 by Heffelfinger and colleagues (75).

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