Chordoma

Herbert B Newton MD (Dr. Newton, Director of the Neuro-Oncology Center at the Florida Hospital Cancer Institute, has no relevant financial relationships to disclose.)
Rimas V Lukas MD, editor. (Dr. Lukas has received honorariums from AstraZeneca as an advisory board member and AbbVie as a guest speaker.)
Originally released September 7, 1994; last updated March 1, 2017; expires March 1, 2020

This article includes discussion of chordoma; chordomas of the sacrum; chordomas of the skull base, clivus, and intracranial cavity; and chordomas of the true vertebrae. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

Overview

In this article, the author provides an in-depth review of the pathology, biology, clinical presentation, and treatment options for chordoma. These tumors are thought to derive from notochordal remnants and are locally invasive. Chordomas usually present at the skull base, but can arise anywhere along the spinal axis and pelvis. 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 continues to be investigated for therapeutic potential, especially molecular targeted agents.

Key points

 

• Chordomas are histologically non-anaplastic tumors that are locally invasive.

 

MRI typically shows a locally invasive, enhancing mass within the bones of the clivus and skull base, sacrum, or vertebral bodies.

 

• A small percentage of 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 radiotherapy should be considered in all cases; heavy ion therapy (eg, protons) may provide a slight therapeutic advantage.

Historical note and terminology

A chordoma of the clivus was first noted by both Virchow and Luschka in 1856 (Virchow 1857; Luschka 1864). 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" (Muller 1858). The first description of a symptomatic chordoma was made in 1864 by Klebs in a patient with a tumor of the spheno-occipital region (Klebs 1864). 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 (Ribbert 1894; Ribbert 1895). 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 (Congdon 1952).

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

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