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. 2021 Apr;14(4):84.
doi: 10.3892/mco.2021.2246. Epub 2021 Feb 26.

Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Chi-Yuan Yeh  1
Affiliations

Radiotherapy of extraosseous nasopharyngeal chordoma: A case report and literature review

Chi-Yuan Yeh. Mol Clin Oncol. 2021 Apr.

Abstract

Chordomas are slow-growing aggressive tumors that account for 1-4% of all bone tumors. The anatomical distribution of chordomas includes 50-60% in the sacrococcygeal region, 25-30% in the skull base and 15% in the mobile spine. Virchow was the first to describe and term these tumors as 'ecchordosis physaliphora' in 1857, and Muller established their notochordal origin in 1895. Extraosseous chordomas of the nasopharynx are very rare, and they exhibit similarities with other lesions of the nasopharynx, presenting as a soft tissue mass. Gross total resection combined with postoperative radiotherapy offers the best chance of long-term control. We herein present the case of a 63-year-old female patient with complaints of left temporal headaches, dizziness, left nasal obstruction, left maxillary area numbness, left ear hearing loss and swallowing difficulty. Computed tomography imaging examination revealed an 8.2x3.2x5.7-cm space-occupying lesion with central necrosis in the nasopharynx and oropharynx, partially occluding the pharyngeal lumen; the mass had infiltrated the left parapharyngeal space, the left medial and lateral pterygoid muscle and the left parotid gland, with bone erosion of the left mandible. The patient was diagnosed with extraosseous chordoma of the nasopharynx, conventional type, stage IIB. The patient underwent surgery and high-dose radiotherapy and local control of the chordoma was achieved. However, the patient succumbed to a lung metastasis. The details of the case are discussed, and a review of the current medical literature is presented to provide an updated discussion on the current status of nasopharyngeal chordoma research.

Keywords: chordoma; nasopharynx; radiotherapy; volumetric modulated arc therapy.

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Conflict of interest statement

The author declares no competing interests.

Figures

Figure 1
Figure 1
Histological images of the patient's tumor. (A) Tumor exhibited a bubbly vacuolated appearance of physaliphorous cells (arrows) (magnification, x400). (B) Hematoxylin and eosin staining of a cross section of the tumor, showing lobulation with extracellular myxoid matrix (magnification, x100).
Figure 2
Figure 2
Immunohistochemical staining of the chordoma (magnification, x100). The tumor stained positive for (A) p63, (B) cytokeratin, (C) epithelial membrane antigen, (D) S100 and (E) Ki67, whereas it was negative for (F) smooth muscle actin.
Figure 3
Figure 3
Gadoterate meglumine-enhanced T1-weighted magnetic resonance imaging showing a large left parapharyngeal mass extending from the nasopharynx to the oropharynx, with involvement of the skull base and left parotid gland. Red arrows are used to indicate the extent of the extraosseous nasopharyngeal chordoma.
Figure 4
Figure 4
RapidArc volumetric modulated arc therapy using 3 partial arc linear accelerator gantry rotation for irradiation of the tumor volume.
See this image and copyright information in PMC

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