Chordomas are midline tumors of notochordal origin, occurring anywhere from the skull base to the coccyx. Although one-third of chordomas occur in the spheno-occipital region, to our knowledge only 1 case of jugular foramen chordoma with unusual extension into the neck has been reported in the literature to date. A 21-year-old woman presented with a 3-year history of a large neck mass and partly compensated lower cranial nerve symptoms of insidious onset. Imaging revealed a tumor involving the posterior cranial fossa and carotid space, with widening and erosion of the jugular foramen. Characteristic histopathologic findings and immunohistochemical staining confirmed the diagnosis. The tumor was removed by a combined retrosigmoid and lateral cervical approach. The patient was disease free 18 months after treatment.
Axial computed tomogram showing tumor encroaching in the right carotid space, with erosion of the transverse process and lamina of the axis.
T1-weighted magnetic resonance images. A, Coronal T1-weighted image showing lobulated tumor with widening of right jugular foramen and posterior fossa extension. B, Sagittal postcontrast fat suppression T1-weighted image showing variegated enhancement with nonenahancing areas of necrosis and anterior displacement of the internal carotid artery.
Microscopic features. A, Histologic features showing a moderately cellular tumor composed of clusters and cords of polygonal cells within a prominent myxoid matrix. The cells show minimally to mildly atypical nuclei and a moderate to abundant amount of cytoplasm that is frequently vacuolated (arrow) (physaliferous cells) (hematoxylin-eosin, original magnification ×200). B, Immunohistochemical staining shows diffuse nuclear expression of S-100 protein within the cords and clusters of cells, supporting the diagnosis of chordoma (original magnification ×40).
Thank you for submitting a comment on this article. It will be reviewed by JAMA Otolaryngology–Head & Neck Surgery editors. You will be notified when your comment has been published. Comments should not exceed 500 words of text and 10 references.
Do not submit personal medical questions or information that could identify a specific patient, questions about a particular case, or general inquiries to an author. Only content that has not been published, posted, or submitted elsewhere should be submitted. By submitting this Comment, you and any coauthors transfer copyright to the journal if your Comment is posted.
* = Required Field
Disclosure of Any Conflicts of Interest*
Indicate all relevant conflicts of interest of each author below, including all relevant financial interests, activities, and relationships within the past 3 years including, but not limited to, employment, affiliation, grants or funding, consultancies, honoraria or payment, speakers’ bureaus, stock ownership or options, expert testimony, royalties, donation of medical equipment, or patents planned, pending, or issued. If all authors have none, check "No potential conflicts or relevant financial interests" in the box below. Please also indicate any funding received in support of this work. The information will be posted with your response.
Some tools below are only available to our subscribers or users with an online account.
Download citation file:
Web of Science® Times Cited: 2
Customize your page view by dragging & repositioning the boxes below.
More Listings atJAMACareerCenter.com >
Enter your username and email address. We'll send you a link to reset your password.
Enter your username and email address. We'll send instructions on how to reset your password to the email address we have on record.
Athens and Shibboleth are access management services that provide single sign-on to protected resources. They replace the multiple user names and passwords necessary to access subscription-based content with a single user name and password that can be entered once per session. It operates independently of a user's location or IP address. If your institution uses Athens or Shibboleth authentication, please contact your site administrator to receive your user name and password.