Chordoma of skull base presenting as nasopharyngeal mass

Abstract

While the nasopharynx is most commonly regarded by the otolaryngologist as a primary site of neoplastic involvement, it is also an avenue of spread of base-of-the-skull tumors presenting as bulging nasopharyngeal masses. Chordoma is a relatively rare tumor of the skull base and sacrum thought to originate from embryonic remnants of the notochord. Chordomas arising from the skull base/clivus are typically locally aggressive with lytic bone destruction. The optimal treatment may be photon/proton radiotherapy alone or combined with a gross total resection, when feasible. We report a case of intracranial chordoma presenting as nasopharyngeal mass.

Clival chordoma presenting as a parapharyngeal mass: a diagnostic challenge

“…As they present with compressive features and an intact overlying mucosa, they spectrum of clinical presentation varies from dysphagia to nasal obstruction and breathlessness, rarely even epistaxis. 1,5,6 Cell of origin of chordomas as described by Virchow are physalipherous cells. These are found along the midline from the skull base to the axial skeleton which corresponds to the notochordal elements.…”

“…While MRI is useful for soft tissue extension, CT is more helpful in gauging the bony lesion. 5 Many of the cases in the base of skull lesions and head and neck region have involvement of the clivus. 8 CT shows a hypoattenuated lobular with areas of bone erosions and calcification.…”

“…9 Histopathology shows a tumour having cells in cords and nests which are dispersed in a characteristic myxoid stroma separated by fibrous bands. 5 The cells are polyhedral with eosinophilic cytoplasm and are interspersed with a variable number of classic physalipherous cells, containing abundant vacuolated bubbly cytoplasm and a bland nucleus. Cellular atypia, mitotic figures and areas of necrosis may be present as was seen in the given case.…”

“…Best results have been found with the use of surgery along with adjuvant highdose proton RT. 5 After the surgical excision, disease free period of follow-up ranges from 12 months to 4.5 years. 11 The patient is planned for further management and is currently for follow up regarding the same.…”

A Parapharyngeal Soft Tissue Chordoma Presenting with Synchronous Cervical Lymph Node Metastasis: An Unusual Presentation

“…Chordomas rarely arise within soft tissue and are extremely uncommon in head and neck surgical practice [3]. Rarely, skull base chordomas may occur in an extraosseous location and present as a nasopharyngeal mass with an associated clival sinus tract [4,5]. Only a few cases of soft tissue chordomas have been reported in the head and neck region involving the lateral nasal wall [6], paranasal sinuses [7,8], oropharynx [9], parapharyngeal space [10], and soft tissues of the neck [11][12][13].…”