1

Introduction

Chordomas are rare, relatively slow-growing tumors of notochordal origin that are aggressive, locally invasive, and radioresistant with a high rate of treatment failure . Although local recurrence continues to be the predominant form of treatment failure and best predictor of mortality, metastasis and recurrence along the surgical pathway are becoming more recognized, especially as stereotactic radiation becomes more common . Tumor seeding along the pathway of surgical access is an infrequent mechanism for treatment failure, with only isolated cases documented in the literature . We report seeding from a transseptal approach in a patient with both residual clival chordoma at the primary location and implanted seeded metastasis on the surgical pathway following prior partial resections and postoperative stereotactic radiotherapy. It is important for the otolaryngologist to recognize seeding of clival chordoma as a surgical complication from skull base approaches in order to choose appropriate initial management as well as to include seeding on the differential diagnosis when a mass arises in the pathway of prior surgical access.

2

Case presentation

A 35-year-old female presented to the University of Mississippi Medical Center Emergency Department with a 1-day history of acute onset upper and lower extremity weakness. Physical exam revealed a left hemiparesis as well as a left intranasal mass. Otolaryngology was consulted for evaluation of the nasal mass. Pertinent medical history included a clival chordoma treated at an outside hospital with multiple partial resections, most recently via a transseptal approach over 2 years prior, along with postoperative Gamma Knife radiotherapy. Outside records suggested only incomplete or subtotal resections for debulking purposes instead of intent to cure.

Nasal endoscopy revealed a gelatinous, submucosal soft tissue mass completely occluding the left nasal passage and causing mild contralateral septal deviation. The right nasal passage was unremarkable. Magnetic resonance imaging (MRI) demonstrated acute left posterior internal capsule and right lateral thalamic infarctions, as well as a 4-cm sellar mass encasing the bilateral internal carotids with partial destruction of the superior clivus, left sphenoid sinus, and left posterior ethmoid air cells. Radiology described a partially calcified heterogeneously enhancing mass compatible with known chordoma. There was an additional 2.8 × 2.5-cm T1 hypointense, T2 hyperintense heterogeneously enhancing mass in the left nasal cavity with mass effect and septal deviation. The two masses were confirmed by radiology to be separate entities without an obvious connection ( Figs. 1 and 2 ). Biopsy of the left nasal mass was performed and histopathologic examination confirmed the presence of chordoma with characteristic physalliferous cells with pale bubbly cytoplasm in a myxoid stroma ( Fig. 3 ).

Axial T1-weighted MR image demonstrated a 4 cm hypointense seller mass along with a separate 2.8 × 2.5 cm hypointense mass.

Sagittal T2-weighted MR image characterized a hyperintense heterogeneously enhancing mass in the left nasal cavity with mass effect and septal deviation.

Histopathologic examination of the biopsy confirmed the presence of chordoma by characteristic physalliferous cells with pale bubbly cytoplasm in a myxoid stroma.

The patient was diagnosed as having chordoma recurrence following implantation in the surgical pathway and was managed nonoperatively throughout her hospital course given multiple other neurologic comorbidities. Over the following several months, she had multiple complications including severe intraventricular hemorrhage, left upper and lower extremity spasticity, and respiratory failure. At last available follow-up, the patient was discharged to a long-term acute care facility due to ventilator dependence.