AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 5 ( 2 0 14 ) 43 1–4 3 4
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Clival chordoma of the nasal septum secondary to surgical pathway seeding☆ J. Peyton Hines, MD a,⁎, Mary G. Ashmead, MD b , Scott P. Stringer, MD, MS b a b
Department of Otolaryngology, Mayo School of Graduate Medical Education, Phoenix, AZ, USA Department of Otolaryngology and Communicative Sciences, University of Mississippi Medical Center, Jackson, MS, USA
ARTI CLE I NFO
A BS TRACT
Article history:
Educational objective: At the conclusion of this presentation, the participants should be
Received 19 December 2013
able to recognize seeding as a form of treatment failure in transseptal resection of clival chordomas. Objectives: The purpose is to present a case of implanted metastases in the nasal septum after a transseptal approach for resection of clival chordoma and to compare it with other reported cases in the literature. Study design: Case report and literature review. Methods: The clinical history, radiologic imaging, and pathology of a single patient are reviewed. Results: A 35-year-old female presented with a left intranasal mass that completely occluded the left nasal passage. The patient had a history of clival chordoma treated at an outside institution with multiple partial resections via a transseptal approach and postoperative Gamma Knife radiotherapy. A 2.5 cm mass in the left nasal cavity as well as a 4 cm sellar mass was identified on MRI. Biopsy of the left nasal mass confirmed the diagnosis of chordoma, which was presumed to be secondary to seeding from a previous resection attempt. The patient received no further treatment due to multiple comorbidities. Conclusions: Recurrence of clival chordoma due to seeding along the surgical pathway is an infrequent mechanism of treatment failure, with only rare cases documented in the literature. When deciding on the appropriate surgical approach, the surgeon must consider the risk of septal seeding during a transseptal approach. The emergence of transnasal endoscopic skull base approaches may reduce the likelihood of surgical pathway tumor seeding. © 2014 Elsevier Inc. All rights reserved.
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 [1–5].
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 [2,3]. Tumor seeding along the
☆ Each of the authors has contributed to, read and approved this manuscript.None of the authors have any conflict of interest, financial or otherwise.This manuscript is original and it, or any part of it, has not been previously published; nor is it under consideration for publication elsewhere. ⁎ Corresponding author at: 17608 N 56th Place, Scottsdale, AZ 85254, USA. Tel.: + 1 662 571 7129. E-mail address:
[email protected] (J.P. Hines).
0196-0709/$ – see front matter © 2014 Elsevier Inc. All rights reserved. http://dx.doi.org/10.1016/j.amjoto.2013.12.018
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pathway of surgical access is an infrequent mechanism for treatment failure, with only isolated cases documented in the literature [2]. 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
Fig. 1 – Axial T1-weighted MR image demonstrated a 4 cm hypointense seller mass along with a separate 2.8 × 2.5 cm hypointense mass.
Fig. 2 – Sagittal T2-weighted MR image characterized a hyperintense heterogeneously enhancing mass in the left nasal cavity with mass effect and septal deviation.
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). 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.
Fig. 3 – Histopathologic examination of the biopsy confirmed the presence of chordoma by characteristic physalliferous cells with pale bubbly cytoplasm in a myxoid stroma.
AM ER IC AN JOUR NA L OF OTOLARY NG OLOG Y –H EA D A N D N E CK ME D I CI N E AN D SUR G E RY 3 5 ( 2 0 14 ) 43 1–4 3 4
3.
Discussion
Chordomas are rare tumors that originate from undifferentiated embryonic remnants of the notochord and arise within the axial skeleton predominantly in the clivus, vertebral column, or sacrococcygeal region with an almost equal distribution [1–5]. These tumors have a suggested incidence of 0.8 per 1,000,000, with a peak incidence in the fourth through sixth decades of life, and 2:1 male predilection [1,4]. While considered histologically low-grade neoplasms with an indolent and slow-growing nature, chordomas are aggressive and locally invasive [2,4]. Because of these characteristics, they often do not present clinically until late stages of disease and have a high rate of recurrence after treatment [2,4]. Local recurrence is the most common form of treatment failure, occurring in 21%–29% of patients, and is considered the most important predictor of mortality [3,4]. Distant metastasis is uncommon except in very late-stage diagnosis, and tends to have a lesser effect on patient survival than does the local growth of tumor [3,4]. Tumor recurrence from seeding along the pathway of prior surgical access is becoming more recognized as a mechanism of treatment failure but is much less common than the aforementioned mechanisms. In a review of several case series, the combined incidence of tumor recurrence from seeding of the surgical pathway was reported in 1.9% of patients after surgery ± radiotherapy [3]. Fishbein et al. describe surgical pathway recurrence of chordoma as tumor occurring along the pathway of prior surgical access that is separated from the primary tumor or marginal zone by a substantial amount of normal tissue in the absence of distant metastasis or apparent lymphatic or perineural pathway of spread. Several criteria should be fulfilled in order to warrant classification as surgical pathway recurrence: (1) disease occurred along the route of entry for the original tumor resection, (2) disease is greater than 2.5 to 3.0 cm away from the margin of primary tumor to exclude marginal failures (3) no evidence of nodal or distant metastasis or perineural spread at the time of diagnosis, and (4) greater than 2 years have elapsed between the surgical removal and disease recurrence [2,3,6]. In this case, all of the above criteria were met. Our patient did have continued evidence of chordoma at the primary site, but the recurrent seeded mass was separated from the recurrent primary tumor by >2.5 cm. Intracranial chordomas represent about one third of cases and usually occur at the clivus. Management of clival chordoma is especially difficult because the surgical access, resectability, and delivery of radiotherapy are limited due to proximity to vital anatomical structures, specifically the brainstem, spinal cord, visual pathways, and carotid and basilar arteries [3]. Maximal tumor resection while preserving neurological function followed by postoperative irradiation is the most effective treatment of clival chordomas [4,7]. Currently, conventional chemotherapeutics have been found to be ineffective in the treatment of chordoma but there might be a role for new molecular-targeting agents in the future [4]. Multiple variations of radiation therapy have been utilized for treatment of intracranial chordomas, specifically conventional photon beam as well as particle beam therapy, intensity-modulated radiation therapy, and stereotactic de-
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livery techniques. The current accepted treatment of chordomas at most large cancer centers is proton-beam therapy following wide surgical excision [4,7]. For now, most studies suggest that proton-beam therapy is the optimal postoperative irradiation modality to improve duration of survival due to its ability to deliver higher doses to the tumor while decreasing injury to the surrounding neurologic structures [7]. The primary goal of postoperative radiation therapy, regardless of the type used, is to prevent recurrence at the primary site, which does not routinely include the surgical pathway [1,2,6,8]. With the use of stereotactic modalities and the increase in precision of more highly conformal radiation delivery, the field of radiation treatment is narrowed further and thus surgical pathway recurrence may become more common [2]. In order to potentially decrease the incidence of recurrence from seeding, consideration should be given to also cover the entire operative route in the radiation treatment field [1,2,6,8]. Intraoperative techniques have been proposed to decrease the incidence of seeding such as filling or coating the operative tunnel with fibrin glue and cotton patties after the surgical approach is obtained but prior to tumor removal; changing instrument, gloves, and drapes before graft harvest and closure; and copious rinsing of the operative field [5,6,8]. Since there has not been a proven method to decrease seeding during the procedure, the current consensus to control tumor recurrence within the surgical route relies upon meticulous surgical techniques and postoperative radiation [6]. Due to bone involvement and possible invasion of important soft-tissue structures both CT and MRI are usually employed for evaluation, treatment, and follow-up of intracranial chordomas. The gold standard imaging modality for post-surgical follow-up of these intracranial chordomas is MRI [1,2]. Just as in primary chordoma, the profound hyperintensity on T2 MRI is one characteristic that is particularly beneficial in differentiation tumor recurrence from nasal cysts, polyps, or postoperative changes such as granulation tissue formation or foreign body reaction [1,2,8]. In lesions that are more nonspecific, a definitive diagnosis should be reached with a tissue biopsy [2]. If chordoma recurrence is detected in the surgical pathway on follow-up, it should be treated aggressively with an emphasis on decreasing morbidity to the patient, just as primary site recurrence or metastatic lesions [8]. Effective management of surgical pathway recurrence ultimately relies on early diagnosis and treatment.
4.
Conclusion
In conclusion, recurrence of clival chordoma due to seeding along the surgical pathway is a rare form of treatment failure but may become more frequent with more conformal radiation fields. When deciding on the appropriate initial surgical approach and postoperative radiotherapy, the surgeon must consider the risk of septal seeding during skull base approaches. The emergence of endoscopic skull base approaches eliminates recurrence within the transseptal surgical pathway and should be studied for a concordant decrease in recurrence from seeding. In order to confirm the diagnosis of surgical pathway recurrence and initiate appropriate treatment, a high index of suspicion for tumor recurrence is warranted when a
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mass develops along the path of previous surgical access in patients with a history of chordoma resection. REFERENCES
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