In this study, we report a case of a late-stage clival chordoma in a 29-year-old African American male and the unfortunate linear progression course since his initial diagnosis. Upon his initial encounter in 2020, radiation therapy did not offer any promising curative outcome. He was initially treated with a combination treatment of partial resection, radiation, and proposed oral imatinib, none of which modified the natural history and progression of his illness. Instead, these methods were performed as palliative measures to reduce the current size of the tumors and decrease growth rates to minimize his pain. Social issues acted as a contributory risk factor in his prognosis and due to the patient\'s socioeconomic barriers, he was not able to continue seeking available radiotherapy, leading to disease exacerbation. Poor adherence was noted with his follow-up. The risks of being affected by this disease are likely multifactorial and more reports of such cases need to be added to bridge this gap in the current literature. In addition, there is a gap in the current study of reports of such tumors found in diverse racial groups and in patients who are in their first few decades of life. Novel treatment strategies were reviewed, and it is expected they could generate assertive treatment guidelines.

Clivus chordomas are semi-malignant, but infiltratively growing tumors. Currently, a widely-accepted treatment concept encompasses maximal, but safe, surgical resection and radiotherapy. Caused by the size and the tumor extension, different surgical approaches, especially in recurrent cases, might be necessary.
Retrospective review of 50 patients on whom 70 surgeries were performed: 29 in primary and 41 in recurrent cases. Based on MRI images, all cases were asserted according to the size and the extension of the tumor. Used surgical approaches were evaluated. Postoperative complications, neurological function prior to and after the surgery, the extent of tumor resection on postoperative MR images were assessed and progression-free survival was calculated.
Tumor size was estimated as small (< 5 cm3) in 8, as medium (5-20 cm3) in 21, as large (20-100 cm3) in 17, and as giant (> 100 cm3) in 4 patients. Most frequently used surgical approaches in primary cases were the transsphenoidal one and midfacial degloving (51.7 % and 17.2 %, respectively). In recurrent cases, dependent on the tumor extension, transsphenoidal (21.9 %), retrosigmoidal (29.3 %), and pterional (19.5 %) approaches, as well as midfacial degloving (17.1 %) were used. Due to the vast tumor extension and infiltration, gross total or near total resection could be achieved in 12 patients (24 %), only. There was no mortality and no major complications in primary cases. In recurrences, however, postoperative hemorrhages and strokes emerged in 4.9 % and 7.1 %. Minor complications occurred in 17.1 % and were dominated by CSF leaks (12.2 %), both in primary in recurrent cases. While most cranial nerve impairments were caused by tumor infiltration of the cavernous sinus, and hence have not improved by treatment, the sixth nerve palsy as a consequence of tumor mass compression, could significantly be improved by surgery. Following surgery, patients were subjected to radiotherapy (68.9 % for primary cases, and 36.6 % for recurrences) mainly with carbon ions. Overall, 5-year progression-free survival was 44.7 %.
Caused by the heterogenous pattern of growth of clivus chordomas, surgical approaches should be chosen individually. Vast and infiltrative tumor extension constitute major limitations of surgical resection, and hence result in poor progression-frees survival.

BACKGROUND: Chordomas are rare tumors showing locally aggressive nature and high rates of local recurrences. Distant metastases are infrequently reported and cardiac metastases are extremely rare.
METHODS: A 32-year-old woman had been diagnosed with a large chordoma involving the clivus. She received a total removal of the tumor and adjuvant intensity-modulated radiotherapy. Four and a half years after the surgery, she presented with multifocal lesions in the ventricles and the right atrium, although an open thoracotomy revealed chronic inflammation. Two years after finding the cardiac lesions, thoracoscopic biopsy of the chest wall lesion revealed metastatic chordoma. She received palliative imatinib for 2 weeks before she died of tumor progression.
CONCLUSIONS: This case illustrates the metastatic potential of clivus chordoma, and the heart can be a site of metastasis.

Resection of clivus chordomas through extensive skull base approaches is associated with high mortality and morbidity even in experienced hands. We report our experience with endoscopic trans-sphenoidal surgery, or a \"wait-and-scan\" strategy in selected patients.
Ten patients were diagnosed with clivus chordomas during an 8-year period. Six patients underwent primary treatment with endoscopic trans-sphenoidal surgery, followed by adjuvant proton-beam therapy in three of these patients. Four patients with minor symptoms were followed-up untreated. Mean follow-up was 91 months.
Of the six patients operated on, total or gross total resection was achieved in four, partial resection in one and biopsy was taken in one. Preoperative cranial neuropathies resolved in three out of five patients, and no new cranial nerve palsies were encountered. Postoperative cerebrospinal fluid leak occurred in one patient. Four patients were initially followed-up without any treatment, and three of these have remained stable without tumour progression for a mean of 94 months. Due to a slow, though progressive growth of tumour, one patient was operated on after 80 months of initial observation.
The natural course of clivus chordomas has yet to be defined. The endoscopic trans-sphenoidal approach is a valid, minimally invasive alternative for the treatment of clival chordomas, and in selected patients a \"wait and scan\" strategy can be considered. Our long-term results show low mortality and good functional outcome. An endonasal endoscopic trans-sphenoidal approach should be a principal part of the armamentarium of surgeons treating clivus chordomas.

OBJECTIVE In the past decade, the role of the endoscopic endonasal approach (EEA) has relevantly evolved for skull base tumors. In this study, the authors review their surgical experience with using an EEA in the treatment of clival chordomas, which are deep and infiltrative skull base lesions, and they highlight the advantages and limitations of this ventral approach. METHODS All consecutive cases of chordoma treated with an EEA between 1998 and 2015 at a single institution are included in this study. Preoperative assessment consisted of neuroimaging (MRI and CT with angiography sequences) and endocrinological, neurological, and ophthalmological evaluations, which were repeated 3 months after surgery and annually thereafter. Postoperative adjuvant therapies were considered. RESULTS Sixty-five patients (male/female ratio 1:0.9) were included in this study. The median age was 48 years (range 9-80 years). Gross-total resection (GTR) was achieved in 47 cases (58.7%). On univariate analysis, primary procedures (p = 0.001), location in the superior or middle third of the clivus (p = 0.043), extradural location (p = 0.035), and histology of conventional chordomas (p = 0.013) were associated with a higher rate of GTR. The complication rate was 15.1%, and there were no perioperative deaths. Most complications did not result in permanent sequelae and included 2 CSF leaks (2.5%), 5 transient cranial nerve VI palsies (6.2%), and 2 internal carotid artery injuries (2.5%), which were treated with coil occlusion of the internal carotid artery without neurological deficits. Three patients (3.8%) presented with complications resulting in permanent neurological deficits due to a postoperative hematoma (1.2%) causing a hemiparesis, and 2 permanent ophthalmoplegias (2.5%). Seventeen patients (26.2%) have died of tumor progression over the course of follow-up (median 52 months, range 7-159 months). Based on Kaplan-Meier analysis, the survival rate was 77% at 5 years and 57% at 10 years. On multivariate analysis, the extent of tumor removal (p = 0.001) and the absence of previous treatments (p = 0.001) proved to be correlated with a longer survival rate. CONCLUSIONS The EEA was associated with a high rate of tumor removal and symptom control, with low morbidity and preservation of a good quality of life. These results allow for a satisfactory overall survival rate, particularly after GTR and for primary surgery. Considering these results, the authors believe that an EEA can be a helpful tool in chordoma surgery, achieving a good balance between as much tumor removal as possible and the preservation of an acceptable patient quality of life.

Local recurrence is common after surgical resection of clivus chordoma. We report the results of maximum surgical resection followed by intensity-modulated radiotherapy with simultaneous integrated boost (IMRT-SIB).
We reviewed 14 consecutive clivus chordoma cases undergoing postoperative IMRT-SIB using the institutional protocol between 2005 and 2013. Total and near-total resections were achieved in 11 patients (78.6 %), partial in 2 patients (14.3 %), and 1 patient (7.1 %) received RT for recurrent tumor after total resection. Gross residual or the high-risk area defined the planning target volume (PTV)1; PTV2 was the postoperative tumor bed plus a 3-5-mm margin, and PTV3 was PTV2 plus a 5-10 mm margin. A moderate hypofractionation schedule was used: doses to PTV1, PTV2 and PTV3 were 3.9 Gy, 3.15 Gy and 2.8 Gy through 15 fractions for the first two patients, and the rest received 2.5 Gy, 2.2 Gy and 1.8 Gy through 25 fractions. The biologically equivalent dose in 2-Gy fractions (EQD2) was 65-68 Gy for PTV1, 52-56 Gy for PTV2, and 44.3-44.8 Gy for PTV3.
Median follow-up was 41 months. Eight patients were free of disease for median 42.5 months (range 23-91 months), four patients had stable disease for median 60.5 months (range 39-113 months), and 1 patient showed partial response for 38 months after RT. Local progression was seen in one patient who received EQD2 67.8 Gy after partial resection. Estimated 5-year progression-free and overall survival rates were 92.9 %. Surgery improved the neurologic deficit in six patients, and IMRT-SIB was well tolerated without lasting toxicity.
Our experience suggests that maximum resection and high-dose IMRT-SIB can achieve local control without significant morbidities.

Clivus chordomas present a great challenge for neurosurgeons, and the prognosis is poor. To investigate bone invasiveness characteristics in regard to the prognosis of clivus chordomas, a retrospective study of 19 patients with primary clivus chordoma was performed. Clinical data, MRI, CT scans and follow-up data were examined, and the bone invasiveness of the chordoma was classified into two growth patterns (endophytic and exophytic) which were analyzed with regard to prognosis. The overall survival rate was 78.9% with radical surgery and adjuvant radiation, with a mean follow-up of 44.5months. There were 12 patients in the endophytic group and seven in the exophytic group, and the exophytic group exhibited a higher recurrence rate than the endophytic group (p=0.006). Chordomas with an exophytic growth pattern were more likely to recur than those with an endophytic growth pattern, and the surgical approach can be tailored according to each growth pattern.

The purpose of this prospective, observational study was to evaluate the management of skull base chordomas surgically resected via a 3D-endoscopic transnasal approach.
Thirteen consecutive patients were observed and only 9 were surgically treated using a 3D-endoscopic transnasal approach assisted by a novel 3D visualization system.
Nine consecutive male patients (mean age, 57.4 years) underwent exclusive 3D-endoscopic transnasal transsphenoidal resection of clival chordomas; gross total resection was achieved in 66.6% of cases (6 of 9 patients), near-total resection in 11.2% (1 of 9 patients), and partial resection in 22.2% (2 of 9 patients). The complications observed were 2 cases of postoperative cerebrospinal fluid (CSF) leaks and 1 case of temporary VI cranial nerve palsy.
No discomfort was recorded; when a dura opening was required, 3D vision allowed an accurate intradural sharp dissection and a precise repair of the skull base. © 2015 Wiley Periodicals, Inc. Head Neck 38: E1814-E1819, 2016.

Chordomas are rare malignant tumors arising from embryonic remnants of the primitive notochord, around which the skull base and vertebral column develop. They are locally aggressive but metastasize rarely. To our knowledge, this is the first reported case of synchronous intraosseous chordomas. A 32-year-old man presented with intermittent double vision secondary to a right-side abducent nerve palsy. Imaging revealed a clivus chordoma and an asymptomatic synchronous second primary chordoma in the fifth lumbar vertebra. Both chordomas were surgically excised: the clivus using the endonasal, endoscopic route and the L5 vertebra by total vertebral excision and replacement with a titanium prosthesis. The patient made an uneventful and complete recovery. We have modified our departmental practice as we believe that all patients diagnosed with chordoma should have magnetic resonance imaging of their entire spinal tract to exclude a second primary chordoma.