Chordoma Research Articles

Disruptions in antigen processing and presentation machinery on sarcoma

BackgroundThe antigen processing machinery (APM) plays a critical role in generating tumor-specific antigens that can be recognized and targeted by the immune system. Proper functioning of APM components is essential for presenting these antigens on the surface of tumor cells, enabling immune detection and destruction. In many cancers, defects in APM can lead to immune evasion, contributing to tumor progression and poor clinical outcomes. However, the status of the APM in sarcomas is not well characterized, limiting the development of effective immunotherapeutic strategies for these patients.MethodsWe investigated 126 patients with 8 types of bone and soft tissue sarcoma operated between 2001–2021. Tissue microarrays mapped 11 specific areas in each case. The presence/absence of APM protein was determined through immunohistochemistry. Bayesian networks were used.ResultsAll investigated sarcomas had some defects in APM. The least damaged component was HLA Class I subunit β2-microglobulin and HLA Class II. The proteasome LMP10 subunit was defective in leiomyosarcoma (LMS), myxoid liposarcoma (MLPS), and dedifferentiated liposarcoma (DDLPS), while MHC I transporting unit TAP2 was altered in undifferentiated pleomorphic sarcoma (UPS), gastrointestinal stromal tumor (GIST), and chordoma (CH). Among different neoplastic areas, high-grade areas showed different patterns of expression compared to high lymphocytic infiltrate areas. Heterogeneity at the patient level was also observed. Loss of any APM component was prognostic of distant metastasis (DM) for LMS and DDLPS and of overall survival (OS) for LMS.ConclusionSarcomas exhibit a high degree of defects in APM components, with differences among histotypes and tumoral areas. The most commonly altered APM components were HLA Class I subunit β2-microglobulin, HLA Class I subunit α (HC10), and MHC I transporting unit TAP2. The loss of APM components was prognostic of DM and OS and clinically relevant for LMS and DDLPS. This study explores sarcoma molecular mechanisms, enriching personalized therapeutic approaches.

A single-center experience of the upright proton therapy for skull-base chordomas and chondrosarcomas: Updated results

AimTo access efficacy and safety of the upright proton therapy for the skull-base chordomas and chondrosarcomas. Materials and methodsThe study encompasses single-center experience of proton therapy in chordomas (CA) and chondrosarcomas (CSA) of skull-base localization. We evaluate overall survival, local control and toxicity. Tumor response was assessed in accordance with RANO criteria. Treatment-related toxicity was evaluated with the help of CTCAE v 5.0 scale. ResultsProton therapy in the upright position was utilized for 51pts (patients) with CA-CSA (40 pts with chordoma and 11pts with chondrosarcoma) at the A. Tsyb Medical Radiological Research Center in 2016–2023. Median tumor volume constituted 30 cm3 (IQR (interquartile range) 15–41 cm3). Median total dose was 70 GyRBE. Median number of fractions was 35. Overall survival (OS) at 1-, 2- and 3-year rates reached 98.0 %, 88.6 % and 82.7 %, respectively. Median follow-up time was 36 months. The 1-, 2- and 3-year local control (LC) rates constituted, respectively, 98 %, 78.6 % and 66.3 %. Prior surgery showed statistically significant association with better prognosis (p = 0.023). Brainstem-to-tumor dose coverage compromise became the major pattern of LC failure (p = 0.03). The late radiation toxicity reactions included temporal lobe necrosis grade 2 in 2 pts, xerostomia grade 1 in 1pt, radiation cataract grade 2 in 1pt and persistent headache grade 2 in 4 pts. Severe late toxicity reactions were observed in 2 cases (4 %): 1 myelitis grade 3 and brainstem damage grade 5 in 1pt. ConclusionLocal control was achieved in the majority of patients receiving the scanning-beam upright proton therapy for skull-base CA-CSA. The LC rates after a surgery-radiotherapy combination treatment were higher compared with irradiation alone. Pattern of failure is mostly brainstem-tumor dose compromise. The high OS and LC rates were accompanied by low toxicity incidence. Even in complex case of the skull base CA-CSA upright proton therapy shows promising clinical outcomes.

Long-term outcome of adjunctive Gamma Knife radiosurgery in skull-base chordomas and chondrosarcomas: An Indian experience

Skull-base chordomas (CD) and chondrosarcomas (CS) are locally-invasive tumors, have similar clinical presentations, while differing in their nature of growth and outcomes. In this study, we compare the long-term outcomes of Gamma Knife Radiosurgery (GKRS) as an adjunctive treatment modality for residual skull-base CD and CS. A retrospective analysis of clinico-radiological, pathological, radiotherapeutic and outcome data was carried out in patients who underwent adjunctive GKRS for residual skull-base CD and CS at P D Hinduja Hospital, Mumbai, between 1997 and 2020. All 27 patients included had either histopathologically proven CD (20 patients) or CS (7 patients). Brachyury immunohistochemistry in CD specimens gave 70.6% positivity. Total sessions of GKRS in CD and CS groups were 22 and 7, respectively. Mean tumor volume and mean margin dose in CD group were 6.53 ± 4.18 cm3 and 15.95 ± 1.49 Gy respectively, while for CS group, they were 4.16 ± 2.79 cm3 and 18.29 ± 3.15 Gy. With mean follow-up periods of 5.25 ± 4.73 years and 6 ± 2.07 years respectively, the CD and CS groups showed 5-year progression free survival (PFS) of 56.8% and 57.1%, and a 5-year overall survival (OS) of 82.1% and 100%. Sub-group analysis in both CD and CS groups revealed a better 5-year PFS with the following factors – CS histopathology, patient age < 45 years, margin dose > 16 Gy, tumor volume < 7 cm3 (p-value < 0.05), gross total resection, and brachyury positivity. Adjunctive radiotherapy for skull-base CD and CS holds promise.

Outcomes of osteosarcoma, chondrosarcoma and chordoma treated with image guided-intensity modulated radiation therapy

Background & purposeTo evaluate the efficacy and toxicity of dose-escalated image guided-intensity modulated radiation therapy (IG-IMRT) in osteosarcoma (OGS), chondrosarcoma (CS) and chordoma (CH) of head and neck (H&N) and pelvis. Methods and MaterialsIn this prospective non-randomized study, 65 patients of H&N or pelvic OGS (24), CS (7) and CH (34) mandating definitive or post-operative radiotherapy from May 2013 to December 2018 were included. Radiotherapy doses in definitive setting were 72.0 Gy for CH and 70.2 Gy for OGS and CS; while in post-operative setting it was 66.6 Gy and 64.8 Gy respectively (at 1.8 Gy per fraction). ResultsPlanned doses of radiotherapy could be completed in 61 (93.8%) patients; with grade III or higher acute and late toxicities of 3% and 0% respectively. With a median follow-up of 52 (range 6–92) months, the five-year actuarial local control (LC) rates were 66% in OGS, 38.1% in CS and 75.9% in CH; while cause-specific survival (CSS) rates were 54.7%, 64.3% and 92.2% respectively. There was no statistically significant difference in outcomes for patients receiving definitive and post-operative radiotherapy. Locally controlled disease at first follow-up after radiotherapy was associated with improved CSS and OS in CS (p = 0.014) and CH (p < 0.001). Radiotherapy resulted in significant and sustained improvement in Musculoskeletal tumour society (MSTS) score and reduction in pain score. Salvage re-irradiation was feasible in local progression after radiotherapy, with good outcomes and tolerability. ConclusionDose-escalated IG-IMRT results in good LC & functional improvement with minimal toxicity in OGS, CS and CH.

Cervical chordomas: multicenter case series and meta-analysis.

En bloc spondylectomy is the gold standard for surgical resection of sacral chordomas (CHO), but the effect of extent of resection on recurrence and survival in patients with CHO of the cervical spine remains elusive. MEDLINE, Embase, Scopus, and Cochrane were systematically reviewed. Patients with cervical CHO treated at three tertiary-care academic institutions were reviewed for inclusion. We performed an individual participant data meta-analysis to assess the overall survival (OS) and progression free survival (PFS) after en bloc-gross total resection (GTR) and intralesional-GTR compared to subtotal resection (STR). We then performed an intention-to-treat analysis including all patients with attempted en bloc resection in the en bloc group, regardless of the surgical margins. There was a total of 13 series including 161 patients with cervical CHO, including our current series of 22 patients. GTR (en bloc-GTR + intralesional-GTR) was associated with a significant decrease in the risk of local progression (pooled hazard ratio (PHR) = 0.22; 95% CI 0.08-0.59; p = 0.003) and risk of death (PHR 0.31; 95%; CI 0.12-0.83; p = 0.020). A meta-regression analyses determined that intralesional-GTR improved PFS (PHR 0.35; 95% CI 0.16-0.76; p = 0.009) as well as OS (PHR 0.25; 95% CI 0.08-0.79; p = 0.019) when compared to STR. En bloc-GTR was associated with a significant reduction in the risk of local progression (PHR 0.06; 95% CI 0.01-0.77; p = 0.030), but not a decreased OS (PHR 0.50; 95% CI 0.19-1.27; p = 0.145). Our intention-to-treat analyses revealed a near significant improvement in OS for the en bloc group (PHR: 0.15; 95% CI 0.02-1.22; p = 0.054), and nearly identical improvement in PFS. Radiation data was not available for the studies included in the meta-analysis. This is the first and only meta-analysis of patients with cervical CHO. We found that both en bloc-GTR and intralesional-GTR resulted in improved local tumor control when compared to STR.

A novel energy layer optimization framework for spot-scanning proton arc therapy.

Spot-scanning proton arc therapy (SPAT) is an emerging modality to improve plan conformality and delivery efficiency. A greedy and heuristic method is proposed in the existing SPAT algorithm to select energy layers and sequence energy switching with gantry rotation, which does not promise optimality in either dosimetry or efficiency. We aim to develop a method to solve the energy layer switching and dosimetry optimization problems in an integrated framework for SPAT. In an integrated approach, energy layer optimization for spot-scanning proton arc therapy (ELO-SPAT) is formulated with a dose fidelity term, a group sparsity regularization, a log barrier regularization, and an energy sequencing (ES) penalty. The combination of L2,1/2-norm group sparsity regularization and log barrier function allows one energy layer being selected per control point. The ES regularization term sorts the delivery sequence from high energy to low energy to reduce the total energy layer switching time (ELST) and subsequently the total delivery time. Within the ES penalty, the gradient of layer weights between adjacent beams is first calculated along beam direction and then along energy direction. The gradients indicate energy switch patterns between two adjacent beams. The time-wise costly energy switch-up is more heavily penalized in the ES term. This ELO-SPAT method was tested on one frontal base-of-skull (BOS) patient, one chordoma (CHDM) patient with a simultaneous integrated boost, one bilateral head-and-neck (H&N) patient, and one lung (LNG) patient. We compared ELO-SPAT with intensity-modulated proton therapy (IMPT) using discrete beams and SPArc by Ding et al. For the two arc algorithms, both the plans with and without energy sequencing were created and compared. Energy layer optimization for spot-scanning proton arc therapy reduced the runtime of optimization by 84% on average compared with the greedy SPArc method. In both the ELO-SPAT plans with and without ES, one energy layer per control point was selected. Without ES regularization, the energy sequence was arbitrary, with around 40-60 switch-up for the tested cases. After adding ES regularization, the number of energy switch-up was reduced to less than 20. Compared with the energy sequenced SPArc plans, the ELO-SPAT plans with ES led to 24% less total ELST for synchrotron plans and 14% less for cyclotron plans. Both the ELO-SPAT and SPArc plans achieved better sparing compared with the IMPT plans for most Organs-at-risks (OARs), with or without ES. Without ES, the ELO-SPAT plans achieved further improvement of the OARs compared with the SPArc plans, with an averaged reduction of OAR [Dmean, Dmax] by [1.57, 3.34] GyRBE. Adding the ES regularization degraded the plan quality, but the ELO-SPAT plans still had comparable or slightly better sparing than the SPArc plans with ES, with an averaged reduction of OAR [Dmean, Dmax] by [1.42, 2.34] GyRBE. We developed a computationally efficient spot-scanning proton arc optimization method, which solved energy layer selection and sequencing in an integrated framework, generating plans with good dosimetry and high delivery efficiency.

Diagnostic value of SPECT/CT tomographic fusion imaging and magnetic resonance imaging in primary bone tumors

Objective To study the effects of single photon emission computed tomography/computed tomography (SPECT/CT) and magnetic resonance imaging (MRI) in the diagnosis of primary bone tumors. Methods A total of 104 patients with primary bone tumors in Zibo Central Hospital hospital from January 2016 to January 2018 were enrolled in this study. SPECT/CT and MRI examinations were performed on these patients, and the results of the examinations were compared with the results of surgical pathology. The diagnostic efficacy of the two methods for primary bone tumors were compared. Results ⑴Results of pathological examination: 70 cases were malignant tumors, and 34 cases were benign tumors; results of SPECT/CT: 72 cases were malignant tumors, and 32 cases were benign tumors; MRI: 76 cases were malignant tumors, and 28 cases were benign tumors; there was no difference between the two methods in the diagnosis of primary bone tumors. ⑵ The sensitivity, specificity, accuracy, positive predictive value and negative predictive value of SPECT/CT were 92.86%, 79.41%, 88.46%, 90.28% and 84.38% respectively, slightly higher than those of MRI (91.43%, 64.71%, 82.69%, 84.21% and 78.57%), but the difference was not statistically significant (P>0.05). ⑶ There was a high consistency between SPECT/CT and MRI in the diagnosis of primary bone tumors (Kappa value=0.613). ⑷ SPECT/CT is more accurate in the diagnosis of osteosarcoma, giant cell tumor, myeloma and fibrous dysplasia, while MRI is more accurate in the diagnosis of osteosarcoma, chordoma, Ewing's sarcoma and schwannoma. Conclusions SPECT/CT fusion imaging and MRI have high sensitivity and accuracy in the diagnosis of primary bone tumors, and the results of the two diagnostic methods have high consistency. SPECT/CT has a high accuracy in the diagnosis of ossification and calcification, while MRI has a high accuracy in the diagnosis of bone tumors in bone soft tissue and nervous system. Therefore, the joint application of the two methods can make up for each other's shortcomings and make the diagnosis of primary bone tumor more accurate. Key words: Tomography, emission-computed, single-photon; Magnetic resonance imaging; Bone neoplasms

Clival Chordomas: Contemporary Results.

Clival Chordomas: Contemporary Results.

Pathological feature change and prognostic role of primary and local recurrent chordoma in skull base

Objective To investigate the pathological characteristics and changing trend in primary and local recurrent skull base chordoma, and to analyze their correlation with the outcomes. Methods A total of 89 samples of primary and local recurrent tumors pathologically confirmed from 38 patients who were treated in Neurosurgery Department of Beijing Tiantan Hospital, Capital Medical University between February 2005 and December 2014 were retrospectively enrolled into this self-control study. After HE staining, the pathological features such as nuclear atypia, mitosis, matrix ratio, necrosis, bone invasion and pathological subtypes were analyzed. The expressions of p53, Ki-67, EGFR, VEGFR and TRAF6 were detected by immunohistochemical staining. The changes of pathological indexes as above in primary and recurrence stages were compared. Cox analyses were used to analyze the effects of above pathological indexes on the progression-free survival (PFS) of patients with primary tumor, and the effects of the changes regarding those factors on the overall survival (OS). Results Ten cases (35.7%) of classical subtype changed to myeloid subtype, while none of chondroid chordoma changed to myeloid subtype after recurrence. Twelve cases (31.6%) demonstrated necrosis companied with tumor recurrence. Twenty-five cases (73.5%) of primary tumor showed high-expression of TRAF6, and 19 cases (50.0%) had decreased expression intensity of TRAF6 after tumor recurrence. There were 16 cases (42.1%) and 15 cases (39.5%) with upregulation of P53 and EGFR respectively. Necrosis (HR=7.1, 95% CI: 1.4-37.1, P=0.006), pathological subtype (HR=3.9, 95% CI: 1.2-7.2, P=0.040) and TRAF6≥3 grade (HR=0.2, 95% CI: 0.1-0.5, P<0.001) in primary tumors were directly related to PFS, and TRAF6≥3 grade (HR=0.2, 95% CI: 0.1-0.5, P<0.010) was an independent protective factor for PFS. There was no significant difference in the OS rate among the above pathological changes. Conclusions Classical chordoma shows malignant transformation to the myeloid subtype, which is not found in chondroid subtype. The expression of TRAF6 decreases gradually with tumor recurrence, and its high expression in primary tumor is an independent protective factor for PFS. Key words: Chordoma; Skull base neoplasms; Pathology; TNF receptor-associated factor 6; Prognosis

Application of microprobe vascular Doppler in the operation of skull base lesions

Objective To investigate the effect of microprobe vascular Doppler (MVD) on the operation of skull base lesions. Methods From September 2017 to September 2018, 13 patients with skull base lesions at Neurosurgery Department of the first Affiliated Hospital of Soochow University were retrospectively analyzed. The 13 cases included 6 cases of meningioma in Sellar region, 2 cases of meningioma in petroclival region, 3 cases of pituitary adenoma of Knosp Ⅲ-Ⅳ grade, 1 case of chordoma in Sellar clival region, and 1 case of invasive growth of granulation tissue in intraorbital, skull base, sphenoid and ethmoid sinus after Aspergillus infection. The surgical methods included neuroendoscopic transnasal approach and microsurgical resection under craniotomy microscope. MVD was applied in all operations of this series. Medical imaging examination and follow-up were performed after operation. Results Among the 13 patients, total resection of meningioma in Sellar region was performed in 6 cases and subtotal resection in 2, total resection of Knosp grade Ⅲ-Ⅳ pituitary adenoma was conducted in 2 and subtotal resection in 1. Total resection was performed in 1 case of invasive growth of granulation tissue in intraorbital, skull base, sphenoid and ethmoid sinus after Aspergillus infection and 1 case of chordoma in Sellar base clival region. Uncorrected left eye vision was reported in the patient with Aspergillus infection post surgery. Postoperative symptoms of the other patients were improved. The prolactin level of 1 patient with prolactin adenoma was more than 204 ng/ml before operation. At 2 months post operation, the prolactin level was 105.66 ng/ml after oral bromocriptine combined with radiotherapy. The last reexamination was 11.3 ng/ml without hypophysis dysfunction. Six patients had varying degrees of electrolyte disorder after operation. Four patients had fever after operation and all returned to normal before discharge from hospital after treatment. All patients were followed up for (16.1±3.3) months(10-22 months), and there was no recurrence in imaging examination. Conclusion With the aid of MVD during the surgical resection of invasive growth lesions and large space occupying lesions in the skull base, the important blood vessels invaded, wrapped, pushed and displaced by the lesions can be monitored in real time, which might help avoid the disastrous consequences during and after operation and reduce the risk of surgery. Key words: Skull base neoplasms; Microsurgery; Natural orifice endoscopic surgery; Microprobe vascular Doppler

Advantages of carbon ion radiotherapy and its application in tumor therapy

Compared with proton and photon, heavy ion radiotherapy has unique physical and biological advantages, which enables it to kill tumor tissues to the greatest degree and protect surrounding normal tissues as much as possible. Carbon ion is recognized as the most suitable heavy ion for radiotherapy at present. Carbon ion radiotherapy in chordoma, head and neck cancer, non-small cell lung cancer, prostate cancer, cervical cancer and other malignant tumor treatment advantages have been preliminarily reflected. Therefore, understanding the characteristics of carbon ion radiotherapy and its application in tumor therapy will help clinicians make better clinical decisions in the future. Key words: Heavy ion radiatherapy; Neoplasms; Carbon ion

Children and Adults With Chordoma

Children and Adults With Chordoma

MiR-1290 inhibits chordoma cell proliferation and invasion by targeting Robo1

Background: Chordoma is a low-grade aggressive bone tumor with a high local recurrence. MicroRNAs (miRNAs) have been reported to play crucial roles in the development of chordoma. Our previous study has shown miR-1290 is associated with muscle invasion and the prognosis of chordoma. However, the underlying mechanism of miR-1290 in chordoma remains unclear. In this study, we aimed to explore the function of miR-1290 in the biological behaviors of chordoma. Methods: Sixteen sacral chordoma samples and 10 fetal nucleus pulposus specimens were collected for the detection of miR-1290 and Robo1 at the First Affiliated Hospital of Soochow University. Bioinformatic analysis and a luciferase reporter assay was used to verify the interaction between miR-1290 and the target gene robo1 in chordoma. Effects of miR-1290 expression on chordoma cell proliferation and invasion were explored by clone formation and Transwell assay in vitro. The underlying mechanisms of miR-1290 and Robo1 in chordoma cell proliferation and invasion were also explored in the U-CH1 cell line. Results: In vitro functional analysis, including clone formation, and Transwell assays indicated overexpression of miR-1290 significantly suppressed chordoma cell proliferation and invasion. Bioinformatic analysis revealed Robo1 as a potential target of miR-1290, and luciferase reporter assays demonstrated the association between miR-1290 and the Robo1 gene in U-CH1 cells. Robo1 was further confirmed to be up-regulated in chordoma tissues by immunohistochemistry (IHC), which is negatively correlated with miR-1290 expression in chordoma tissue. Additionally, we found down-regulation of miR-1290 could induce the expression of Robo1 in chordoma cells, while the elevation of miR-1290 expression could inhibit Robo1 expression in chordoma cells. Conclusions: miR-1290 inhibits chordoma cell proliferation and invasion by negatively regulating the Robo1 gene.

Solamargine inhibits proliferation and promotes apoptosis of CM-319 human chordoma cells through suppression of notch pathway

Background: Solamargine (SM), which represents a natural steroid alkaloid glycoside compound and a cytotoxic agent, has been proved to enhance the sensitivity of lung cancer cells to tumor necrosis factors (TNFs). In this study, we aimed to investigate the roles and mechanisms of SM in chordoma. Methods: Cell viability, proliferation, apoptosis and cell cycle were measured by cell counting Kit-8 (CCK-8) assay, 5(6)-carboxyfluorescein diacetate succinimidyl ester (CFSE) labeling and flow cytometry (FCM), respectively. Western blot and quantitative real-time reverse transcription PCR (qRT-PCR) assays were performed to detect the expressions of related mRNAs and proteins. Results: The results revealed that SM distinctly suppressed the proliferation of CM-319 cells. SM significantly induced the CM-319 cells apoptosis through up-regulating the expression levels of Caspase-3/8/9. The cell cycle of CM-319 cells was blocked by SM in G1 phase. Moreover, SM could significantly suppress the Notch pathway in CM-319 cells. Conclusions: In conclusion, SM suppressed the proliferation and enhanced the apoptosis ability of CM-319 cells via suppressing the Notch pathway. The results suggested that SM might be a novel therapeutic agent and supported the utilization of SM in chordoma.

Research Advances in Molecular Regulatory Mechanism of Chordoma

Research Advances in Molecular Regulatory Mechanism of Chordoma

Analysis of misdiagnosis of primary skull base chordomas: A case-control study

Objective To investigate the clinical and imaging features of misdiagnosed cases of skull base chordomas and to improve the rate of correct diagnosis. Methods A retrospective analysis was conducted on 155 cases of skull base chordomas that were consecutively admitted to Department of Neurosurgery, Beijing Tiantan Hospital, Capital Medical University from February 2005 to December 2014. We measured the signal intensity ratio in T2WI and enhanced T1WI sequences (RT2 and REN). The demographics, radiological and pathological features were compared between the misdiagnosed group (n=50) and control group (n=105). Further inter-group comparison and analysis were conducted about the tumor location and common features of misdiagnosed cases. Results Among the 50 cases in misdiagnosed group, 19 cases were misdiagnosed as schwannomas, 12 as epidermoid cysts, 8 as meningiomas, 5 as other osseous tumors, 3 as pituitary adenomas and 3 as cavernous malformations. The mean RT2 and REN in misdiagnosed group were 2.49 (0.79) and 0.99 (0.77), respectively, which were not statistical different from those in control group (P>0.05). However, there were significant differences when the tumor location (P<0.001) and extent of bone invasion (P<0.001) were concerned. Multivariate logistic regression showed that tumors in spheno-petrous (SP) (P=0.001) and petrous-occipital (PO) (P=0.008) regions were easily misdiagnosed compared with spheno-clival (SC) type. Exophytic chordoma was significantly easier to be misdiagnosed compared with endophytic type (P<0.001). Conclusions Skull base chordomas seem to demonstrate varying imaging features and those located in the lateral skull base and presented in the exophytic status are more prone to be misdiagnosed. The most common mimicking tumors are schwannomas in the lateral skull base and epidermoid cysts in the midline area. Signal intensity ratio on MRI could provide quantitative reference, which needs further research on its value in the diagnosis and differential diagnosis of chordomas. Key words: Chordoma; Skull base neoplasms; Diagnostic errors; Magnetic resonance imaging; Signal intensity ratio

Letter to the Editor Regarding “Comparison of the Effectiveness of Radiotherapy with Photons and Particles for Chordoma After Surgery: A Meta-Analysis”

Letter to the Editor Regarding “Comparison of the Effectiveness of Radiotherapy with Photons and Particles for Chordoma After Surgery: A Meta-Analysis”

Endoscopic endonasal surgery of recurrent skull base chordomas

Objective To discuss the strategy and technique of endoscopic endonasal surgery of recurrent skull base chordomas. Methods The data of 123 patients with recurrent skull base chordomas were retrospectively studied who underwent surgical treatment between August 2007 and March 2018 at Neurosurgery Department of Beijing Tiantan Hospital, Capital Medical University. Recurrent skull base chordomas were classified into 4 types based on the tumor location: total clivus type (n=43) superior-middle clivus type (n=51), middle-inferior clivus type (n=8), and extensive type (n=21). All tumors were resected through EEA (endoscopic endonasal approach). Results Among all 123 cases, gross total resection was achieved in 39 (31.7%) cases, near-total resection in 58 (47.2%) and partial resection in 26 (21.1%). Following the surgery, cerebrospinal fluid leakage occurred in 9 cases and secondary surgical repair was performed. Intracranial infection occurred in 3 patients among the 9 cases who received the secondary repair. Among them, 2 patients recovered and 1 died. Postoperative new cranial nerve palsy occurred in 14 cases. Intraoperative internal carotid artery rupture occurred in 3 cases. The rupture in 2 cases was minor and complete hemostasis was achieved during operation. The rupture in 1 case was severe and endovascular covered stent was used for complete hemostasis. No new functional deficits was noted post operation for all 3 cases. Within 6 months post surgery, the improvement rates of hypopsia and visual field defects, diplopia, facial numbness and pain, tinnitus and hearing loss, dysphagia and hoarseness and cough, headache and dizziness, nasal obstruction, and dyskinesia were 47.6% (10/21), 32.3% (10/31), 41.7% (5/12), 30.0% (3/10), 25.0% (4/16), 86.6% (58/67), 100% (9/9) and 66.7% (8/12), respectively. Conclusions The resection of recurrent skull base chordomas seems difficult, in which endoscopic endonasal surgery might be more advantageous. Correct surgical strategy and techniques combined with the uses of neuronavigation, neurophysiological monitoring and Doppler ultrasound are the key factors for high-quality operation. Key words: Chordoma; Skull base neoplasms; Recurrence; Natural orifice endoscopic surgery

1526TiP - CDK4/6 inhibition in locally advanced/metastatic chordoma (NCT PMO-1601)

1526TiP - CDK4/6 inhibition in locally advanced/metastatic chordoma (NCT PMO-1601)

EGFR Inhibition in a Pretreated Sacral Chordoma: A Role for Erlotinib? Case Report and a Brief Review of Literature.

We describe the case of a 69-year old male with an EGFR- positive Imatinib refractory sacral chordoma with synchronous lung metastases, treated with erlotinib, a first-generation EGFR inhibitor. After disease progression following first-line Imatinib and a combination therapy with everolimus plus metformin, we made a challenge with an EGFR tyrosine kinase inhibitor (EGFR TKI), erlotinib. Despite a brief clinical benefit, the patient presented a rapid clinical deterioration leading to death, after 8 weeks of treatment.