Embryonic Notochord Research Articles

Surgical management of skull base and spinal chordomas: A case series with comprehensive review of the literature

BackgroundChordomas are rare, slow growing, locally aggressive malignant bone tumors that arise from remnants of the embryonic notochord with variable presenting symptoms depending on tumor location. MethodsAll patients with craniospinal chordoma managed at our institution between 1982 and 2023 were retrospectively reviewed. Demographics, tumor characteristics, clinical course and treatment, and long-term neurological and survival outcomes were collected. Adjuvant radiotherapy (RT) was stratified into standard dose fractionated radiotherapy (standard XRT) for doses of 50 to 60 Gy at 1.8 Gy fractions or high dose hyperfractionated stereotactic radiotherapy (HD-FSRT) for doses of 60 to 81 Gy at 1.2-1.5 Gy fractions per treatment. Descriptive statistics, univariate analysis, Log-rank test, and Kaplan-Meier survival analysis were performed. ResultsA total of 37 patients were included in our cohort (mean age 46.0 ± 20.8 years; 22 male). Clival chordomas accounted for the majority of patients (56.8%), followed by vertebral (27%) and sacral (10.8%) chordomas. Thirty-five patients (94.6%) underwent gross total resection (GTR) or subtotal resection (STR), and 2 patients underwent excisional biopsy only. Postoperatively, functional status trended towards improvement (KPS: Preop- 80 [range 40–100] vs. Post op- 90 [60–100], p = .0911) and all patients either maintained or improved their neurological function. Median overall survival (OS) after diagnosis was 16.5 years. Age < 65, clival tumor location, post-operative Frankel grade E, and administration of adjuvant RT following initial STR significantly improved OS. OS of GTR patients was not significantly affected by adjuvant RT treatment. ConclusionsOur results show the best long-term survival outcomes for chordoma patients undergoing GTR of tumor tissue. Higher postoperative neurological function was significantly associated with OS, highlighting the importance of maximal but safe total tumor resection. Moreover, adjuvant RT improved long-term survival for patients that underwent STR but had no effect on survival outcomes for patients that underwent GTR.

The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study.

Chordoma, a rare malignant tumor originating from embryonal notochord remnants, exhibits high resistance to conventional treatments, making surgical resection imperative. However, the factors influencing prognosis specifically for cervical spine chordoma have not been clearly identified. We investigate the prognosis of cervical spine chordoma with factors influential in a nationwide multicenter retrospective study. This study included all patients diagnosed with cervical spine chordoma at 7 tertiary referral centers from January 1998 to March 2023, excluding those with clivus and thoracic spine chordomas extending into the cervical spine. Local recurrence (LR) was identified through follow-up magnetic resonance imaging, either as reappearance in completely resected tumors or regrowth in residual tumors. The study assessed LR and overall survival, analyzing factors influencing LR and death. Forty-five patients with cervical spine chordoma had a mean age of 46.4 years. Over a median follow-up of 52 months, LR and distant metastasis were observed in 21 (46.7%) and 4 patients (8.9%), respectively, and 16 patients (36%) were confirmed dead. The 5-year and 10-year cumulative LR rates were 51.3% and 60%, respectively, while the 5-year and 10-year survival rates were 82% and 53%. Age was the only significant factor affecting mortality (hazard ratio, 1.04; 95% confidence interval, 1.04-1.07; p=0.015). Notably, the degree of resection and adjuvant therapy did not statistically significantly impact local tumor control and mortality. This study, the largest multicenter retrospective analysis of cervical spine chordoma in Korea, identified age as the only factor significantly affecting patient survival.

Role of Proton Beam Therapy in Spinal Chordomas: A Narrative Review of The Literature.

Chordomas are rare malignant neoplasms arising from vestigial remnants of the embryonic notochord. Approximately 55-70% of chordomas develop within the vertebral column. Their affinity to develop within the bones of the axial skeleton and propensity to locally invade and recur makes them challenging candidates for complete surgical excision. Adjuvant therapies are hence necessary to improve outcomes; for which chemotherapy has been observed to be largely ineffective, owing to the tumour being resistant to it. Radiotherapy is the current adjuvant therapy of choice for chordoma management. Over the years, proton beam therapy (PBT) has been the subject of medical attention, given the dosimetric benefits it confers over traditional radiotherapy, allowing more concentrated radiation to be given to the target of interest and reducing damage to surrounding normal tissue. A review of the current literature reveals PBT offers significantly better outcomes when used as an adjuvant to maximal surgical resection rather than as a definitive therapy.

Matrix metalloproteinase Nas15 regulates the lumen formation and expansion in Ciona notochord

Lumen formation, as a key process of biological tube construction, is essential in various physiological processes such as nutrient and waste transporting, gas exchanging, and structural supporting. However, the mechanisms underlying tubular lumen development are still not fully understood. In the present study, we identified a matrix metalloproteinase, Nas15, which is enriched in the apical domain of the Ciona embryonic notochord. The expression level of the Nas15 gene significantly increased during notochord lumen formation and expansion. Nas15 loss-of-function resulted in abnormal notochord lumen expansion in Ciona embryos. Besides, yeast two-hybrid screening and CO-IP results indicated a Phosphatase 2 Catalytic Subunit Alpha (PPP2CA) physically interacted with Nas15. PPP2CA also involved in notochord lumen formation via localizing Nas15. Furthermore, we investigated the distribution of laminin in Nas15 disrupted embryos. In conclusion, our results revealed a mechanisms of how notochord cells regulating lumen expansion via metalloproteinase-mediated ECM localization. This findings provide insight into the mechanisms of tubular organ lumen formation and serve as a reference for research on human abnormal lumenogenesis diseases.

Proton versus photon adjuvant radiotherapy: a multicenter comparative evaluation of recurrence following spinal chordoma resection.

Chordomas are rare tumors of the skull base and spine believed to arise from the vestiges of the embryonic notochord. These tumors are locally aggressive and frequently recur following resection and adjuvant radiotherapy. Proton therapy has been introduced as a tissue-sparing option because of the higher level of precision that proton-beam techniques offer compared with traditional photon radiotherapy. This study aimed to compare recurrence in patients with chordomas receiving proton versus photon radiotherapy following resection by applying tree-based machine learning models. The clinical records of all patients treated with resection followed by adjuvant proton or photon radiotherapy for chordoma at Mayo Clinic were reviewed. Patient demographics, type of surgery and radiotherapy, tumor recurrence, and other variables were extracted. Decision tree classifiers were trained and tested to predict long-term recurrence based on unseen data using an 80/20 split. Fifty-three patients with a mean ± SD age of 55.2 ± 13.4 years receiving surgery and adjuvant proton or photon therapy to treat chordoma were identified; most patients were male. Gross-total resection was achieved in 54.7% of cases. Proton therapy was the most common adjuvant radiotherapy (84.9%), followed by conventional or external-beam radiation therapy (9.4%) and stereotactic radiosurgery (5.7%). Patients receiving proton therapy exhibited a 40% likelihood of having recurrence, significantly lower than the 88% likelihood observed in those treated with nonproton therapy. This was confirmed on logistic regression analysis adjusted for extent of tumor resection and tumor location, which revealed that proton adjuvant radiotherapy was associated with a decreased risk of recurrence (OR 0.1, 95% CI 0.01-0.71; p = 0.047) compared with photon therapy. The decision tree algorithm predicted recurrence with an accuracy of 90% (95% CI 55.5%-99.8%), with the lowest risk of recurrence observed in patients receiving gross-total resection with adjuvant proton therapy (23%). Following resection, adjuvant proton therapy was associated with a lower risk of chordoma recurrence compared with photon therapy. The described machine learning models were able to predict tumor progression based on the extent of tumor resection and adjuvant radiotherapy modality used.

Single cell RNA sequencing reveals emergent notochord-derived cell subpopulations in the postnatal nucleus pulposus.

Intervertebral disc degeneration is a leading cause of chronic low back pain. Cell-based strategies that seek to treat disc degeneration by regenerating the central nucleus pulposus (NP) hold significant promise, but key challenges remain. One of these is the inability of therapeutic cells to effectively mimic the performance of native NP cells, which are unique amongst skeletal cell types in that they arise from the embryonic notochord. In this study, we use single cell RNA sequencing to demonstrate emergent heterogeneity amongst notochord-derived NP cells in the postnatal mouse disc. Specifically, we established the existence of progenitor and mature NP cells, corresponding to notochordal and chondrocyte-like cells, respectively. Mature NP cells exhibited significantly higher expression levels of extracellular matrix (ECM) genes including aggrecan, and collagens II and VI, along with elevated transforming growth factor-betaand phosphoinositide 3 kinase-protein kinase B signaling. Additionally, we identified Cd9 as a novel surface marker of mature NP cells, and demonstrated that these cells were localized to the NP periphery, increased in numbers with increasing postnatal age, and co-localized with emerging glycosaminoglycan-rich matrix. Finally, we used a goat model to show that Cd9+ NP cell numbers decrease with moderate severity disc degeneration, suggesting that these cells are associated with maintenance of the healthy NP ECM. Improved understanding of the developmental mechanisms underlying regulation of ECM deposition in the postnatal NP may inform improved regenerative strategies for disc degeneration and associated low back pain.

Outcomes of the Endoscopic Endonasal Approach for the Treatment of Clival Chordomas: A Single-Center Experience.

Objective Chordoma is a low-grade malignant tumor that originates from the remnant tissue of the embryonic notochord. Postoperative or definitive radiotherapy (RT) has been used to enhance local control. This study aims to assess the outcomes of the expanded endoscopic endonasal approach (EEA) for maximal removal of clival chordomas followed by RT for visualized residual or tumor recurrence. Materials and Methods A retrospective review was performed on consecutive patients with clival chordoma who underwent endoscopic endonasal resection in the otorhinolaryngology and neurosurgery departments, between 2016 and 2021. We included all patients with pathologically confirmed clival chordoma who were treated using the EEA. Patients who underwent combined external and endoscopic approaches or transcranial surgery were excluded. Results Seventeen patients were included in this study. Most of them had tumors located in the middle clivus. Regarding RT, the majority of patients underwent postoperative RT. Almost half of them underwent CyberKnife (CK) RT. None of them had severe toxicities (grade 3 or higher). Three patients died, resulting in a mortality rate of 17.6% none of them related to radiation side effect. The 2-year overall survival was 82.4% (mean standard error [SE] = 1.765, 95% confidence interval [CI] = 1.505-2.024), and the progression-free survival (PFS) was 76.5% (mean SE = 3.403, 95% CI = 2.791-4.016). No distal metastasis was reported in our series. Conclusion This series demonstrates that expanded endoscopic endonasal approach (EEA) for the resection of skull base chordomas, followed by CyberKnife radiosurgery, presents a viable alternative to proton beam therapy; however, further research is necessary to directly compare these modalities.

Flexural rigidity of pressurized model notochords in regular packing patterns

The biomechanics of embryonic notochords are studied using an elastic membrane model. An initial study varying internal pressure and stiffness ratio determines tension and geometric ratios as a function of internal pressure, membrane stiffness ratio, and cell packing pattern. A subsequent three-point bending study determines flexural rigidity as a function of internal pressure, configuration, and orientation. Flexural rigidity is found to be independent of membrane stiffness ratio. Controlling for number and volume of cells and their internal pressure, the eccentric staircase pattern of cell packing has more than double the flexural rigidity of the radially symmetric bamboo pattern. Moreover, the eccentric staircase pattern is found to be more than twice as stiff in lateral bending than in dorsoventral bending. This suggests a mechanical advantage to the eccentric WT staircase pattern of the embryonic notochord, over patterns with round cross-section.

Activation of Wnt Pathway Suppresses Growth of MUG-Chor1 Chordoma Cell Line.

Chordoma as a malignant bone tumor, occurs along the axial skeleton and does not have an effective therapy. Brachyury, which is a crucial player for the formation of early embryonic notochord, is abundantly found in both sporadic and familial chordoma. During embryonic development, Brachyury expression was reported to be regulated by the Wnt pathway. The objective of the study is to investigate the role of Wnt signaling in a human chordoma cell line in terms of proliferation, survival, and invasiveness. We tried to elucidate the signaling events that regulate Chordoma cancer. In this regard, Wnt pathway was activated or inhibited using various strategies including small molecules, siRNA-based knockdown and overexpression applications. The results indicated the negative regulatory effect of Wnt signaling activity on proliferation and migration capacity of the chordoma cells. It was revealed that when GSK3β was inhibited, the Wnt pathway was activated and negatively regulated T/Bra expression. Activity of the Wnt pathway caused cell cycle arrest, reduced migration potential of the cells, and led to cell death. Therefore, the present study suggests that the Wnt pathway plays a key role in suppressing the proliferation and invasive characteristics of human chordoma cells and has a great potential as a therapeutic target in further clinical studies.

Skull Base Chordoma: About Two Cases and Literature Review

Skull base chordomas are rare primitive bone tumors developed at the expense of remnants of the embryonic notochord at the level of the clivus, characterized by invasive behavior at the local level with possibility of extension to neighbouring organs. The deep localization of these tumors and their proximity to the structures of the base of the skull round a carcinological resection difficult if not impossible. Radiation therapy, both adjuvant to surgery and exclusive, is a therapeutic approach to improve local control and quality of life for patients. We present two cases of patients followed for skull base chordoma treated with exclusive radiotherapy.

Cytoskeletal Keratins Are Overexpressed in a Zebrafish Model of Idiopathic Scoliosis.

Idiopathic scoliosis (IS) is a three-dimensional rotation of the spine >10 degrees with an unknown etiology. Our laboratory established a late-onset IS model in zebrafish (Danio rerio) containing a deletion in kif7. A total of 25% of kif7co63/co63 zebrafish develop spinal curvatures and are otherwise developmentally normal, although the molecular mechanisms underlying the scoliosis are unknown. To define transcripts associated with scoliosis in this model, we performed bulk mRNA sequencing on 6 weeks past fertilization (wpf) kif7co63/co63 zebrafish with and without scoliosis. Additionally, we sequenced kif7co63/co63, kif7co63/+, and AB zebrafish (n = 3 per genotype). Sequencing reads were aligned to the GRCz11 genome and FPKM values were calculated. Differences between groups were calculated for each transcript by the t-test. Principal component analysis showed that transcriptomes clustered by sample age and genotype. kif7 mRNA was mildly reduced in both homozygous and heterozygous zebrafish compared to AB. Sonic hedgehog target genes were upregulated in kif7co63/co63 zebrafish over AB, but no difference was detected between scoliotic and non-scoliotic mutants. The top upregulated genes in scoliotic zebrafish were cytoskeletal keratins. Pankeratin staining of 6 wpf scoliotic and non-scoliotic kif7co63/co63 zebrafish showed increased keratin levels within the zebrafish musculature and intervertebral disc (IVD). Keratins are major components of the embryonic notochord, and aberrant keratin expression has been associated with intervertebral disc degeneration (IVDD) in both zebrafish and humans. The role of increased keratin accumulation as a molecular mechanism associated with the onset of scoliosis warrants further study.

Anatomical Considerations and Plastic Surgery Reconstruction Options of Sacral Chordoma Resection.

Introduction Chordomas are slow-growing malignant bone tumors arising from remnant embryonic notochord cells with predilection for the sacrum. They rarely metastasize, and early surgical resection with clear margins is the treatment of choice followed by plastic surgery reconstruction supplemented with adjuvant radiotherapy based on the local treatment protocol or in cases with a contaminated surgical field. Aim The aim of the present study is to present our experience in surgical management of sacral chordomas and propose a surgical reconstruction algorithmconsidering anatomical parameters after partial or total sacrectomy. Materials and methods Twenty-seven patients with sacral chordomas were treated in our Orthopaedic Surgery Department between January 1997 and September 2022, and 10 of them had plastic surgery reconstruction. Patients were divided into groups based on the type of sacrectomy, sacrum anatomical vascular or neural variations, partial or total, and the type of soft tissue reconstruction. The postoperative complications and the functional outcomes in each patient were assessed. Results Bilateral gluteal advancement flaps or gluteal perforator flaps are the first choice in patients with partial sacrectomy, intact gluteal vessels, and without preoperative radiotherapy followed by transpelvic vertical rectus abdominis myocutaneous flap or free flaps in those patients with near total sacrectomy and preoperative radiation therapy. Conclusion There are four reliable options for patients after sacral chordomaresection: direct closure, bilateral gluteal advancement flaps, transpelvic vertical rectus abdominis myocutaneous flaps,and free flaps. Each time, tumor-free margins and a good reconstructive plan according to the defect and patient characteristics are mandatory.

SKULL BASE CHORDOMA: ABOUT TWO CASES AND LITERATUREREVIEW

Skull base chordomasare rare primitive bone tumors developed at the expense of remnants of the embryonic notochord at the level of the clivus, characterized by invasive behavior at the local levelwithpossibility of extension to neighbouringorgans. The deep localization of these tumors and their proximity to the structures of the base of the skull round a carcinological resection difficult if not impossible. Radiation therapy, both adjuvant to surgery and exclusive, is a therapeutic approach to improve local control and quality of life for patients. Wepresenttwocases of patients followed for skull base chordomatreatedwith exclusive radiotherapy.

Letter: Chordoma and Ecchordosis Physaliphora: 2 Sides of the Same Coin.

To the Editor: Chordoma and ecchordosis physaliphora (EP) are tumors arising from the notochord's remains along the axial skeleton from the dorsum sella to the sacrococcygeal region. Each year, 1 person in a million is diagnosed with chordoma, but only 45 cases of EP have been reported.1-12 In 1857, Virchow invented the acronym EP for the newly discovered dorsum sella lesion, believing it was cartilage related. A year later, Muller discovered notochord rests in the synchondrosis sphenooccipitalis and hypothesized that these were the source of the EP dorsum sella masses. Ribbert then endorsed this theory.13 Over a century later, Congdon and Wolfe coined the terms benign chordoma and intradural chordoma.14,15 Cha et al justified distinguishing EP from chordoma because they are treated differently and have a different prognosis because one is more aggressive than the other. So far, approximately 45 cases of EP have been diagnosed, with 10 patients displaying symptoms. In 1 of these cases, the patient died as a result.13 Two chief aspects spring to mind when pondering this subject. The first is the manner in which these instances were diagnosed and distinguished from chordoma cases. The second issue stems from the first, namely how to determine the ideal modality of treatment when there is no objective way to distinguish between those pathologies. The articles concur that it is impossible to distinguish between them based on histology or immunohistochemistry staining results because they are derived from the same embryonic notochord. Although certain histopathologic characteristics such as hypocellularity, sparse pleomorphism, and the lack of mitosis may aid in the differentiation of EP from chordoma, they are not considered diagnostic criteria.5,6,16 In addition, it has been stated that an intradural location favors EP over chordoma, yet there are instances of chordoma being an intradural.17 From an imaging point of view, chordoma frequently presents a contrast-enhanced lesion. However, it may occasionally exhibit a mild or nonenhanced lesion similar to EP.18 A study of 28 patients found that 65% of magnetic resonance images of chordoma showed mild or no enhancement.19 Moreover, all reported spinal EPs demonstrated enhanced extradural mass, similar to chordoma lesions.3-6,13 In conclusion, differentiation based on histology, immunohistochemistry staining, radiologic results, or the presence of symptoms is inaccurate. Based on the foregoing, it is impossible, or at least impractical, to distinguish between the 2 terms because they refer to the same ailment. Additional environmental or genetic factors may influence the severity and progression of the disease.20 It would be prudent to consolidate these 2 disorders as part of the spectrum of the same disease, chordoma, and classify it based on severity according to the radiologic findings for size, location, and bone involvement. We hope this paradigm shift will lay the groundwork for novel approaches to chordoma management.

Dedifferentiated chordoma: A rare tumor with diagnostic challenge

Chordomas are rare locally invasive malignant bone tumors arising from remnants of embryonic notochord. Dedifferentiated chordoma (DC), a rare subtype, is characterized by the presence of a sarcomatous component in conventional chordoma (CC) which may arise de novo or as a malignant transformation of previously treated chordoma. The presence of dedifferentiation warrants a poor prognosis due to distant metastasis and recurrences. De novo DCs pose a diagnostic challenge especially in small biopsies and at metastatic sites. Here, we report the case of a 45-year-old female presenting with a long history of backache and constipation, finally diagnosed as DC. Radiological as well as histomorphological pictures of the tumor posed diagnostic challenges because they can mimic other tumors occurring in a similar location. We found this case worth reporting as de novo DC is rarely reported in the literature and it has the potential to pose diagnostic as well as therapeutic challenges. Here we report a case of dedifferentiated chordoma presenting with diagnostic challenge both radiologically and histomorphologically and discuss the role of immunohistochemistry.

A 13-year patient journey of infant giant clival chordoma: case report and literature review

Chordomas are rare malignant bone tumours that develop from the ectopic remnants of the embryonic notochord. In contrast to adults, the majority in children under 16 present intra-cranially (63%). In 2006, we reported the youngest case of a large clival chordoma, a 15-week old baby, the second case to present without skull base involvement and the fourth case of chordoma in a patient with tuberous sclerosis (TS) Kombogiorgas (Childs Nerv Syst 22(10):1369–1374, 2006). In this report, we provide an update on this patient’s journey through a range of therapeutic options and summarize an update of the literature, since 2006, for this patient group.

TMIC-44. TARGETING BRACHYURY REPROGRAMS ENDOTHELIAL TRANSDIFFERENTIATION OF GLIOBLASTOMA

Abstract Glioblastoma (GBM) is the most common and aggressive primary brain tumor with a median survival of only 15 months and a 5-year survival of less than 5% despite the best available treatments. Tumor recurrence/progression and therapy resistance are major obstacles for GBM treatment. GBM is characterized by intensive vascular proliferation that is associated with tumor cell growth, invasion, resistance to chemo/radiotherapy, and decreased disease-free survival. Increasing data have shown the existence of cells with endothelial characteristics called tumor derived endothelial cells, which come from the transdifferentiation of GBM cells and, more specifically, from GBM stem cells (GSCs). However, the molecular mechanisms underlying this process remain largely unknown. GSCs are tightly regulated by distinct transcriptional programs that are driven by nuclear transcription factors. Brachyury is a transcription factor expressed in normal, undifferentiated embryonic notochord in the axial skeleton and plays an important role in stem cell development and differentiation during normal embryonic development. Here, we show that brachyury is highly expressed in patient-derived GBM cells. Functional studies demonstrate that brachyury is regulated by fibroblast growth factor receptor 1 (FGFR1)/mitogen-activated protein kinase (MAPK). Our studies further disclose that FGFR1/MAPK-directed brachyury activation promotes GSC survival and endothelial formation via vascular endothelial growth factor receptor 2 (VEGFR2), whereas pharmacological inhibition of FGFR1 and MAPK or shRNA-mediated downregulation of brachyury decreases GSC transdifferentiation into endothelial cells and suppresses GBM cell growth and stemness via VEGFR2. Our findings highlight the importance of FGFR1/MAPK-regulated brachyury activation in GSC transdifferentiation into endothelial cells via VEGFR2, and targeting the FGFR1-MAPK-brachyury-VEGFR2 signal pathway may represent a novel therapeutic strategy by reprogramming GSCs driving vascular transdifferentiation and tumor progression.

A Rare Presentation of Thoracic Intramedullary Chordoma with Adjacent Bone Involvement: A Case Report and Review of the Literature

Abstract Introduction/Objective Chordoma originates from remnants of the embryonal notochord, and arise in bones anywhere along the spine and skull base. The most common location was thought to be the sacrum, followed by the clivus, and to a much lesser extent the rest of the spine. However, some studies have suggested an equal distribution among the skull base (32%), mobile spine (32.8%), and sacro-coccygeal bones (29.2%). Here we report a case of chordoma involving the thoracic spine. at the level of T2. Methods/Case Report A 63-year-old male with no significant past medical history who presented with 5-6 months of intermittent, bilateral lower extremity weakness and numbness in the trunk and lower extremities. MRI of the thoracic spine demonstrated a contrast enhancing mass at T2 vertebral level with spinal cord compression and adjacent bone destruction. T1-3 laminectomy with debulking of the tumor was performed. Microscopically, the tumor cells have a lobulated architecture and are composed of epithelioid cells arranged in cords, clusters or nests, embedded in a myxoid mucinous matrix. The epithelioid cells have a variably vacuolated cytoplasm ("physaliphorous" cells). The epithelioid cells are positive for CK AE1/3, Cam5.2, EMA and Brachyury (nuclear stain), and S100 (focal). These findings support a diagnosis of chordoma. Results (if a Case Study enter NA) N/A. Conclusion The most important and difficult differential diagnosis of chordoma is with well-differentiated chondrosarcoma. Although both chordomas and chondrosarcomas express S100, chondrosarcomas do not express cytokeratins, EMA or brachyury. Chordomas have an aggressive clinical course and poor outcome with local extension, recurrence and even metastasis. The treatment is en block surgical resection with adjuvant radiotherapy. The extent of the initial surgical resection is the most significant prognostic factor.

A Rare Chordoma-The Epiglottic chordoma.

Chordomas are rare malignant bone tumors. Chordomas originate from notochordal elements. Chordomas have the phenotype of the embryonic notochord, characterized by the dual expression of cytokeratin and brachyury. Chordomas occur anywhere along the central axis. Rarely, chordomas occur in extra-axial structures. We could not find any reports on epiglottic chordoma. Here, we present a case of epiglottic chordoma to highlight this rare cause of laryngeal mass.

Factors associated with artificial airway retention after skull base chordoma resection: A retrospective cohort study.

BackgroundChordoma is a malignant bone and soft tissue tumor derived from embryonic notochord remnants, and skull base chordoma accounts for ~1/3 of all chordoma cases. Skull base chordoma is closely related to the brainstem and cranial nerves and has a high recurrence rate. The purpose of this study was to investigate the influence of the timing of tracheal extubation on perioperative pulmonary complications. We also aimed to explore predictors of postoperative artificial airway (AA) retention in patients with skull base chordoma.MethodsThis was a single-center, retrospective cohort study. The study population included all skull base chordoma patients undergoing surgical treatment between January 2019 and December 2021 at Beijing Tiantan Hospital. The primary outcome was the incidence of postoperative pulmonary complications. Several patient characteristics were evaluated for potential associations with AA retention.ResultsA total of 310 patients with skull base chordoma were enrolled. The frequency of AA retention after surgery for skull base chordoma was 30.97%. The incidence of postoperative pulmonary complications was much lower in those without AA retention (3.74 vs. 39.58%, P < 0.001). Factors with the highest point estimates for the odds of AA retention included body mass index, cranial nerve involvement, maximum tumor diameter, operative method, hemorrhage volume, operative duration and intraoperative mechanical ventilation duration.ConclusionsIn this retrospective cohort study, most of the factors associated with postoperative airway retention were closely related to the patient's tumor characteristics. These data demonstrate that respiratory management in patients with skull base chordoma remains an ongoing concern.