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

OBJECTIVE: 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. METHODS: 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. RESULTS: 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. CONCLUSION: This study, the largest multicenter retrospective analysis of cervical spine chordoma in Korea, identified age as the only factor significantly affecting patient survival.

Skull Base Chordoma in a Young Lady - A Tale of Mismanagement and Redemption: A Case Report and Review of Literature.

Skull base chordomas are a rare entity that requires multidisciplinary decision-making for management. We report a case wherein the patient was initially mismanaged at a peripheral centre, and was then redeemed by a multidisciplinary tumor board decision-making and specialized surgical procedures. We also present a brief review of the therapeutic options.

Poorly differentiated chordoma: recognising this complex and rare aggressive tumour with characteristic immunohistochemical profile.

INTRODUCTION: Poorly differentiated chordoma (PDC) is an uncommon subtype of chordoma, distinct in its occurrence in paediatric age group, location, variable epithelioid/rhabdoid/spindled histomorphology and the lack of physaliphorous cells (classical of chordoma) and immunohistochemistry (INI-1 loss, brachyury positive). We describe two cases of PDC. CASE REPORTS: A 3-year-old male and 4-year-old female child presented with neck stiffness and infiltrating tumour involving the skull base and upper cervical vertebral segments. Histopathology showed a tumour with sheets of cells having epithelioid to rhabdoid morphology and absence of physaliphorous cells. The tumour cells were positive for pan-cytokeratin, EMA, CD99 and vimentin and showed loss of INI-1 suggesting differentials of epithelioid sarcoma and atypical teratoid/rhabdoid tumour. On careful review of the clinical, radiological and pathological features, the additional immunohistochemistry for brachyury was performed, and its positivity clinched the diagnosis of PDC. Both the patients succumbed within a short span post-surgery. CONCLUSION: The present case study helps in creating an awareness and attempts to expand our knowledge in relation to the spectrum of chordoma (clinico-histological) and its immunohistochemical profile.

Tailored surgical approaches for spinal chordomas: A multidisciplinary perspective.

INTRODUCTION: The treatment of spinal chordomas presents a significant challenge due to their resistance to both radiotherapy and chemotherapy as well as the complexity of the surgical procedures required. This study presents a series of cases of primary spinal chordomas, focusing on the development of a personalized therapeutic strategy that is tailored to each patient's unique clinical status. This approach aims to ensure that treatments are optimally aligned with the patient's overall prognosis and surgical eligibility. METHODS: This retrospective study analyzed 14 patients with primary spinal chordomas treated at our institution. We evaluated surgical strategies, clinical outcomes, and survival rates, The therapeutic strategy was formulated after interdisciplinary conferences with sarcoma management specialists. Data were collected on patient demographics, surgical details, postoperative outcomes, and follow-up status. RESULTS: All patients presented with neurological deficits preoperatively, which generally improved post-surgery. The study included a detailed analysis of two distinct surgical approaches: five patients underwent en bloc resection with dorsal stabilization and nine received decompression only. Patients undergoing en bloc resection showed a reduced need for additional surgery due to the comprehensive removal of the tumor. As anticipated, 40% of the patients who underwent decompression experienced tumor progression within the first three months. However, given the poor overall prognosis, the objective of maintaining neurological function was achieved. CONCLUSIONS: Surgical en bloc resection offers a viable and effective intervention for spinal chordomas, enhancing neurological function. It is imperative to tailor treatment strategies to individual prognoses, integrating insights from multidisciplinary discussions that meticulously evaluate surgical risks. This collaborative approach aids in selecting the most appropriate surgical technique tailored to each patient's specific condition.

Spinal chordoma and chondrosarcoma treatment experiences - a 20-year retrospective study from databases of two medical centers.

The research retrospectively analyzed cases of spinal chordoma and chondrosarcoma involving patients who received treatment at the two hospitals between 2001 and 2023. Among the 48 patients studied (39 chordoma and 9 chondrosarcoma cases), the average age was 53.9 ± 15.8 years, with a range of 17 to 86 years. Out of these patients, 43 underwent excision surgery and were categorized based on tumor margin into negative (R0) or microscopically positive (R1) margin (n = 14) and macroscopically positive (R2) margin (n = 29) groups. The mean overall survival (OS) for R0/R1 and R2 groups was 156.5 ± 19.3 and 79.2 ± 11.9 months, respectively (p value = 0.012). The mean progression-free survival (PFS) for R0/R1 and R2 was 112.9 ± 24.4 and 25.5 ± 5.5 months (p value < 0.001). The study showed that regardless of whether patients in the R0/R1 or R2 groups received radiation therapy (RT) or not, there was no significant improvement in OS or PFS. Specifically, the OS and PFS for the RT only group were 75.9 ± 16.6 and 73.3 ± 18.0 months. In conclusion, the recommended treatment approach for spinal chordoma and chondrosarcoma remains en bloc resection surgery with an appropriate margin. Patients who are unsuitable for or decline surgery may find a beneficial disease control rate with traditional external beam photon/proton therapy.

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.

Chordoma combined with Trousseau syndrome: a case report and literature review.

Background: Trousseau syndrome (TS) refers to spontaneous, recurrent, and wandering arterial and venous thromboembolic events in patients with tumors. It results from abnormalities in coagulation and fibrinolytic mechanisms of varying degrees throughout the course of the disease. It has a high fatality rate, and it is commonly seen in patients with highly invasive tumors, such as lung, pancreatic, gastrointestinal, and breast cancers; however, to date, there has been no report of TS combined with chordoma. Case Description: A 56-year-old male with a diagnosis of chordoma underwent surgery, immunotherapy, immunotherapy combined with antiangiogenic therapy, chemotherapy combined with immunotherapy, and proton therapy for localized metastases. Subsequent to the worsening of chest tightness, a repeat chest computed tomography angiography (CTA) scan suggested pulmonary artery embolisms; eventually, a diagnosis of TS was made. After anticoagulation and synchronized antitumor therapy, the patient's condition remained recurrent, eventually leading to death. Conclusions: TS is a frequent but easily overlooked clinical complication that can occur in a variety of tumors, including chordoma, and is currently diagnosed clinically. Thus, further exploration of its sensitive markers is needed. We have reported a case of chordoma combined with TS and conducted a literature review on TS to increase clinicians' awareness of tumor-related thromboembolism and explore strategies to optimize the diagnosis, treatment, and prevention of TS.

Preservation of olfactory function following pediatric endoscopic endonasal skull base surgery with nasoseptal flap reconstruction.

OBJECTIVE: Endoscopic endonasal surgery is a feasible approach to lesions of the pediatric skull base. Olfactory outcomes following endoscopic endonasal approach (EEA) have been reported in the adult literature, but pediatric outcome data is lacking. The purpose of this preliminary study is to evaluate post-operative olfactory outcomes in pediatric patients who underwent EEA through transsphenoidal and/or transclival corridors. METHODS: Retrospective chart review of patients < 18 years of age who underwent EEA with nasoseptal flap skull base reconstruction between March 2019-January 2022. Following their first post-operative debridement and 6 months following surgery, patients were administered the University of Pennsylvania Smell Identification Test (UPSIT) to assess olfactory function. RESULTS: Fifteen patients (7 female, 8 male) with a mean age of 14.9 years met inclusion criteria. Thirteen (86.7%) patients required a transsphenoidal approach and 2 (13.3%) patients underwent a combined transsphenoidal and transclival approach. Mean time between surgical resection and first olfactory testing was 6.3 weeks (range 4.6-7.3 weeks). Nine patients (60.0%) had normosmia following EEA while 3 (20.0%) had mild microsmia, and 3 (20.0%) had moderate microsmia at 6 weeks following surgery. Results improved at their 6 month follow up visits. No patients experienced post-operative severe microsmia or anosmia. CONCLUSIONS: Olfactory preservation is possible following pediatric EEA, though 40% of patients will develop some degree of microsmia in the immediate post-operative period and 20% will have some persistent dysfunction at 6 months. Long-term data is necessary to determine if microsmia continues to improve over time.

Chronic hearing loss turns out being a calcified chondroid mesenchymal neoplasm with FN1::FGFR2 fusion.

A 53 year old female presented with a six-year history of right-sided slow deterioration in hearing and a feeling of pressure in the right ear. The patient had not experienced any pain but reported some paresthesia of the right half of the tongue, whereas no further other cranial nerve deficits were evident. The otoscopy was unremarkable as well as the rest of the clinical ENT examination except for a slight asymptomatic swelling of the right cheek. Imaging findings showed an expansive tumor infiltrating and destroying the right lateral skull base. The tumor was partially composed of cystic/regressive lesions with high contrast media uptake. The tumor had high-signal intensity with water-sensitive sequences (T2w) and was hypointense on T1w images. We performed a tumor resection via a transparotideal-infratemporal approach. Histologically, the tumor was composed of granular variably calcified chondroid matrix with extensive regressive changes and granulation-like tissue reaction associated with calcinosis and crystal deposition. Molecular analysis of the tumor via the TruSight- RNA-Fusion panel detected a fusion involving FN1::FGFR2, consistent with "calcified chondroid mesenchymal neoplasm" (CCMN), a rare tumor entity recently defined by Liu et al 2021. In regular follow-up care no residual tumor has been detected in imaging studies (MRI and CT) within 2 years and 4 months. The biology and consequently the radio sensitivity cannot be defined precisely since long term results are missing due to the first description of this entity in 2021. As a consequence, surgical resection is recommended as the treatment of choice. Thorough clinical and radiological follow-up is mandatory as local recurrences are to be expected due to the infiltrative behavior. In case of a loco regional recurrence the fusion with FGFR2 may represent a therapeutic option for a targeted therapy on molecular level.

Clinical-proteomic classification and precision treatment strategy of chordoma.

Chordoma is a rare and heterogeneous mesenchymal malignancy, with distinct clinical and biological behaviors. Till now, its comprehensive clinical-molecular characteristics and accurate molecular classification remain obscure. In this research, we enroll 102 patients with chordoma and describe their clinical, imageological, and histopathological features. Through tandem mass tag-based proteomic analysis and nonnegative matrix factorization clustering, we classify chordoma into three molecular subtypes: bone microenvironment-dominant, mesenchymal-derived, and mesenchymal-to-epithelial transition-mediated pattern. The three subtypes exhibit discrete clinical prognosis and distinct biological attributes of osteoclastogenesis and immunogenicity, oxidative phosphorylation, and receptor tyrosine kinase activation, suggesting targeted therapeutic strategies of denosumab, S-Gboxin, and anlotinib, respectively. Notably, these approaches demonstrate positive treatment outcomes for each subtype in vitro and in vivo. Altogether, this work sheds light on the clinical-proteomic characteristics of chordoma and provides a candidate precision treatment strategy for chordoma according to molecular classification, underscoring their potential for clinical application.

Therapeutic strategies for mobile spine chordoma: en bloc Versus intralesional surgery with adjuvant charged-particle therapy.

PURPOSE: The aim of this retrospective study is to analyze the impact of en bloc resection with negative margins versus intralesional resection plus adjuvant hadron-therapy (HT) on local control (LC) and overall survival (OS) in patients with mobile spine chordomas. Mechanical complications incidence as well as risk factors, and outcome differences are investigated as secondary endpoints. METHODS: 33 patients in a period from January 2013 to December 2021 were enrolled for the final analysis. The inclusion criteria were: lesions located in the mobile spine (C1-L5), age ≥ 15 years, minimum follow-up of 2 years, en bloc or intralesional surgical resection, virgin or recurrent chordomas, with only one previous surgical treatment. RESULTS: No difference was found in terms of LC between the two groups. The presence of pathologic fracture at pre-operative imaging and the presence of macroscopic residual tumor after surgery, independently from its entity, seemed to be associated with an increased risk of LR. No difference was found between planned en bloc and planned intralesional surgery in terms of mechanical complications occurrence. Eight patients (24.24%) had mechanical complications during the follow up period: male sex, presence of pathologic fracture at baseline, a combined surgical approach, the use of carbon fiber-only hardware appeared to be associated with an increased risk of mechanical complications after the primary surgery. CONCLUSIONS: En bloc resection, whenever possible, is always to be preferred for its widely recognized potential in LC and OS improvement. However, technology advances in high-dose conformal charged-particle therapy have allowed improvement of local control rates as an adjuvant therapy of intralesional surgery for mobile spine chordoma, with acceptable acute and chronic toxicity.

Tumor-derived IL-6 promotes chordoma invasion by stimulating tumor-associated macrophages M2 polarization and TNFα secretion.

AIMS: Chordoma is a rare and aggressive bone tumor with high-recurrence and lack of effective treatment methods. Tumor associated macrophages (TAMs) are abundant in tumor microenvironment (TME) and polarize toward M2 in chordoma. It has been observed that the high proportion of M2 cells is associated with chordoma rapid progression. However, the mechanism of TAMs polarization and promotion to tumor progression in chordoma is still unclear. The is an urgent need for further research. MATERIALS AND METHODS: Flow cytometry and immunohistochemical staining was used to detect the degree of macrophages infiltration in chordoma. A co-culture model of chordoma cells and macrophages was established in vitro to investigate the effects of their interaction on cell function, cytokine secretion, and RNA transcriptome expression. KEY FINDINGS: In this study, we found M2 macrophage was predominantly abundant immune cell population in chordoma, and its proportion was associated with the degree of bone destruction. We demonstrated that interleukin 6 (IL-6) derived from chordoma cells could induce TAMs polarization by activating STAT3 phosphorylation, and TAMs could enhance chordoma cells migration and invasion through TNFα/NF-κB pathway. The interaction of chordoma cells and TAMs could promote the bone destruction-related factor Cathepsin B (CTSB) and inhibitory immune checkpoints expression. We also confirmed blocking IL-6/STAT3 pathway could significantly attenuate the M2 polarization of TAMs and decrease the secretion of TNFα. SIGNIFICANCE: This study illustrates the dynamics between chordoma cells and TAMs in promoting chordoma invasion and suggests that IL-6/STAT3 pathway is a potential therapeutic target to reduce TAM-induced chordoma invasion.

Endoscopic endonasal extradural posterior clinoidectomy: A key maneuver to access the retrosellar and upper retroclival area.

BACKGROUND: Endoscopic endonasal posterior clinoidectomy represents an important maneuver to improve access and visualization of the retrosellar and upper clivus area [1]. Three different techniques have been described in order to access and remove the posterior clinoid: 1) the intradural pituitary transposition [2], the interdural pituitary transposition [3] and a completely extradural technique [4]. CASE DESCRIPTION: We present here a case of retrosellar and retroclival chordoma that has been removed through endoscopic endonasal approach. Technical nuances of the endonasal extradural posterior clinoidectomy are presented. CONCLUSION: Endoscopic endonasal extradural posterior clinoidectomy allows to access this complex anatomical region with the advantage of avoiding major bleeding from the cavernous sinus and endocrinological impairment.

Chordoma incidence, treatment, and survival in the 21st century: a population-based Ontario cohort study.

OBJECTIVE: This study estimates the incidence, treatment patterns, and overall survival for patients with chordoma treated in Ontario. METHODS: A 17-year (2003-2019) population-based cohort study was conducted, including all patients in the Ontario Cancer Registry with histologically proven chordoma. Primary outcomes of interest were age-standardized annual incidence, overall survival, and rates of radiation therapy, chemotherapy, and open resection. RESULTS: A total of 208 patients were diagnosed with chordoma over the study period: 97 patients with skull base chordoma, 37 with mobile spine chordoma, and 65 with sacropelvic chordoma. A total of 133 patients were treated with either open or endoscopic surgery, of whom 99 were also treated with some form of radiation therapy. Across the 17-year study period, the average annual age-standardized incidence was 12.04 cases per 10 million (95% CI 9.31-14.78 cases per 10 million). There was no significant change in the annual incidence rate over the study period (average annual percent change 2.27, 95% CI -1.74 to 6.44; p = 0.25). The odds of receiving radiation therapy or chemotherapy significantly increased by 8% per year (95% CI 1%-16% per year, p = 0.036) over the study period. The odds of receiving open resection significantly decreased by 14% per year (95% CI 8%-20% per year, p < 0.001). The odds of receiving endoscopic surgery among patients with skull base chordoma increased by 38% per year (95% CI 22%-60% per year, p < 0.001), while the odds of patients receiving biopsy alone did not change significantly over the study period (p = 0.684). After diagnosis of chordoma, the 5-, 10-, and 15-year overall survival probabilities were 0.74 (95% CI 0.69-0.81), 0.58 (95% CI 0.51-0.67), and 0.48 (95% CI 0.40 to 0.59), respectively. There was no significant association between hazard of death and year of diagnosis (p = 0.126) or anatomical location (p = 0.712, skull base vs mobile spine chordoma; p = 0.518 skull base vs sacropelvic chordoma). CONCLUSIONS: Chordoma is a rare disease with no significant change in the average annual incidence rate between 2003 to 2019. During this time, treatment with less invasive modalities increased, particularly for skull base chordoma. Overall survival exceeds 10 years for many patients, with no change in the hazard of death across the study period.

Immunologic and Targeted Molecular Therapies for Chordomas: A Narrative Review.

Chordomas are rare sarcomas arising from notochordal tissue and occur most commonly in the spine. The standard of care for chordomas without evidence of metastatic disease generally consists of en bloc resection followed by adjuvant radiotherapy. However, long-term (20-year) survival rates are approximately 30%. Chordomas are generally considered as chemo resistant. Therefore, systemic therapies have rarely been employed. Novel immunotherapies, including antibody therapy and tumor vaccines, have shown promise in early trials, leading to extended progression-free survival and symptom relief. However, the outcomes of larger trials using these vectors are heterogeneous. The aim of this review is to summarize novel chordoma treatments in immune-targeted therapies. The current merits, trial outcomes, and toxicities of these novel immune and targeted therapies, including those targeting vascular endothelial growth factor receptor (VEGFR) targets and the epidermal growth factor receptor (EGFR), will be discussed.

Case report of large solitary skull base metastasis from renal cell carcinoma as initial clinical presentation: Radiological findings and differentials.

A 58-year-old male with good past health presented with headache and visual disturbance for 1 month. Computed tomography (CT) and magnetic resonance imaging (MRI) were performed, showing a large aggressive midline mass with epicenter at the skull base and sellar-suprasellar region. There was marked heterogenous enhancement and intratumoral calcifications. It demonstrated clivus erosion, sphenoid sinus invasion, cavernous sinus invasion and optic chiasm compression. The imaging features were considered to be consistent with clival chordoma and the patent underwent emergent endoscopic transsphenoidal surgical excision. The difficult operation found an unexpected highly vascularized tumor and histopathology of the excision showed clear cell renal cell carcinoma metastasis. Subsequent work up of the patient with positron emission tomography-computed tomography (PET-CT) confirmed suspicion of the primary tumor, arising from left kidney. This case demonstrates an underestimated differential diagnosis of a large aggressive skull base mass as the first initial clinical presentation of metastatic disease.

Comparative overall survival analysis of chordomas of the base of the skull from the Surveillance, Epidemiology, and End Results (SEER) program between 2000 and 2020.

Chordomas of the skull base are rare, slow growing, locally invasive cancers with limited long-term survival analysis reported in the literature. We seek to provide comparative survival analysis of patients on a long-term (20-year) basis using population-level data. The Surveillance, Epidemiology, and End Results (SEER) program was queried for cases of chordoma relegated to the base of the skull, diagnosed between 2000 and 2020. Demographic, disease, and treatment information were analyzed using Cox proportional hazards and log-rank comparisons. 630 patients with chordoma of the skull base were identified. Age ≤ 49 years at diagnosis was associated with increased five-, 10-, and 20-year overall survival (hazard ratio (HR) = 0.39, 0.33, and 0.30, respectively; p < 0.001 for all). Treatment with surgery and adjuvant radiotherapy was associated with increased five-, 10-, and 20-year survival (HR = 0.71, 0.79, and 0.79, respectively; p < 0.001 for all). On univariate analysis, widowed patients had decreased survival (20-year overall survival = 34.8% [15.3%-34.8%] compared to married patients (74.4% [68.1%-80.8%]. Surgery remains the primary treatment associated with increased survival among patients with chordoma of the skull base, with adjuvant radiotherapy serving a complimentary role. Demographic factors such as marital status are also associated with changes in survival.

Degenerative Atypia in Clival Chordoma: Two Case Reports.

In this study, we report surgical management combined with radiotherapy in two patients with typical chordoma. Different types of radiation have varied effects on chordomas when they are radiated. Classical cases display cellular atypia and fibrosis following irradiation, while necrosis and fibrosclerosis are observed after carbon ion therapy, implying that it is possible to control the tumor more effectively using carbon ion therapy with minimal side effects.

Muscle metastasis from cervical chordoma: a case report.

Chordomas are rare primary bone tumours that commonly occur in the sacrococcygeal and skull base region and have high rates of local recurrence. They have a locally aggressive course and the most common site of distant metastasis is the lung. The aim of this case report is to present the imaging findings of instance of muscle metastasis, a rare occurrence in cervical chordoma.

Acute peritumoral brainstem hemorrhage of a clival chondrosarcoma successfully treated via endoscopic endonasal surgery: illustrative case.

BACKGROUND: Chondrosarcoma is typically a slow-growing tumor, and intratumoral hemorrhage is rare. Acute brainstem hemorrhage due to chondrosarcoma has rarely been reported. OBSERVATIONS: A 77-year-old man presented with the sudden onset of headache and vomiting followed by a declining level of consciousness, progressive right hemiparesis, and left ophthalmoplegia. Magnetic resonance imaging showed pontine hemorrhage and a mass in the retroclival space compressing the brainstem. Emergency endoscopic endonasal surgery was performed. Intraoperative observation revealed that a hematoma was located in the pons and subdural space around the tumor mass, suggesting that the hematoma had likely been caused by the rupture of small vessels around the pons, not by intratumoral hemorrhage. The pathological diagnosis was chondrosarcoma. The patient recovered well and underwent radiotherapy. LESSONS: This report describes a case of sudden neurological deterioration due to hemorrhage in a patient with chondrosarcoma of the skull base. An emergency endoscopic endonasal approach for mass reduction and hematoma removal was effective in the acute setting. This approach revealed the suspected etiology of peritumoral hemorrhage, not intratumoral hemorrhage. https://thejns.org/doi/10.3171/CASE2460.