Materiały źródłowe

• #1

  https://link.springer.com/article/10.1007/s00586-018-5764-0

  The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival. […] Incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. […] Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients.

• #2 Chordoma Spontaneous Regression After COVID-19

  https://www.mdpi.com/1999-4915/17/1/10

  Chordomas are rare primary bone midline tumors that are thought to arise from remnants of embryonal notochord. Their incidence rate ranges from 0.18 to 0.84 per million people per year, showing variation across countries and racial groups. They are very challenging tumors to treat and are considered to be a low-to-intermediate-grade slow-growing subtype of sarcoma. This information sometimes leads to the mistake that they are relatively benign tumors. They are aggressive and have a propensity to grow in a locally destructive and invasive way with a tendency to show local recurrence and metastasis in 10–40% of cases. Currently, the median survival of patients is 7.7 years with a 5-year overall survival (OS) rate of 50–70%. When gross total resection with free margins is achieved, the 5-year OS rate increases to 65%; in inoperable cases, it decreases to 40%. So, en bloc radical resection is the main treatment and poses various challenges, even for more experienced surgeons. Especially when the tumor is located in the skull base involving nerves and vessels, gross total resection (GTR) is often not possible without causing significant morbidity. In addition to GTR, radiotherapy and chemotherapy, many researchers have debated about the use of targeted immunotherapy.

• #3 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #4 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #5

  https://www.massgeneral.org/orthopaedics/oncology/conditions-and-treatments/chordoma

  Chordomas may develop at any age but usually occur between 40 and 70 years of age. […] About 300 patients are diagnosed with chordoma each year in the United States, and about 700 in all of Europe. […] Surveillance: After the initial postop visits (typically at 2 and 6 weeks), routine surveillance is for 10 years with imaging: Every 3 months for 2 years, Then every 6 months for 3 years, which equals 5 years of follow-up, Annual visit thereafter for 5 more years, which concludes the surveillance standard of care of 10 years (but most patients like to continue with an annual visit).

• #6 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #7 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  Chordomas account for 1-4% of all bone malignancies and have an incidence of 0.088 per 100, 000 people per year. […] Although chordomas are histologically benign, they are highly recurrent and clinically aggressive, making their clinical course similar to more malignant clival, sacral, or spinal neoplasms. […] Chordomas are midline and can be further characterized into the following subgroups from highest to lowest prevalence: cranial, sacral, and spinal. Prevalence rates are similar among the three distributions. […] The median age of diagnosis is 58.5 years, and the incidence of chordomas increases with age, with the highest incidence among adults in the 75-84-year age group. […] Epidemiological analyses indicate that chordomas more commonly affect males. […] In fact, among patients with skull-base chordomas, males tend to have higher risk of disease progression and mortality.

• #8 Chordoma of the skull base – UpToDate

  https://www.uptodate.com/contents/chordoma-of-the-skull-base

  Chordomas are rare tumors with an annual incidence of 0.2 to 1.2 per million people worldwide. They represent 1 to 4 percent of all primary bone malignancies and 0.2 percent of intracranial neoplasms. Skull base chordomas make up approximately one-third of all chordomas; the remaining are roughly equally distributed between the sacrum and the rest of the mobile spine.

• #9

  https://link.springer.com/article/10.1007/s00586-018-5764-0

  The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival. […] Incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. […] Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients.

• #10 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #11 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  Chordomas account for 1-4% of all bone malignancies and have an incidence of 0.088 per 100, 000 people per year. […] Although chordomas are histologically benign, they are highly recurrent and clinically aggressive, making their clinical course similar to more malignant clival, sacral, or spinal neoplasms. […] Chordomas are midline and can be further characterized into the following subgroups from highest to lowest prevalence: cranial, sacral, and spinal. Prevalence rates are similar among the three distributions. […] The median age of diagnosis is 58.5 years, and the incidence of chordomas increases with age, with the highest incidence among adults in the 75-84-year age group. […] Epidemiological analyses indicate that chordomas more commonly affect males. […] In fact, among patients with skull-base chordomas, males tend to have higher risk of disease progression and mortality.

• #12

  https://link.springer.com/article/10.1007/s00586-018-5764-0

  The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival. […] Incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. […] Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients.

• #13 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #14 Orphanet: Chordoma

  https://www.orpha.net/en/disease/detail/178

  They are predominantly found in adults, and comprise 0.2% of all central nervous system tumors and 2-4% of all primary bone neoplasms, with an estimated prevalence of 1 in 2 million people and a male-to-female ratio of 2:1. […] Prognosis depends on the extent and completeness of the tumor excision. Long-term follow-up is required because of the high rate of recurrence of these tumors.

• #15 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #16 Pediatric Chordoma: New Updates and Breakthroughs 2024

  https://oncodaily.com/oncolibrary/cancer-types/77031

  Chordoma is an exceptionally rare tumor, with an annual incidence of approximately 1 per 1,000,000 people in the United States and Europe, leading to about 350 cases per year in the U.S. Pediatric chordomas are even rarer, accounting for less than 5% of all chordoma cases. […] Most pediatric chordomas occur in the cranium, particularly at the skull base, and are more common in males than females.

• #17 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #18 Chordoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/chordoma?embed_domain=hackmd.io%252F%2540yIPUAFeCSL2JsU8smR5nJQ%252Fbnjhjgjghjghjghfavicon.ico&lang=us

  Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors. […] Chordomas can occur at any age but are usually seen in adults (30-70 years). Those located in the spheno-occipital region most commonly occur in patients 20-40 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years). There is an overall male to female predilection of 2:1. […] Although some publications have reported greater numbers of white patients being diagnosed with chordoma, differences in incidence rates by ethnicity have not been established. […] Rarely, chordomas are encountered in children with tuberous sclerosis. Rare familial cases have also been described with mutations of the TBXT gene.

• #19 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Chordoma is a rare tumor that occurs along the axial spine in children and in adults, with an incidence of approximately 350 cases per year in the United States and a reported annual worldwide incidence of 0.08 per 100,000 cases. […] Chordomas typically affect those in the 40- to 60-year age group but have been reported in children and in the very elderly. Most believe males are more commonly affected than females, at an approximately 2:1 ratio, with an annual incidence of 1:1,000,000 for new diagnoses. Chordomas account for approximately 20% of primary spinal tumors and only 3% of all bone tumors. The most common location is the sacrum/coccygeal region (50%), followed by the spheno-occipital region (35%) and the mobile spine (about 10-15%). […] Less than 5% of chordomas occur in children. In a population-based study comparing pediatric versus adult skull base chordoma, Xu and colleagues reviewed data from the National Cancer Database from 2004 to 2015 on patients 18 years of age and older versus those younger than 18 years. They found that pediatric patients were likely to have larger tumor size (41.4 15.7 mm vs 34.1 15.8 mm; P 0.01) and were universally treated at academic facilities. They found no difference in overall survival.

• #20 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://www.mdpi.com/2077-0383/10/5/1054

  Chordomas may present in any location along the axis of what was formerly the notochord and accordingly, population studies have shown that they arise with a similar distribution in the sacrum (29.2%), mobile spine (32.8%) and skull base (32%) [3]. […] The incidence of chordoma is only 0.08 in 100,000 individuals [3]. Males (incidence rate 0.10) are more commonly affected than females (incidence rate 0.06), with a peak age between 50–60 years (median, 58.5 years). Children and adolescents are rarely affected (<5% of cases) [3]. [...] Chordomas are typically sporadic but a number of suspected familial forms have been documented, with a variety of familial genetic mutations identified in these cases, including recurrent germ-line mutations in the T gene (6p27), which encodes brachyury, as mentioned previously [28]. Chordomas have also been reported to arise in association with other pathological syndromes, such as tuberous sclerosis complex, Ollier disease and Maffuci syndrome [53,54,55].

• #21 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  According to the recent descriptive epidemiology in the United States, the age-adjusted incidence rate of all types of chordomas was 0.88 per million persons per year from 2001 to 2014. The annual age-standardized incidence rate of chordomas in Taiwan from 2003 to 2010 was 0.4 per million, showing male predominance. […] Chordomas commonly affect the adult population with a median age at diagnosis of 60 years. There is a male predominance for all types of the chordomas in the adult group. Cranial chordomas are the most common type, accounting for 38.7% of all types of chordomas followed by the sacral type (34.3%) and spinal type (27.0%). Cranial chordomas occurred more in the Asian/Pacific individuals, whereas the incidences of spinal and sacral chordomas were highest in the Caucasians population.

• #22 Descriptive epidemiology of chordomas in the United States | Scilit

  https://www.scilit.com/publications/e0084b8cedb24183b76f3adfe3481298

  Descriptive epidemiology of chordomas in the United States […] Purpose Chordomas account for 1% to 4% of all bone malignancies and 0.5% of all primary intracranial central nervous system tumors. Prior epidemiologic literature is based on limited population data. The purpose of this study is to provide the largest and most inclusive population based study of the descriptive epidemiology of chordomas. […] Methods The Centers for Disease Control and Prevention and National Program of Cancer Registries were queried for chordoma in all locations. Age-adjusted incidence per 100,000 persons was calculated by age, sex, race, and ethnicity. Annual percentage change was calculated using Joinpoint. […] Results From 2004 to 2014, a total of 3670 chordomas were diagnosed in the US. The most common location was cranial (38.7%), followed by sacral (34.3%) and spinal (27.0%). The average age-adjusted incidence rate was 0.088 per 100,000 persons per year (95% CI 0.086–0.091), with an annual percentage change of 1.29% (95% CI 0.31–2.28%). For all chordomas, the incidence peaks in the 75–84 year age group. The male-to-female incidence rate ratio is 1.54 (p < 0.001). American Indian/Alaskan Native and Black patients had a statistically lower incidence rate than White and Asian/Pacific Islander patients.

• #23 Azthena logo with the word Azthena

  https://www.news-medical.net/health/What-is-Chordoma.aspx

  Chordoma is a rare, low-grade, malignant neoplasm originating from remnants of embryonic notochord along the spine. […] The epidemiology of chordoma is variable with an incidence of 1.7, 1, and 0.5 in 1 million in India, Japan, and Australia respectively. […] In both the United States and Europe, the incidence of Chordoma is around 0.08 per 100,000, with a median age at diagnosis of around 60 years. Chordoma has a substantial age-related variability in incidence: it is extremely rare in children (1.4 and 0.8 per 10 million in the United States and Europe, respectively). It rises with the population’s age and reaches a pinnacle in the eighties. In both the United States and Europe, there is a small male predominance, with a 1.5 incidence ratio between men and women. […] In terms of chordoma primary site distribution, % of chordoma instances in the United States are spinal, 32% cranial, 29% sacral, and 6% have other primary sites. In Europe, 36% of primary sites are sacral, 30% are cranial, 23% are spinal, and 11% have other primary sites.

• #24 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  Chordomas could also affect the children and young adults populations. There is a female predominance in the pediatric group and is more commonly occurring at the craniocervical junction, with decreasing incidence caudally along the spine, in contrast to the craniocervical and sacrococcygeal predominance in the adult populations.

• #25 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #26 Conventional Chordoma – SFA

  https://curesarcoma.org/sarcoma-subtypes/conventional-chordoma/

  Conventional chordoma is a malignant tumor that develops in the axial skeleton, mimicking notochord tissue. It most commonly occurs in the base of the skull, spine, and sacrum. […] The incidence of chordoma is 0.08 cases per 100,000 person-years, with an M:F ratio of approximately 1.8:1. Chordoma rarely occurs in the black African population but appears to be represented equally in people of other ethnic groups. However, in one review, African-Americans were more represented in the pediatric cohort than in the adult cohort. All ages are affected, but chordoma most commonly occurs in the fifth to seventh decades of life.

• #27 Chordoma at the skull base, spine, and sacrum: A pictorial essay – Journal of Clinical Imaging Science

  https://clinicalimagingscience.org/chordoma-at-the-skull-base-spine-and-sacrum-a-pictorial-essay/

  According to the recent descriptive epidemiology in the United States, the age-adjusted incidence rate of all types of chordomas was 0.88 per million persons per year from 2001 to 2014. The annual age-standardized incidence rate of chordomas in Taiwan from 2003 to 2010 was 0.4 per million, showing male predominance. […] Chordomas commonly affect the adult population with a median age at diagnosis of 60 years. There is a male predominance for all types of the chordomas in the adult group. Cranial chordomas are the most common type, accounting for 38.7% of all types of chordomas followed by the sacral type (34.3%) and spinal type (27.0%). Cranial chordomas occurred more in the Asian/Pacific individuals, whereas the incidences of spinal and sacral chordomas were highest in the Caucasians population.

• #28 Clinical findings in families with chordoma with and without T gene duplications and in patients with sporadic chordoma reported to the Surveillance, Epidemiology, and End Results program in: Journal of Neurosurgery Volume 134 Issue 5 (2020) Journals

  https://thejns.org/view/journals/j-neurosurg/134/5/article-p1399.xml

  To gain insight into the role of germline genetics in the development of chordoma, the authors evaluated data from 2 sets of patients with familial chordoma, those with and without a germline duplication of the T gene (T-dup+ vs T-dup), which was previously identified as a susceptibility mechanism in some families. The authors then compared the patients with familial tumors to patients with sporadic chordoma in the US general population reported to the National Cancer Institutes Surveillance, Epidemiology, and End Results (SEER) program through 2015. […] The occurrence of young age at diagnosis, skull base presentation, or multiple primary chordomas should encourage careful review of family history for patients diagnosed with chordoma as well as screening of at-risk family members by MRI for early detection of chordoma. Furthermore, given genetic predisposition in some patients with familial chordoma, identification of a specific mutation in a family will permit surveillance to be limited to mutation carriers and consideration should be given for imaging the entire neuraxis in any chordoma patient presenting at an early age or with a blood relative with chordoma. Finally, future studies should explore racial differences in age at diagnosis and presenting site in chordoma.

• #29 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #30 Chordoma | Radiology Reference Article | Radiopaedia.org

  https://radiopaedia.org/articles/chordoma?embed_domain=hackmd.io%252F%2540yIPUAFeCSL2JsU8smR5nJQ%252Fbnjhjgjghjghjghfavicon.ico&lang=us

  Chordomas are uncommon malignant tumors of the axial skeleton that account for 1% of intracranial tumors and 4% of all primary bone tumors. […] Chordomas can occur at any age but are usually seen in adults (30-70 years). Those located in the spheno-occipital region most commonly occur in patients 20-40 years of age, whereas sacrococcygeal chordomas are typically seen in a slightly older age group (peak around 50 years). There is an overall male to female predilection of 2:1. […] Although some publications have reported greater numbers of white patients being diagnosed with chordoma, differences in incidence rates by ethnicity have not been established. […] Rarely, chordomas are encountered in children with tuberous sclerosis. Rare familial cases have also been described with mutations of the TBXT gene.

• #31 Chordoma – Wikipedia

  https://en.wikipedia.org/wiki/Chordoma

  In the United States, the annual incidence of chordoma is approximately 1 in one million (300 new patients each year). […] Sacral chordomas make up 2 to 4% of all primary bone tumours and 44% of all primary sacral tumours, thus making it the most common malignant sacral tumor. About 50 to 60% of chordomas are located in the sacrococcygeal region. Males aged between 40 and 50 years are twice as likely as women to get sacral chordoma. […] There are currently no known environmental risk factors for chordoma. As noted above germline duplication of brachyury has been identified as a major susceptibility mechanism in several chordoma families. […] While most people with chordoma have no other family members with the disease, rare occurrences of multiple cases within families have been documented. This suggests that some people may be genetically predisposed to develop chordoma. Because genetic or hereditary risk factors for chordoma may exist, scientists at the National Cancer Institute are conducting a Familial Chordoma Study to search for genes involved in the development of this tumor.

• #32 Chordoma—Current Understanding and Modern Treatment Paradigms

  https://www.mdpi.com/2077-0383/10/5/1054

  Chordomas may present in any location along the axis of what was formerly the notochord and accordingly, population studies have shown that they arise with a similar distribution in the sacrum (29.2%), mobile spine (32.8%) and skull base (32%) [3]. […] The incidence of chordoma is only 0.08 in 100,000 individuals [3]. Males (incidence rate 0.10) are more commonly affected than females (incidence rate 0.06), with a peak age between 50–60 years (median, 58.5 years). Children and adolescents are rarely affected (<5% of cases) [3]. [...] Chordomas are typically sporadic but a number of suspected familial forms have been documented, with a variety of familial genetic mutations identified in these cases, including recurrent germ-line mutations in the T gene (6p27), which encodes brachyury, as mentioned previously [28]. Chordomas have also been reported to arise in association with other pathological syndromes, such as tuberous sclerosis complex, Ollier disease and Maffuci syndrome [53,54,55].

• #33 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #34 Clinical findings in families with chordoma with and without T gene duplications and in patients with sporadic chordoma reported to the Surveillance, Epidemiology, and End Results program in: Journal of Neurosurgery Volume 134 Issue 5 (2020) Journals

  https://thejns.org/view/journals/j-neurosurg/134/5/article-p1399.xml

  To gain insight into the role of germline genetics in the development of chordoma, the authors evaluated data from 2 sets of patients with familial chordoma, those with and without a germline duplication of the T gene (T-dup+ vs T-dup), which was previously identified as a susceptibility mechanism in some families. The authors then compared the patients with familial tumors to patients with sporadic chordoma in the US general population reported to the National Cancer Institutes Surveillance, Epidemiology, and End Results (SEER) program through 2015. […] The occurrence of young age at diagnosis, skull base presentation, or multiple primary chordomas should encourage careful review of family history for patients diagnosed with chordoma as well as screening of at-risk family members by MRI for early detection of chordoma. Furthermore, given genetic predisposition in some patients with familial chordoma, identification of a specific mutation in a family will permit surveillance to be limited to mutation carriers and consideration should be given for imaging the entire neuraxis in any chordoma patient presenting at an early age or with a blood relative with chordoma. Finally, future studies should explore racial differences in age at diagnosis and presenting site in chordoma.

• #35 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #36 Chordoma: New Updates and Breakthroughs 2024

  https://oncodaily.com/oncolibrary/cancer-types/76224

  There are no established environmental, dietary, or lifestyle factors that have been linked to an increased risk of developing this tumor. The majority of cases appear to occur sporadically without any identifiable external cause. […] Genetic Predisposition: As mentioned, genetic factors such as the presence of the brachyury gene SNP and familial history of chordoma can increase the risk, although these cases are rare.

• #37 Chordoma: New Updates and Breakthroughs 2024

  https://oncodaily.com/oncolibrary/cancer-types/76224

  Chordomas are extremely rare, with an incidence of approximately 0.08 to 0.88 per 100,000 persons per year, varying between countries and races. In the United States, the annual incidence is about 350 cases, translating to roughly 1 per million people. These tumors account for 1% to 4% of all primary bone malignancies and about 20% of primary spinal tumors. They are more common in men, with a male-to-female ratio ranging from 1.5:1 to 2:1. […] The incidence peaks in individuals aged 40 to 70 years, although they can occur at any age, including in children. Pediatric chordomas are rare, accounting for less than 5% of cases, and tend to present with larger tumors and more aggressive behavior. […] Age and Gender: These tumors are more commonly diagnosed in adults between the ages of 40 and 70, with a slight predominance in males.

• #38 Chordoma: New Updates and Breakthroughs 2024

  https://oncodaily.com/oncolibrary/cancer-types/76224

  There are no established environmental, dietary, or lifestyle factors that have been linked to an increased risk of developing this tumor. The majority of cases appear to occur sporadically without any identifiable external cause. […] Genetic Predisposition: As mentioned, genetic factors such as the presence of the brachyury gene SNP and familial history of chordoma can increase the risk, although these cases are rare.

• #39 Local recurrence | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/local-recurrence/

  More than half of all chordoma tumors grow again after initial treatment. This is called a recurrence. It is not unusual for chordoma patients to have a recurrence several years after initial treatment. Many patients have more than one recurrence over time. […] There is no way to know for sure if a chordoma will recur because several factors affect your chance of having a recurrence. These include the size of your original tumor, how much of the tumor was removed during your initial surgery, your age, and the nature of your initial surgery and/or radiation. […] Chordomas that recur are difficult to control and are rarely curable. However, there are certain circumstances in which a recurrent chordoma might be cured with appropriate treatment. […] It is critical to know if a cure is possible or if the best outcome that can be expected is to extend life or improve quality of life. This knowledge can help you and your medical team pursue a treatment plan that offers the best outcome possible while avoiding treatments that can expose you to unnecessary or unacceptable risks.

• #40 Chordomas | Duke Health

  https://www.dukehealth.org/treatments/chordoma

  Chordomas, a very rare type of cancer, are often misdiagnosed. […] Fortunately, chordomas are usually caught before they metastasize. […] Chordomas have about a 30% to 50% chance of recurrence. And, because the cancer is so slow-growing, it can metastasize years after successful treatment. We follow a strict surveillance schedule to catch any regrowth as soon as possible. For the first two years, you’ll be asked to return regularly for follow-up appointments and imaging scans. After that, appointments will space out incrementally over the next eight years.

• #41 Chordomas | Duke Health

  https://www.dukehealth.org/treatments/chordoma

  Chordomas, a very rare type of cancer, are often misdiagnosed. […] Fortunately, chordomas are usually caught before they metastasize. […] Chordomas have about a 30% to 50% chance of recurrence. And, because the cancer is so slow-growing, it can metastasize years after successful treatment. We follow a strict surveillance schedule to catch any regrowth as soon as possible. For the first two years, you’ll be asked to return regularly for follow-up appointments and imaging scans. After that, appointments will space out incrementally over the next eight years.

• #42 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #43 Local recurrence | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/local-recurrence/

  For patients with isolated recurrences, high-dose radiation given with or without surgery provides the best chance of achieving a cure or long-term disease control. […] The Chordoma Global Consensus Group guidelines state that you should have an MRI every 6 months for the first 5 years after treatment. […] After 5 years, you should have an MRI at least once a year for 15 years.

• #44

  https://www.massgeneral.org/orthopaedics/oncology/conditions-and-treatments/chordoma

  Chordomas may develop at any age but usually occur between 40 and 70 years of age. […] About 300 patients are diagnosed with chordoma each year in the United States, and about 700 in all of Europe. […] Surveillance: After the initial postop visits (typically at 2 and 6 weeks), routine surveillance is for 10 years with imaging: Every 3 months for 2 years, Then every 6 months for 3 years, which equals 5 years of follow-up, Annual visit thereafter for 5 more years, which concludes the surveillance standard of care of 10 years (but most patients like to continue with an annual visit).

• #45 Thoracic Chordoma Misdiagnosed as Primary Adenocarcinoma of the Mediastinum

  https://www.jchestsurg.org/journal/view.html?uid=6241&vmd=Full

  Chordoma is a rare malignant bone tumor that likely arises from the embryonic notochord. […] However, the incidence and bodily distribution of chordoma are subject to debate. The US Surveillance, Epidemiology, and End Results registry lists an incidence of 0.8 per million persons per year, whereas in European and Taiwanese population-based studies, the respective rates are 0.18 and 0.52 per million persons per year. […] Most chordomas are sacrococcygeal (50%55%) in location, followed by the craniooccipital region (25%30%), cervical spine (8%), and lumbar spine (5%). Only 3% of all chordomas develop in the thoracic spine. […] Monitoring patients for at least 15 years is advised as surveillance for potential recurrence.

• #46 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #47 Whole genome sequencing of skull-base chordoma reveals genomic alterations associated with recurrence and chordoma-specific survival | Nature Communications

  https://www.nature.com/articles/s41467-021-21026-5

  Chordoma is a rare bone tumor with an unknown etiology and high recurrence rate. […] Based on the United States Surveillance Epidemiology and End Results data, the incidence of chordoma varies by gender and race, however, little is known about the etiologic factors that predispose to it. […] Chordomas are considered slow growing; however, the recurrence rate is high, especially among skull-base chordoma patients, largely due to incomplete tumor resection. […] The clinical progression of skull-base chordoma is highly variable, and there are no validated clinical or molecular prognostic panels available. […] A better understanding of the molecular processes in chordoma is critically needed to develop prognostic prediction tools and to discover druggable targets. […] Our findings suggest that the combination of SWI/SNF alterations and 22q deletion show a strong association with clinical outcomes, demonstrating the potential of designing a multi-marker panel in prognostic prediction.

• #48 The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study

  https://www.e-neurospine.org/journal/view.php?number=1607

  This study represents the first multicenter retrospective analysis in Korea on the treatment and prognosis of cervical spine chordoma. Despite the low incidence of cervical spine chordoma, this study conducted a comprehensive analysis of a total of 45 patients over an extended period. The 5-year cumulative recurrence rate was 51.3%, and the 10-year rate was 60%. The 5-year survival rate for cervical chordoma was 82%, and the 10-year survival rate was 53%. Analysis of prognostic factors found no statistically significant factors affecting local recurrence, with the only statistically significant factor influencing death being the age at the time of surgery. […] This nationwide multicenter retrospective study presented the clinical outcomes for cervical spine chordoma, revealing a 5-year cumulative LR rate was 51.3%, a 10-year cumulative LR rate was 60%, a 5-year OS rate of 82%, and a 10-year survival rate of 53%. Age was the only factor found to statistically significantly affect survival.

• #49 The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study

  https://www.e-neurospine.org/journal/view.php?number=1607

  This study represents the first multicenter retrospective analysis in Korea on the treatment and prognosis of cervical spine chordoma. Despite the low incidence of cervical spine chordoma, this study conducted a comprehensive analysis of a total of 45 patients over an extended period. The 5-year cumulative recurrence rate was 51.3%, and the 10-year rate was 60%. The 5-year survival rate for cervical chordoma was 82%, and the 10-year survival rate was 53%. Analysis of prognostic factors found no statistically significant factors affecting local recurrence, with the only statistically significant factor influencing death being the age at the time of surgery. […] This nationwide multicenter retrospective study presented the clinical outcomes for cervical spine chordoma, revealing a 5-year cumulative LR rate was 51.3%, a 10-year cumulative LR rate was 60%, a 5-year OS rate of 82%, and a 10-year survival rate of 53%. Age was the only factor found to statistically significantly affect survival.

• #50 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #51 Chordoma: Practice Essentials, History of the Disease, Epidemiology

  https://emedicine.medscape.com/article/1263740-overview

  Due to high local recurrence of chordomas, most physicians recommend lifelong surveillance with magnetic resonance imaging (MRI) with and without gadolinium contrast. Metastatic chordoma should be on the differential if new lesions arise elsewhere in the body, as up to 20% of chordomas can metastasize.

• #52 Local recurrence | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/local-recurrence/

  More than half of all chordoma tumors grow again after initial treatment. This is called a recurrence. It is not unusual for chordoma patients to have a recurrence several years after initial treatment. Many patients have more than one recurrence over time. […] There is no way to know for sure if a chordoma will recur because several factors affect your chance of having a recurrence. These include the size of your original tumor, how much of the tumor was removed during your initial surgery, your age, and the nature of your initial surgery and/or radiation. […] Chordomas that recur are difficult to control and are rarely curable. However, there are certain circumstances in which a recurrent chordoma might be cured with appropriate treatment. […] It is critical to know if a cure is possible or if the best outcome that can be expected is to extend life or improve quality of life. This knowledge can help you and your medical team pursue a treatment plan that offers the best outcome possible while avoiding treatments that can expose you to unnecessary or unacceptable risks.

• #53 Chordoma Survival | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD

  https://www.aaroncohen-gadol.com/en/patients/chordoma/survival/overview

  Chordoma is a rare, slow-growing type of bone cancer. It often occurs in the sacrum (sacral chordoma or tailbone chordoma), the spine, or the base of the skull (skull base chordoma). […] The survival rate after a diagnosis of chordoma depends on many factors, such as the specific type of chordoma, its location, the treatment, and your general health. Usually, half of those who are diagnosed with the condition survive at least 5 years. This is known as the 5-year survival rate. This rate can improve to 65% to 70% if the tumor is entirely removed by surgery. […] Conversely, if complete surgical removal is not feasible, the 5-year survival rate decreases to 40%. Additionally, individuals with an advanced stage of the disease generally experience a lower 5-year survival rate. […] Chordomas can be difficult to treat, and recurrence is common. A combination of radiation therapy and in rare cases, chemotherapy may be used to treat recurrent or advanced chordomas.

• #54

  https://journals.lww.com/co-oncology/fulltext/2019/03000/chordoma__update_on_disease,_epidemiology,_biology.10.aspx

  Chordoma is an exceedingly rare subtype of bone sarcoma. This review aims to provide a comprehensive insight into chordoma epidemiology, and an update on the recent advances in disease, biology and medical therapies. […] The incidence of chordoma is approximately 0.08/100 000 and the 5-year overall age-adjusted relative survival is 72% in the United States and 61% in Europe. […] Chordoma is a complex disease because of its rarity, biological heterogeneity and peculiar clinical behaviour.

• #55

  https://link.springer.com/article/10.1007/s10143-024-02815-0

  Chordomas of the skull base are rare, slow growing, locally invasive cancers with limited long-term survival analysis reported in the literature. […] 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. […] A SEER database analysis by Chambers et al. reported an overall three-, five-, and 10-year survival for cranial chordomas to be 80.9%, 73.5%, and 58.7% respectively. […] Another SBC SEER analysis identified several factors influencing survival, including age at diagnosis, tumor location, disease stage, surgical treatment, and tumor size. […] To date, no long-term (20-year), comprehensive multivariate analysis of survival in relation to demographic, disease, and treatment characteristics has been performed.

• #56 The Clinical Outcomes of Cervical Spine Chordoma: A Nationwide Multicenter Retrospective Study

  https://www.e-neurospine.org/journal/view.php?number=1607

  This study represents the first multicenter retrospective analysis in Korea on the treatment and prognosis of cervical spine chordoma. Despite the low incidence of cervical spine chordoma, this study conducted a comprehensive analysis of a total of 45 patients over an extended period. The 5-year cumulative recurrence rate was 51.3%, and the 10-year rate was 60%. The 5-year survival rate for cervical chordoma was 82%, and the 10-year survival rate was 53%. Analysis of prognostic factors found no statistically significant factors affecting local recurrence, with the only statistically significant factor influencing death being the age at the time of surgery. […] This nationwide multicenter retrospective study presented the clinical outcomes for cervical spine chordoma, revealing a 5-year cumulative LR rate was 51.3%, a 10-year cumulative LR rate was 60%, a 5-year OS rate of 82%, and a 10-year survival rate of 53%. Age was the only factor found to statistically significantly affect survival.

• #57 Sacral chordoma: a clinical review of 101 cases with 30-year experience in a single institution – University of Miami

  https://scholarship.miami.edu/esploro/outputs/journalArticle/Sacral-chordoma-a-clinical-review-of/991031723353902976

  Overall survival at 5 and 10 years for the primary tumors was 79% and 59%, respectively. […] For recurrent tumors, the 5- and 10-year overall survival was 65% and 40%, respectively. […] Using a combination of surgical resection and adjuvant radiotherapy allowed us to obtain a good overall survival, local relapse-free survival, and distant relapse-free survival in patients presenting with either a primary tumor or with a first time local recurrent tumor.

• #58

  https://link.springer.com/article/10.1007/s00586-018-5764-0

  The aim of this systematic review is to describe the epidemiology of chordoma and to provide a clear overview of clinical prognostic factors predicting progression-free and overall survival. […] Incidence rates ranged between 0.18 and 0.84 per million persons per year and varied between countries and presumably between races. On average patients were diagnosed in their late fifties and gender data indicate clear male predominance. […] Incidence rate and anatomical distribution vary between countries and presumably between races. Most chordomas arise in the skull base and sacrococcygeal spine, and the tumour shows clear male predominance. Multiple adverse prognostic factors predicting progression-free and overall survival were identified in subgroups of patients.

• #59 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  However, a systematic review of all chordomas revealed that female sex is an adverse prognostic factor for progression-free survival. […] Other adverse risk factors for progression-free survival include older age, large tumor size or spread, subtotal resection, metastasis, recurrence, and differentiation on histological analysis. […] There are no specific environmental risk factors for the onset of chordoma, and most individuals with chordoma lack a positive family history of chordoma, although a rare genetic predisposition may exist among individuals with familial chordoma, either with or without a germline duplication of the T gene.

• #60 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #61 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #62 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #63 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  However, a systematic review of all chordomas revealed that female sex is an adverse prognostic factor for progression-free survival. […] Other adverse risk factors for progression-free survival include older age, large tumor size or spread, subtotal resection, metastasis, recurrence, and differentiation on histological analysis. […] There are no specific environmental risk factors for the onset of chordoma, and most individuals with chordoma lack a positive family history of chordoma, although a rare genetic predisposition may exist among individuals with familial chordoma, either with or without a germline duplication of the T gene.

• #64 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  However, a systematic review of all chordomas revealed that female sex is an adverse prognostic factor for progression-free survival. […] Other adverse risk factors for progression-free survival include older age, large tumor size or spread, subtotal resection, metastasis, recurrence, and differentiation on histological analysis. […] There are no specific environmental risk factors for the onset of chordoma, and most individuals with chordoma lack a positive family history of chordoma, although a rare genetic predisposition may exist among individuals with familial chordoma, either with or without a germline duplication of the T gene.

• #65 Neuro-Ophthalmic Manifestations of Chordoma – EyeWiki

  https://eyewiki.org/Neuro-Ophthalmic_Manifestations_of_Chordoma

  However, a systematic review of all chordomas revealed that female sex is an adverse prognostic factor for progression-free survival. […] Other adverse risk factors for progression-free survival include older age, large tumor size or spread, subtotal resection, metastasis, recurrence, and differentiation on histological analysis. […] There are no specific environmental risk factors for the onset of chordoma, and most individuals with chordoma lack a positive family history of chordoma, although a rare genetic predisposition may exist among individuals with familial chordoma, either with or without a germline duplication of the T gene.

• #66 Whole genome sequencing of skull-base chordoma reveals genomic alterations associated with recurrence and chordoma-specific survival | Nature Communications

  https://www.nature.com/articles/s41467-021-21026-5

  We sought to identify genomic alterations that were associated with patient outcomes (17 deaths and 59 recurrences). […] After adjustment for age, sex, presurgery and post-surgery RT, PBRM1 alterations were significantly associated with worse CSS (hazard ratio (HR)=4.79, 95% confidence interval (CI)=1.5714.59, p=0.0058) and RFS (HR=5.72, 95% CI=2.6812.19, p=6.4106). […] In contrast, CDKN2A/B+ status (9p21.3 homozygous deletion, SVs involving the CDKN2A/2B loci, and CDKN2A mutation) was not significantly associated with CCS (HR=0.88, 95% CI=0.203.92, p=0.86) or RFS (HR=1.68, 95% CI=0.664.31, p=0.28). […] Chromosome 22q harbors an important SWI/SNF gene, SMARCB1/IN1, and the complete loss of SMARCB1 expression on immunohistochemistry due to homozygous SMARCB1 deletion has been used as a marker for a rare chordoma subtype, poorly differentiated chordoma. […] Our results suggest that partial inactivation of this gene was associated with worse patient outcomes and might be used as a prognostic marker in conventional chordoma.

• #67

  https://link.springer.com/article/10.1007/s10143-024-02815-0

  A total of 630 patients diagnosed with SBC between 2000 and 2020 were identified in the Surveillance, Epidemiology, and End Results (SEER) database. […] 87.3% of the identified cohort underwent surgical treatment for SBC, while 56.2% received RT. […] Treatment with both surgery and RT (whether neoadjuvant or adjuvant) was associated with improved OS at all time points compared to either unimodal or no treatment. […] Patients treated with RT only had the lowest survival rates with a five-year OS of 52.60%. […] These findings strongly suggest that a combination of surgery and radiation therapy yields the most favorable long-term survival outcomes for patients. […] Our findings regarding the survival benefits of surgery are consistent with previous research emphasizing the importance of surgical intervention in SBC management. […] Our analysis revealed a significant negative prognostic impact of chemotherapy, consistent with the general consensus that chordomas are relatively insensitive to conventional chemotherapy.

• #68

  https://link.springer.com/article/10.1007/s10143-024-02815-0

  A total of 630 patients diagnosed with SBC between 2000 and 2020 were identified in the Surveillance, Epidemiology, and End Results (SEER) database. […] 87.3% of the identified cohort underwent surgical treatment for SBC, while 56.2% received RT. […] Treatment with both surgery and RT (whether neoadjuvant or adjuvant) was associated with improved OS at all time points compared to either unimodal or no treatment. […] Patients treated with RT only had the lowest survival rates with a five-year OS of 52.60%. […] These findings strongly suggest that a combination of surgery and radiation therapy yields the most favorable long-term survival outcomes for patients. […] Our findings regarding the survival benefits of surgery are consistent with previous research emphasizing the importance of surgical intervention in SBC management. […] Our analysis revealed a significant negative prognostic impact of chemotherapy, consistent with the general consensus that chordomas are relatively insensitive to conventional chemotherapy.

• #69 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #70 Analysis of prognostic factors for survival in patients with primary spinal chordoma using the SEER Registry from 1973 to 2014 | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-018-0784-3

  Spinal chordomas are rare primary osseous tumors that arise from the remnants of the notochord. […] The aim of the study is to describe the epidemiological data and determine the prognostic factors for decreased survival in patients with primary spinal chordoma. […] The overall rate of distant metastatic cases in our cohort was only 7.7%. […] Age 60 years (HR = 2.72; 95%CI, 1.71 to 2.89), distant metastasis (HR = 2.16; 95%CI, 1.54 to 3.02), and non-surgical therapy (HR = 2.14; 95%CI, 1.72 to 2.69) were independent risk factors for survival reduction in analysis. […] Survival did not significantly differ as a factor of tumor site (vertebrae vs sacrum/pelvis) for primary spinal chordoma (HR = 0.93, P=0.16). […] Race (P=0.52), gender (P=0.11), marital status (P=0.94), and urban background (P=0.72) were not main factors which affected overall survival rate.

• #71 Conditional survival and changing risk profile in patients with chordoma: a population-based longitudinal cohort study | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-019-1225-7

  To evaluate the conditional survival of patients with chordoma to potentially help physician planning of optimal cancer surveillance and guide better clinical decisions. […] The prevalence of chordoma is less than 1 per 1,000,000 people. The incidence of chordoma peaks at 50-60 years of age and is very unlikely in patients younger than 40 years old. […] Previous surveys have shown that conditional survival can help physicians plan optimal cancer surveillance and guide better clinical decisions. Therefore, the conditional survival has substantial value for patients and medical professionals. […] Age at diagnosis, tumor size, and disease stage can influence the conditional survival for patients with chordoma. The trend of conditional survival differs in patients with different states. The HRs of different factors change over the survival time. Therefore, understanding the changing risk profile and conditional 5-year disease-specific survival of chordoma is critical for accurate clinical treatment guidance.

• #72 Conditional survival and changing risk profile in patients with chordoma: a population-based longitudinal cohort study | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-019-1225-7

  To evaluate the conditional survival of patients with chordoma to potentially help physician planning of optimal cancer surveillance and guide better clinical decisions. […] The prevalence of chordoma is less than 1 per 1,000,000 people. The incidence of chordoma peaks at 50-60 years of age and is very unlikely in patients younger than 40 years old. […] Previous surveys have shown that conditional survival can help physicians plan optimal cancer surveillance and guide better clinical decisions. Therefore, the conditional survival has substantial value for patients and medical professionals. […] Age at diagnosis, tumor size, and disease stage can influence the conditional survival for patients with chordoma. The trend of conditional survival differs in patients with different states. The HRs of different factors change over the survival time. Therefore, understanding the changing risk profile and conditional 5-year disease-specific survival of chordoma is critical for accurate clinical treatment guidance.

• #73 Chordoma Spontaneous Regression After COVID-19

  https://www.mdpi.com/1999-4915/17/1/10

  Spontaneous tumor regression has been reported in the literature for a long time. In some cases, regression is related to concurrent infections. Four cases of spontaneous chordoma regression have been reported in the literature, but none are related to coronavirus disease (COVID-19). Some patients with hematologic malignancies supposedly demonstrated remission induced by severe acute respiratory syndrome coronavirus 2 (SARS-CoV-2) infection. In this paper, we describe a rare case of spontaneous regression of a solid tumor and histologically and immunohistochemically proven chordoma after COVID-19 disease. […] Following the report of our unique case, it becomes imperative to explore the broader implications of such an interaction. This case, marking a possibly unprecedented occurrence in solid tumors, prompts a closer examination of the mechanisms at play. Interestingly, this phenomenon is not isolated to chordomas alone. Pasin et al. in 2020 published one case of transient remission of lymphoma during the course of COVID-19. The tumor was composed of NK/T-cells, which are mainly suppressed (or even functionally exhausted) in the context of COVID-19. This promotes abnormal immune responses, which are less effective, and a cytokine release syndrome. While both diseases were active together, COVID-19 leads to normal NK/T-cell suppression/exhaustion, which are the cells that form the tumor, leading to remission. Even after recovering from COVID-19, patients may still experience lymphoma recurrence.

• #74 Chordoma Spontaneous Regression After COVID-19

  https://www.mdpi.com/1999-4915/17/1/10

  We believe that in our case, the onset of COVID-19 with exacerbated immune response and improvement in anti-tumor immunity (detection and elimination), by both direct and cross-reactions, had a fundamental role in this surprising chordoma regression with a reduction of 98.9% in the tumor volume. As T-cells are clearly involved in the COVID-19 immune response and were found infiltrating the tumor, we concluded that this cell must represent a critical pathway to this event. Fever and cross-reaction with brachyury may also have enhanced this process. As the tumoral piece resected in surgery was too small, it was not possible to perform additional analysis to elicit the interactions and define the specific mechanism that led to this regression. We encourage further immune analysis in patients with chordoma and confirmed COVID-19 in order to gain a better understanding of the SARS-CoV-2 and chordoma interaction.

• #75 Chordoma Spontaneous Regression After COVID-19

  https://www.mdpi.com/1999-4915/17/1/10

  We believe that in our case, the onset of COVID-19 with exacerbated immune response and improvement in anti-tumor immunity (detection and elimination), by both direct and cross-reactions, had a fundamental role in this surprising chordoma regression with a reduction of 98.9% in the tumor volume. As T-cells are clearly involved in the COVID-19 immune response and were found infiltrating the tumor, we concluded that this cell must represent a critical pathway to this event. Fever and cross-reaction with brachyury may also have enhanced this process. As the tumoral piece resected in surgery was too small, it was not possible to perform additional analysis to elicit the interactions and define the specific mechanism that led to this regression. We encourage further immune analysis in patients with chordoma and confirmed COVID-19 in order to gain a better understanding of the SARS-CoV-2 and chordoma interaction.

• #76 Chordoma Spontaneous Regression After COVID-19

  https://www.mdpi.com/1999-4915/17/1/10

  Regarding chordomas, there are four cases of regression published. In one case, the patient had just a suspected diagnosis since no biopsy was performed. This patient presented partial regression after dermatologic infection by Mycobacterium marinum with a 33% reduction in its volume after regression. The other two cases had a confirmed diagnosis by histopathological analysis but none of them had positive brachyury. One had complete regression associated with post-biopsy Escherichia coli infection. The other patient had no report of concomitant infection but it is possible that some medication, including herbal supplement and oils, could have triggered the 61.8% reduction in volume after regression. In the most recently published case, not related to infection, diagnosis was confirmed by histopathological analysis and positive brachyury immunohistochemical staining. No measure of tumor volume was cited, only noticeable shrinkage of the tumor. Our case showed the most considerable volume reduction of 98.9% after regression and was both histopathologically and immunohistochemically proven to be chordoma.

• #77 Conditional survival and changing risk profile in patients with chordoma: a population-based longitudinal cohort study | Journal of Orthopaedic Surgery and Research | Full Text

  https://josr-online.biomedcentral.com/articles/10.1186/s13018-019-1225-7

  To evaluate the conditional survival of patients with chordoma to potentially help physician planning of optimal cancer surveillance and guide better clinical decisions. […] The prevalence of chordoma is less than 1 per 1,000,000 people. The incidence of chordoma peaks at 50-60 years of age and is very unlikely in patients younger than 40 years old. […] Previous surveys have shown that conditional survival can help physicians plan optimal cancer surveillance and guide better clinical decisions. Therefore, the conditional survival has substantial value for patients and medical professionals. […] Age at diagnosis, tumor size, and disease stage can influence the conditional survival for patients with chordoma. The trend of conditional survival differs in patients with different states. The HRs of different factors change over the survival time. Therefore, understanding the changing risk profile and conditional 5-year disease-specific survival of chordoma is critical for accurate clinical treatment guidance.

• #78 Whole genome sequencing of skull-base chordoma reveals genomic alterations associated with recurrence and chordoma-specific survival | Nature Communications

  https://www.nature.com/articles/s41467-021-21026-5

  Chordoma is a rare bone tumor with an unknown etiology and high recurrence rate. […] Based on the United States Surveillance Epidemiology and End Results data, the incidence of chordoma varies by gender and race, however, little is known about the etiologic factors that predispose to it. […] Chordomas are considered slow growing; however, the recurrence rate is high, especially among skull-base chordoma patients, largely due to incomplete tumor resection. […] The clinical progression of skull-base chordoma is highly variable, and there are no validated clinical or molecular prognostic panels available. […] A better understanding of the molecular processes in chordoma is critically needed to develop prognostic prediction tools and to discover druggable targets. […] Our findings suggest that the combination of SWI/SNF alterations and 22q deletion show a strong association with clinical outcomes, demonstrating the potential of designing a multi-marker panel in prognostic prediction.

• #79 Studies of familial and non-familial chordoma, a rare bone cancer – NCI

  https://dceg.cancer.gov/research/clinical-studies/chordoma

  Chordoma is a rare bone cancer that is diagnosed in only about 300 patients in the U.S. each year. […] Chordoma is diagnosed most often in people who are in their late 50s, but it can occur much earlier or later. It develops in males more often than females and, for unknown reasons, is rare in African Americans. […] No specific environmental factors that increase the risk of developing chordoma have been identified. […] However, a small number of families with multiple relatives with chordoma have been reported worldwide. […] Currently, there is no clear clinical guidance on patient stratification regarding treatment, such as post-surgery radiation therapy. […] Additionally, treatment options for chordoma patients, particularly those with advanced disease, are still limited. […] DCEG investigators are seeking chordoma families to participate in research to identify additional genes that increase the risk of family members developing chordoma. […] DCEG investigators are also seeking individuals with chordoma who are the only member of their family with this cancer.

• #80 Local recurrence | Chordoma Foundation

  https://www.chordomafoundation.org/treatment-guidelines/local-recurrence/

  For patients with isolated recurrences, high-dose radiation given with or without surgery provides the best chance of achieving a cure or long-term disease control. […] The Chordoma Global Consensus Group guidelines state that you should have an MRI every 6 months for the first 5 years after treatment. […] After 5 years, you should have an MRI at least once a year for 15 years.

• #81

  https://www.massgeneral.org/orthopaedics/oncology/conditions-and-treatments/chordoma

  Chordomas may develop at any age but usually occur between 40 and 70 years of age. […] About 300 patients are diagnosed with chordoma each year in the United States, and about 700 in all of Europe. […] Surveillance: After the initial postop visits (typically at 2 and 6 weeks), routine surveillance is for 10 years with imaging: Every 3 months for 2 years, Then every 6 months for 3 years, which equals 5 years of follow-up, Annual visit thereafter for 5 more years, which concludes the surveillance standard of care of 10 years (but most patients like to continue with an annual visit).

• #82 Chordoma Survival | Expert Surgeon | Aaron Cohen-Gadol, MD | Aaron Cohen-Gadol, MD

  https://www.aaroncohen-gadol.com/en/patients/chordoma/survival/overview

  Keep in mind that the 5-year survival rates are only estimates and that everyone’s chordoma experience is different. You should discuss your specific situation with your neurosurgeon. […] Regular follow-up care is essential for anyone with chordoma because the cancer can reappear. Close surveillance and monitoring can help to identify and treat any recurrence as early as possible. […] The outcome for patients with chordoma is generally better for those with a more localized, earlier stage tumor. Advances in treatment strategies, techniques, and technology have also improved the survival rates for those with chordoma. […] End-stage chordoma is the advanced phase of the disease, when the tumor has grown considerably and metastasized to other parts of the body, making it challenging to manage and treat.

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