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Surgical Neurology International 2024Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and...
BACKGROUND
Chordomas are rare, locally aggressive neoplasms recognized as derivatives of the notochord vestiges. These tumors typically involve the midline axial skeleton, and intracranial chordomas exhibit proclivity for the spheno-occipital region. However, purely intrasellar occurrences are extremely rare. We report a case of intrasellar chordoma, which masqueraded as a pituitary neuroendocrine tumor.
CASE DESCRIPTION
An 87-year-old female presented with an acutely altered mental state after a few-week course of headaches and decreased left vision. Adrenal insufficiency was evident, and magnetic resonance imaging revealed an intrasellar lesion with heterogeneous contrast enhancement and marked T2 hyperintensity. Central adrenal insufficiency due to an intrasellar lesion was suspected. Cortisol replacement was initiated, and transsphenoidal surgery was performed. Anterosuperior displacement of the normal pituitary gland and the absence of the bony dorsum sellae were notable during the procedure. Histological examination led to a diagnosis of conventional chordoma, and upfront adjuvant stereotactic radiosurgery was executed. She has been free from tumor progression for 12 months.
CONCLUSION
This case and literature review suggested that the pathognomonic features of intrasellar chordoma were heterogeneous contrast enhancement, marked T2 hyperintensity, osteolytic destruction of the dorsum sellae, and anterosuperior displacement of the pituitary gland. Clinical outcomes seemed slightly worse than those of all skull base chordomas, which were the rationale for upfront radiosurgery in our case. Neurosurgeons should include intrasellar chordomas in the differential diagnosis of intrasellar lesions, carefully dissect them from the adjacent critical anatomical structures, and consider upfront radiosurgery to achieve optimal patient outcomes.
PubMed: 38840611
DOI: 10.25259/SNI_116_2024 -
Surgical Neurology International 2024The radiographic presentation of the primary intraosseous cavernous hemangiomas (PICHs) is nonspecific. We report a case of clival PICH mimicking a chordoma with a...
BACKGROUND
The radiographic presentation of the primary intraosseous cavernous hemangiomas (PICHs) is nonspecific. We report a case of clival PICH mimicking a chordoma with a literature review.
CASE DESCRIPTION
A 57-year-old woman presented with diplopia that started a few days before the presentation. She had transient diplopia at the right lateral gaze and upper gaze with normal eye movement. The symptoms disappeared spontaneously 1 week later. She had no other complaints or neurological deficits. Computed tomography revealed an intraosseous mass lesion and bone erosion of the middle and lower clivus, extending laterally to the right occipital condyle. Magnetic resonance imaging (MRI) showed hyperintense and hypointense components on T2- and T1-weighted images, respectively. The lesion was larger than on MRI performed 10 years earlier. Chordoma or chondroma was considered a possible preoperative diagnosis. An endoscopic transsphenoidal approach removed the tumor. In the operating view, the lesion appeared as "moth-eaten" bony interstices filled with vascular soft tissue. Histologically, an intraosseous cavernous hemangioma was diagnosed.
CONCLUSION
Diagnosis before surgery is difficult without characteristic radiographic findings. When making a differential diagnosis of malignant skull lesions, PICH should be considered.
PubMed: 38840598
DOI: 10.25259/SNI_106_2024 -
ESMO Open Jun 2024We report on a series of consecutive patients with localized radiation-associated angiosarcoma (RAAS) of the breast region (BR) treated at two Italian sarcoma reference...
Outcome improvement with chemotherapy and radiotherapy in primary, localized, radiation-associated angiosarcoma of the breast region: a retrospective case series analysis.
BACKGROUND
We report on a series of consecutive patients with localized radiation-associated angiosarcoma (RAAS) of the breast region (BR) treated at two Italian sarcoma reference centers.
MATERIALS AND METHODS
We retrospectively reviewed all cases of primary, localized, resectable RAAS of the BR, treated at one of the two participating institutions from 2000 to 2019. Relapse-free survival (RFS) and overall survival (OS) were calculated. The prognostic role of several variables was investigated. A propensity score matched (PSM) analysis was carried out.
RESULTS
Eighty-four patients were retrospectively identified. Nineteen out of 84 patients (22.6%) were pretreated with an anthracycline-based regimen for previous cancer. All patients but one underwent surgery, with 37/84 (44.1%) receiving surgery alone and 46/84 (54.8%) a multimodal approach: 18/84 (21.4%) received radiation therapy (RT) and 46/84 (54.9%) received chemotherapy. An anthracycline-based regimen was used in 10/84 patients (11.9%), while a gemcitabine-based regimen was used in 33/84 (39.3%). With a median follow-up of 51 months (interquartile range: 30-126 months), 36/84 patients (42.9%) relapsed and 35/84 patients (41.7%) died (8/84, 9.5% in the lack of metastatic disease). Five-year OS and 5-year RFS were 57% [95% confidence interval (CI) 43% to 68%] and 52% (95% CI 39% to 63%), respectively. Both (neo)adjuvant RT and chemotherapy were associated with better RFS [hazard ratio (HR) 0.25, 95% CI 0.08-0.83; HR 0.45, 95% CI 0.23-0.89] with a trend towards a better OS (HR 0.51, 95% CI 0.18-1.46; HR 0.60, 95% CI 0.29-1.24). Gemcitabine-based regimens seemed to perform better (HR 4.28, 95% CI 1.29-14.14). PSM analysis retained the above results.
CONCLUSIONS
This retrospective study supports the use of (neo)adjuvant RT and chemotherapy, in primary, localized resectable RAAS of the BR. An effort to prospectively validate the role of (neo)adjuvant RT and chemotherapy is warranted.
PubMed: 38833974
DOI: 10.1016/j.esmoop.2024.103474 -
Photodiagnosis and Photodynamic Therapy May 2024Chordoma is a rare congenital low-grade malignant tumor characterized by infiltrative growth. It often tends to compress important intracranial nerves and blood vessels,...
BACKGROUND
Chordoma is a rare congenital low-grade malignant tumor characterized by infiltrative growth. It often tends to compress important intracranial nerves and blood vessels, making its surgical treatment extremely difficult. Besides, the efficacy of radiotherapy and chemotherapy is limited. The photosensitizer hematoporphyrin derivative (HPD) can emit red fluorescence under 405 nm excitation and produce reactive oxygen species for tumor therapy under 630 nm excitation. Herein, we investigated the effects of the photosensitizer hematoporphyrin derivative (HPD) on different cell lines of chordoma and xenograft tumors under 405 nm and 630 nm excitation.
METHODS
The photosensitizer hematoporphyrin derivative (HPD) and Two different chordoma cell lines (U-CH1, JHC7) were used for the test. The in vitro experiments were as follows: (1) the fluorescence intensity emitted by chordoma cells excited by different 405 nm light intensities was observed under a confocal microscope; (2) the Cell Counting Kit-8 (CCK-8) assay was performed to detect the effects of different photosensitizer concentrations and 630 nm light energy densities on the activity of chordoma cells. In the in vivo experiments, (3) Fluorescence visualization of chordoma xenograft tumors injected with photosensitizer via tail vein under 405 nm excitation; (4) Impact of 630 nm excitation of photosensitizer on the growth of chordoma xenograft tumors.
RESULTS
(1) The photosensitizers in chordoma cells and chordoma xenografts of nude mice were excited by 405nm to emit red fluorescence; (2) 630 nm excitation photosensitizer reduces chordoma cell activity and inhibits chordoma xenograft tumor growth in chordoma nude mice.
CONCLUSION
Photodynamic techniques mediated by the photosensitizer hematoporphyrin derivatives can be used for the diagnosis and treatment of chordoma.
PubMed: 38821238
DOI: 10.1016/j.pdpdt.2024.104231 -
Cancer Medicine Jun 2024We endeavored to introduce a novel scoring system (Lumbar Functional Index, LFI) capable of evaluating lumbar function in pelvic bone sarcoma patients who underwent...
AIMS
We endeavored to introduce a novel scoring system (Lumbar Functional Index, LFI) capable of evaluating lumbar function in pelvic bone sarcoma patients who underwent surgical resection and spinal pelvic fixation, while simultaneously identifying the incidence, outcomes, and risk factors of lumbar function impairment among these populations.
PATIENTS AND METHODS
A cohort of 304 primary bone sarcoma patients were recruited. The LFI was created based on the Oswestry Dysfunction Index (ODI) and Japanese Orthopaedic Association (JOA) scores. Lumbar function impairment was defined as LFI score ≥ 18 points, which was identified as high LFI. Demographic data, clinical characteristics, and oncological outcomes were analyzed.
RESULTS
The cohort included chondrosarcoma (39.8%), osteosarcoma (29.9%), Ewing sarcoma (8.6%), bone-derived undifferentiated pleomorphic sarcoma (7.2%), giant cell tumor of bone (7.2%), chordoma (2.3%), and other bone sarcomas (5.0%). The LFI score exhibited significant negative correlation with common scoring systems of bone sarcoma. The incidence of high LFI was 23.0%. Patients with high LFI demonstrated a higher prevalence of type I + II + III + IV pelvic tumor, more sacrificed nerve roots and bilateral lumbar spine fixation during surgery, while lower percentage of R0 resection and local control of pelvic tumor. Decreased median overall survival (30 vs. 52 months, p < 0.001) and recurrence-free survival (14 vs. 24 months, p < 0.001) time were observed in these patients. Type I + II + III + IV pelvic tumor and sacrificed nerve roots≥2 were identified as risk factors for high LFI, while R0 resection and local control were identified as protective factors.
CONCLUSION
The LFI scoring system exhibited a significant negative correlation to current scoring systems. High LFI patients had worse prognosis and distinct characteristics. The risk factors of high LFI included type I + II + III + IV pelvic tumor and sacrificed nerve roots≥2, and the protective factors included R0 resection and local control.
Topics: Humans; Male; Female; Bone Neoplasms; Adult; Middle Aged; Adolescent; Young Adult; Pelvic Bones; Sarcoma; Lumbar Vertebrae; Aged; Child; Risk Factors; Osteosarcoma; Lumbosacral Region
PubMed: 38819113
DOI: 10.1002/cam4.7282 -
Diagnostics (Basel, Switzerland) May 2024Chordomas are very rare malignant neoplasms of the bone occurring almost exclusively along the spine. As the tumours are thought to arise from notochordal remnants, the...
Chordomas are very rare malignant neoplasms of the bone occurring almost exclusively along the spine. As the tumours are thought to arise from notochordal remnants, the vast majority of chordomas express the gene, resulting in detectable nuclear amounts of its gene product brachyury. This T-Box transcription factor is commonly recognised as being essential in chordoma cells, and limiting expression is thought to be the key factor in controlling this tumour. Although the tumour is rare, distinct molecular differences and vulnerabilities have been described with regard to its location and the progression status of the disease, rendering it mandatory for novel cell lines to reflect all relevant chordoma subtypes. Here, we describe a novel chordoma cell line arising from the pleural effusion of a disseminated, poorly differentiated chordoma. This cell line, U-CH22, represents a highly aggressive terminal chordoma and, therefore, fills a relevant gap within the panel of available cell culture models for this orphan disease. CDK7 and CDK9 inhibition was lately identified as being effective in reducing viability in four chordoma cell lines, most likely due to a reduction in brachyury levels. In this study, we determined the capability of the CDK7 inhibitor THZ1 and the CDK1/2/5/9 inhibitor dinaciclib to reduce expression at mRNA and protein levels in a broad range of nine cell lines that are models of primary, recurrent, and metastasised chordoma of the clivus and the sacrum.
PubMed: 38786326
DOI: 10.3390/diagnostics14101028 -
Journal of Bone Oncology Jun 2024Primary malignant bone cancers have extremely low incidence, resulting in poor evaluation of their epidemiological characteristics. The objective of this study was to...
BACKGROUND
Primary malignant bone cancers have extremely low incidence, resulting in poor evaluation of their epidemiological characteristics. The objective of this study was to investigate trends in the incidence of primary malignant bone cancers and related mortality.
MATERIALS AND METHODS
Data from patients diagnosed with malignant bone cancers from 2000 to 2017 in the Surveillance Epidemiology and End Results database were retrospectively analyzed. Annual age-adjusted incidence and mortality were calculated, and the annual percentage change analyzed. Further, characteristics including patient age and sex, as well as the primary site and stage of different tumor types, were analyzed.
RESULTS
The overall age-adjusted incidence rate of primary malignant bone cancers was 7.70 per million people per year, and incidence rates had increased in patients between 60 and 79 years old, or with tumor size ≥ 8 cm. The incidence of chordoma increased significantly (annual percentage change (APC), 3.0 % per year), while those of WHO grade I and II primary bone cancers decreased. During 2000-2017, the mortality rate attributable to malignant bone cancers across the entire United States was 4.41 per million people per year. A positive mortality trend was observed during the study period (APC = 0.7 %, 95 % confidence interval: 0.0 %-1.5 %). Patients with osteosarcoma, and those who were female or of white ethnicity showed significant increasing trends in mortality rate.
CONCLUSIONS
Different tumor types have variable epidemiological manifestations, in terms of incidence and mortality, and exhibited altered trends over recent years. These variables can provide guidance to inform allocation of medical resources.
PubMed: 38778835
DOI: 10.1016/j.jbo.2024.100607 -
JPMA. the Journal of the Pakistan... Apr 2024Enbloc Sacrectomy is the procedure of choice for aggressive sacral lesions but not widely practiced in Pakistan, both by Neurosurgeons and Orthopaedic surgeons. Only one...
Enbloc Sacrectomy is the procedure of choice for aggressive sacral lesions but not widely practiced in Pakistan, both by Neurosurgeons and Orthopaedic surgeons. Only one case has been mentioned in indexed local literature so far and that too not operated in Pakistan. The case of a 27 year old neurologically intact male is presented. He had a huge residual mass and midline non-healing wound after two attempts at intralesional debulking and one full course of local irradiation. He presented to the Mayo Hospital, Lahore on 29th December 2021 for a redo surgery of sacral chordoma. A marginal excision was achieved utilizing posterior only approach. This case will help to understand the key steps in enbloc mid-Sacrectomy and importance of involving multidisciplinary team for ensuring adequate wound closure.
Topics: Humans; Chordoma; Male; Sacrum; Adult; Spinal Neoplasms; Reoperation
PubMed: 38751282
DOI: 10.47391/JPMA.9145 -
Journal of Orthopaedic Surgery (Hong... 2024Chordoma is a bone tumor that tends to occur in middle-aged and elderly people. It grows relatively slowly but is aggressive. The prognosis of middle-aged and elderly...
BACKGROUND
Chordoma is a bone tumor that tends to occur in middle-aged and elderly people. It grows relatively slowly but is aggressive. The prognosis of middle-aged and elderly patients with chordoma is quite different from that of young patients with chordoma.
OBJECTIVES
The purpose of the research was to construct a nomogram to predict the Individualized prognosis of middle-aged and elderly (age greater than or equal to 40 years) patients with chordoma.
METHODS
In this study, we screened 658 patients diagnosed with chordoma from 1983 to 2015 in the Surveillance, Epidemiology, and End Results (SEER) database. We determined the independently prognostic factors that affect the survival of patients by univariate and multivariate Cox proportional hazards model. Based on the independent prognostic factors, we constructed a nomogram to predict the overall survival (OS) rates of middle-aged and elderly patients with chordoma at 3 and 5 years. The validation of this nomogram was completed by evaluating the calibration curve and the C-index.
RESULTS
We screened a total of 658 patients and divided them into two cohort. Training cohort had 462 samples and validation cohort had 196 samples. The multivariate Cox proportional hazards model of the training group showed an association of age, tumor size, histology, primary site, surgery, and extent of disease with OS rates. Based on these results, we constructed the corresponding nomogram. The calibration curve and C-index showed the satisfactory ability of the nomogram in terms of predictive ability.
CONCLUSION
Nomogram can be an effective prognostic tool to assess the prognosis of middle-aged and elderly patients with chordoma and can help clinicians in medical decision-making and enable patients to receive more accurate and reasonable treatment.
Topics: Humans; Chordoma; Nomograms; SEER Program; Male; Female; Middle Aged; Aged; Prognosis; Bone Neoplasms; Adult; Survival Rate; Proportional Hazards Models; Age Factors; Aged, 80 and over
PubMed: 38744697
DOI: 10.1177/10225536241254208 -
Frontiers in Oncology 2024Cancer stem cells (CSCs), a group of tumor-initiating and tumor-maintaining cells, may be major players in the treatment resistance and recurrence distinctive of...
INTRODUCTION
Cancer stem cells (CSCs), a group of tumor-initiating and tumor-maintaining cells, may be major players in the treatment resistance and recurrence distinctive of chordoma. Characterizing CSCs is crucial to better targeting this subpopulation.
METHODS
Using flow cytometry, six chordoma cell lines were evaluated for CSC composition. In vitro, cell lines were stained for B7H6, HER2, MICA-B, ULBP1, EGFR, and PD-L1 surface markers. Eighteen resected chordomas were stained using a multispectral immunofluorescence (mIF) antibody panel to identify CSCs in vivo. HALO software was used for quantitative CSC density and spatial analysis.
RESULTS
In vitro, chordoma CSCs express more B7H6, MICA-B, and ULBP1, assessed by percent positivity and mean fluorescence intensity (MFI), as compared to non-CSCs in all cell lines. PD- L1 percent positivity is increased by >20% in CSCs compared to non-CSCs in all cell lines except CH22. In vivo, CSCs comprise 1.39% of chordoma cells and most are PD-L1+ (75.18%). A spatial analysis suggests that chordoma CSCs cluster at an average distance of 71.51 mm (SD 73.40 mm) from stroma.
DISCUSSION
To our knowledge, this study is the first to identify individual chordoma CSCs and describe their surface phenotypes using in vitro and in vivo methods. PD-L1 is overexpressed on CSCs in chordoma human cell lines and operative tumor samples. Similarly, potential immunotherapeutic targets on CSCs, including B7H6, MICA-B, ULBP1, EGFR, and HER2 are overexpressed across cell lines. Targeting these markers may have a preferential role in combating CSCs, an aggressive subpopulation likely consequential to chordoma's high recurrence rate.
PubMed: 38741774
DOI: 10.3389/fonc.2024.1376622