Browse
Submit Data
Databases
API
Help

Dataset Information

0 Views

0 Connections

0 Citations

0 Reanalyses

0 Downloads

Omics score: 0

Sacral chordoma: can local recurrence after sacrectomy be predicted?

ABSTRACT: Surgical resection margins are reportedly the most important predictor of survival and local recurrence with sacral chordomas. We examined the relevance of invasion of the surrounding posterior pelvic musculature (piriformis and gluteus maximus) at initial diagnosis to local recurrence after sacrectomy. We retrospectively reviewed 18 patients with histologically verified sacral chordoma seen at our institution between 1998 and 2005. There were 14 men and four women with a mean age of 65.1 years (range, 31-78 years). The average overall followup was 4.4 years (range, 0.5-10 years), 5.4 years for the living patients (range, 3-10 years), and 2.8 years for the deceased (range, 0.5-5.4 years). Local recurrence occurred in 12 patients (66%) 29 months postoperatively (range, 2-84 months). Six of these patients had wide excisions at initial surgery, five had marginal excisions, and one had an intralesional excision. Ten patients had wide surgical margins, six of whom (60%) had local recurrences. Tumor invasion of adjacent muscles at presentation was present in 14 patients, 12 of whom (85%) had local recurrences. Sacroiliac joint involvement was seen in 10 patients, nine of whom (90%) had local recurrences. The findings suggest obtaining wide surgical margins posteriorly, by excising parts of the piriformis, gluteus maximus, and sacroiliac joints, may result in better local disease control in patients with sacral chordoma.Level IV, prognostic study. See the Guidelines for Authors for a complete description of levels of evidence.

SUBMITTER: Hanna SA

PROVIDER: S-EPMC2493011 | biostudies-other | 2008 Sep

REPOSITORIES: biostudies-other

ACCESS DATA

Json Xml

Similar Datasets

Sacral chordoma with incidental rectal adenocarcinoma: a case report.

Project description:BackgroundWe report a unique case of synchronous sacrococcygeal chordoma in association with rectal invasive adenocarcinoma. Retrorectal tumors are a rare disease caused by a variety of pathologies. To our knowledge, no prior cases of such a coincidental finding of both cancers have been reported in the literature.Case presentationThis is the case of a 74-year-old white middle eastern man, with known hypertension under treatment, who presented with complaints of progressive lower back pain associated with urinary incontinence over the past 12 months. Magnetic resonance imaging (MRI) of the pelvis showed a large midline, well-defined, oval-shaped lesion replacing the sacrococcygeal portion of the spine, with extension to the presacral region. Computed tomography (CT)-guided Tru-Cut biopsy revealed features suggestive of chordoma. At surgery, we performed excision of the entire mass en bloc, sacrectomy with rectus abdominis myocutaneous flap reconstruction and end sigmoid colostomy. Surgical histopathology proved it to be sacral dedifferentiated chordoma and rectal invasive adenocarcinoma. Overall, the patient recovered well postoperatively, was discharged home with functional stoma and on permanent Foley catheter use.ConclusionTo the best of our knowledge, this is the only reported case of such a presentation, and sheds light on the approach and management. We hope that reporting such a case will add value to the medical literature.

| S-EPMC8040199 | biostudies-literature

Characterization of sacral chordoma and differential diagnosis from other sacral malignancy using [18F]FDG PET/CT.

Project description:2-Deoxy-2-[18F]fluoro-D-glucose ([18F]FDG) positron emission tomography (PET)/computed tomography (CT) is known to be a helpful imaging modality for sacral chordoma, but its detailed characteristics have not been fully described. The purpose of our study was to identify the [18F]FDG PET/CT imaging characteristics of sacral chordoma and compare them with other sacral malignancy. This retrospective study included patients who underwent [18F]FDG PET/CT because of a mass involving the sacrum. Investigated visual findings included visual score and distribution, and semiquantitative parameters measured included standardized uptake values (SUVmax, SUVpeak, SUVmean), tumor-to-liver ratio (TLR), metabolic tumor volume (MTV), total lesion glycolysis (TLG), and tumor size. Comparison studies and receiver operating characteristics (ROC) curve analysis were performed to differentiate between sacral chordoma and other sacral malignancy. Ten patients with sacral chordoma were finally included (M:F = 6:4, median age = 67 yr). On [18F]FDG PET/CT, sacral chordomas presented as a mass with minimal-moderate uptake with a usually heterogenous distribution. Compared with 12 patients with other sacral malignancies (M:F = 4:8, median age 42 yr), sacral chordoma showed a significantly lower TLR (median value 2.1 vs 6.3, P = .021). In ROC curve analysis, TLR showed the largest area under the curve (AUC) of 0.79 (cutoff ≤ 4.0; sensitivity 100.0%, specificity 58.3%; P = .004), and SUVmax showed the second largest AUC of 0.73 (cutoff ≤ 6.9; sensitivity 80.0%, specificity 66.7%; P = .034). [18F]FDG PET/CT of sacral chordoma showed minimal-moderate uptake. The TLR of [18F]FDG PET/CT was significantly lower than that of other sacral malignancy and was the most useful parameter for differentiating sacral chordoma, with the largest AUC. SUVmax could be another helpful semiquantitative parameter.

| S-EPMC10994510 | biostudies-literature

Local and Distant Recurrence in Resected Sacral Chordomas: A Systematic Review and Pooled Cohort Analysis.

Project description:Study designSystematic review.ObjectivesSacral chordomas are rare, primary tumors of the spine, best treated with en bloc resection. The purpose of this study was to assess the literature for resected sacral chordoma and to quantify the prevalence of, risk factors for, and treatment outcomes of local and distant recurrence therein.MethodsWe searched 5 online databases from January 1980 to May 2016 to find articles that report survival, recurrence outcomes, and/or prognostic factors for the resected sacral chordoma patient population. Characteristics and clinical outcomes of the pooled cohort are reported. Fisher exact tests, unpaired t tests, and one-way analysis of variance were used to investigate patient- and treatment-associated prognostic factors for local and distant recurrence. Survival analyses were performed for time to local recurrence and death. The protocol's PROSPERO ID is CRD42015024384.ResultsFifty-seven studies, with 1235 unique sacral chordoma patients, were included in this review. Local and distant recurrence occurred in 42.6% and 22.4% of patients with adequate follow-up, respectively. Kaplan-Meier overall median survival for patients with and without recurrence were 98 and 209 months after surgery, respectively. Wide surgical margin was associated with a lower rate of local recurrence; and wide surgical margin, female sex, and patient age ≥65 years were associated with lower rates of distant recurrence.ConclusionsWhile surgical margin remains the most significant prognostic factor for local and distant recurrence, combined surgical approach may be associated with local recurrence. Male sex and age <65 years may be associated with distant recurrence. Patients with risk factors for recurrence should undergo close monitoring to maximize survival.

| S-EPMC6448196 | biostudies-literature

Sacral chordoma: a diagnosis not to be sat on?

Project description:Sacral chordomas are rare, slow-growing tumours that are amenable to surgery, but unfortunately often diagnosed late. The aim of the study was to identify presenting symptoms, which may aid diagnosis and reduce the treatment time. Forty-four patients were identified with sacral chordoma between 1989 and 2006. Clinical and pathological records were reviewed retrospectively to elicit the symptoms recorded prior to diagnosis, duration of symptoms, surgical treatment, size of tumour and survival. Eleven patients were excluded, leaving 33 patients in the study group. Thirty-one patients had chordomas arising from the sacrum and two patients from the coccyx. The mean duration of symptoms prior to diagnosis was 120 weeks (2.3 years), with a median of length of 104 weeks (two years) and range of 26 to 416 weeks (0.5 to eight years). The mean maximum tumour size at resection was 8.3 cm, with a mean volume of 614 cm(3) (range 9-2,113 cm(3)). Pain, typically dull and worse with sitting, was the most common presenting symptom in 85% of patients. The classic symptoms of cauda equina (saddle anaesthesia, bladder or bowel dysfunction) occurred in 70% patients (23 patients). Sacral chordoma should be considered in cases of back pain with coccydynia, especially with neurological symptoms.

| S-EPMC2269011 | biostudies-other

Higher cMET dependence of sacral compared to clival chordoma cells: contributing to a better understanding of cMET in chordoma.

Project description:Chordomas are rare slow growing, malignant bone tumors of the axial skeleton with no approved medical treatment. As the majority of chordomas express cMET and its ligand, HGF, and crosstalks between EGFR and MET-signaling exist, we aimed to explore cMET activity in chordoma cell lines and clinical samples. We investigated nine chordoma patients and four chordoma cell lines for cMET expression. Two clival and two sacral chordoma cell lines were tested for chromosomal abnormalities of the MET gene locus; we studied the influence of HGF on the autocrine secretion and migration behavior, as well as protein expression and phosphorylation. Two MET/ALK inhibitors were investigated for their effects on cell viability, cell cycle, cyclin alterations, apoptosis, and downstream signaling pathways. Moderate and strong expression of membrane and cytoplasmic cMET in chordoma patients and cell lines used, as well as concentration-dependent increase in phospho cMET expression after HGF stimulation in all four chordoma cell lines was shown. U-CH2, MUG-Chor1, and UM-Chor1 are polysomic for MET. Chordoma cell lines secreted EGF, VEGF, IL-6, and MMP9 upon HGF-stimulation. Sacral cell lines showed a distinct HGF-induced migration. Both inhibitors dose-dependently inhibited cell growth, induce apoptosis and cell-cycle arrest, and suppress downstream pathways. Heterogeneous responses obtained in our in vitro setting indicate that cMET inhibitors alone or in combination with other drugs might particularly benefit patients with sacral chordomas.

| S-EPMC8203686 | biostudies-literature

miRNA profiling of sacral chordoma and nucleus pulposus cells

Project description:Next-generation RNA sequencing (NGS) was used for the combinatorial analysis of mRNA-miRNA gene expression profiles in sacral chordoma and nucleus pulposus samples, and to predict miRNA-mRNA regulatory networks with altered activity in chordoma. We identified a gene set including key regulators of the Hippo pathway, which is targeted by differently expressed miRNAs, and validated their altered expression by RT-qPCR. These newly identified miRNA/RNA interactions are predicted to have a role in the self-renewal process of chordoma stem cells, which might sustain the high rate of recurrence for this tumor.

2022-07-28 | GSE209695 | GEO

Spinal column chordoma: prognostic significance of clinical variables and T (brachyury) gene SNP rs2305089 for local recurrence and overall survival.

Project description:BackgroundChordomas are rare, locally aggressive bony tumors associated with poor outcomes. Recently, the single nucleotide polymorphism (SNP) rs2305089 in the T (brachyury) gene was strongly associated with sporadic chordoma development, but its clinical utility is undetermined.MethodsIn 333 patients with spinal chordomas, we identified prognostic factors for local recurrence-free survival (LRFS) and overall survival and assessed the prognostic significance of the rs2305089 SNP.ResultsThe median LRFS was 5.2 years from the time of surgery (95% CI: 3.8-6.0); greater tumor volume (≥100cm3) (hazard ratio [HR] = 1.99, 95% CI: 1.26-3.15, P = .003) and Enneking inappropriate resections (HR = 2.35, 95% CI: 1.37-4.03, P = .002) were independent predictors of LRFS. The median overall survival was 7.0 years (95% CI: 5.8-8.4), and was associated with older age at surgery (HR = 1.11 per 5-year increase, 95% CI: 1.02-1.21, P = .012) and previous surgical resection (HR = 1.73, 95% CI: 1.03-2.89, P = .038). One hundred two of 109 patients (93.6%) with available pathologic specimens harbored the A variant at rs2305089; these patients had significantly improved survival compared with those lacking the variant (P = .001), but there was no association between SNP status and LRFS (P = .876).ConclusionsThe ability to achieve a wide en bloc resection at the time of the primary surgery is a critical preoperative consideration, as subtotal resections likely complicate later management. This is the first time the rs2305089 SNP has been implicated in the prognosis of individuals with chordoma, suggesting that screening all patients may be instructive for risk stratification.

| S-EPMC5464366 | biostudies-literature

Whole genome sequencing of skull-base chordoma reveals genomic alterations associated with recurrence and chordoma-specific survival.

Project description:Chordoma is a rare bone tumor with an unknown etiology and high recurrence rate. Here we conduct whole genome sequencing of 80 skull-base chordomas and identify PBRM1, a SWI/SNF (SWItch/Sucrose Non-Fermentable) complex subunit gene, as a significantly mutated driver gene. Genomic alterations in PBRM1 (12.5%) and homozygous deletions of the CDKN2A/2B locus are the most prevalent events. The combination of PBRM1 alterations and the chromosome 22q deletion, which involves another SWI/SNF gene (SMARCB1), shows strong associations with poor chordoma-specific survival (Hazard ratio [HR] = 10.55, 95% confidence interval [CI] = 2.81-39.64, p = 0.001) and recurrence-free survival (HR = 4.30, 95% CI = 2.34-7.91, p = 2.77 × 10-6). Despite the low mutation rate, extensive somatic copy number alterations frequently occur, most of which are clonal and showed highly concordant profiles between paired primary and recurrence/metastasis samples, indicating their importance in chordoma initiation. In this work, our findings provide important biological and clinical insights into skull-base chordoma.

| S-EPMC7859411 | biostudies-literature

A Dosiomics Analysis Based on Linear Energy Transfer and Biological Dose Maps to Predict Local Recurrence in Sacral Chordomas after Carbon-Ion Radiotherapy.

Project description:Carbon Ion Radiotherapy (CIRT) is one of the most promising therapeutic options to reduce Local Recurrence (LR) in Sacral Chordomas (SC). The aim of this work is to compare the performances of survival models fed with dosiomics features and conventional DVH metrics extracted from relative biological effectiveness (RBE)-weighted dose (DRBE) and dose-averaged Linear Energy Transfer (LETd) maps, towards the identification of possible prognostic factors for LR in SC patients treated with CIRT. This retrospective study included 50 patients affected by SC with a focus on patients that presented a relapse in a high-dose region. Survival models were built to predict both LR and High-Dose Local Recurrencies (HD-LR). The models were evaluated through Harrell Concordance Index (C-index) and patients were stratified into high/low-risk groups. Local Recurrence-free Kaplan-Meier curves were estimated and evaluated through log-rank tests. The model with highest performance (median(interquartile-range) C-index of 0.86 (0.22)) was built on features extracted from LETd maps, with DRBE models showing promising but weaker results (C-index of 0.83 (0.21), 0.80 (0.21)). Although the study should be extended to a wider patient population, LETd maps show potential as a prognostic factor for SC HD-LR in CIRT, and dosiomics appears to be the most promising approach against more conventional methods (e.g., DVH-based).

| S-EPMC9817801 | biostudies-literature

Radiotherapy for Mobile Spine and Sacral Chordoma: A Critical Review and Practical Guide from the Spine Tumor Academy.

Project description:Chordomas are rare tumors of the embryologic spinal cord remnant. They are locally aggressive and typically managed with surgery and either adjuvant or neoadjuvant radiation therapy. However, there is great variability in practice patterns including radiation type and fractionation regimen, and limited high-level data to drive decision making. The purpose of this manuscript was to summarize the current literature specific to radiotherapy in the management of spine and sacral chordoma and to provide practice recommendations on behalf of the Spine Tumor Academy. A systematic review of the literature was performed using the Preferred Reporting Items for Systematic reviews and Meta-Analyses (PRISMA) approach. Medline and Embase databases were utilized. The primary outcome measure was the rate of local control. A detailed review and interpretation of eligible studies is provided in the manuscript tables and text. Recommendations were defined as follows: (1) consensus: approved by >75% of experts; (2) predominant: approved by >50% of experts; (3) controversial: not approved by a majority of experts. Expert consensus supports dose escalation as critical in optimizing local control following radiation therapy for chordoma. In addition, comprehensive target volumes including sites of potential microscopic involvement improve local control compared with focal targets. Level I and high-quality multi-institutional data comparing treatment modalities, sequencing of radiation and surgery, and dose/fractionation schedules are needed to optimize patient outcomes in this locally aggressive malignancy.

| S-EPMC10136664 | biostudies-literature

OmicsDI is part of the ELIXIR infrastructure

OmicsDI is an Elixir interoperability service. Learn more ›

Tweets

OmicsDI Databases

PRIDE
PeptideAtlas
MassIVE
JPOST Repository
Physiome Model Repository

EGA
EVA
ENA
LINCS
PAXDB
Cell Collective

MetaboLights
Metabolomics Workbench
MetabolomeExpress
GNPS
BioModels
FAIRDOMHub

ArrayExpress
dbGaP
ExpressionAtlas
GEO
NODE

Information

Databases
Help
API
Contact us
Code on GitHub
Terms of use
Submit Data