Thoracic dumbbell spinal chordoma mimicking a schwannoma

Background: Epidural dumbbell-shaped chordomas are localized slow growing, and malignant/aggressive neoplasms. Here, we present a 62-year-old male with a T3-T4 dumbbell-shaped chordoma and reviewed the appropriate literature. Case Description: A 62-year-old male presented with a three-month history of thoracic pain. When the thoracolumbar magnetic resonance (MR) showed a T3-T4 dumbbell-shaped intracanalicular/extradural tumor, he underwent tumor removal. After the histological examination proved the lesion was a spinal chordoma, he underwent a secondary radical transthoracic tumor resection. Postoperatively, the patient was able to walk without assistance, and at 6-month follow-up, was neurologically intact with only residual paresthesias. Conclusion: Malignant spinal chordomas may mimic benign neurinomas on MR scans. Here, biopsy of the lesion to confirm the diagnosis of chordoma was critical and directed subsequent definitive transthoracic tumor resection.

The Sonic Hedgehog Signaling and Its Components in Recurrent Chordoma of the Spine

Objective Chordomas are uncommon primary malignant tumours that have a high rate of recurrence. They are thought to form along the spine from remains of the embryonic notochord. Treatment for recurrent tumours is complicated and contentious.They are unresponsive to conventional chemotherapy and radiotherapy. Chordomomas simply lack a viable chemotherapeutic standard. Throughout the fetus's development, the Sonic Hedgehog (SHH) pathways connecting a variety of processes involved in tissue and organ expansion and differentiation. To investigate the role of signalling the hedgehog in recurrent spinal chordomas, immunohistochemistry was used to identify SHH and GLI1 levels. In situ hybridization was also used to differentiate PTCH1 and GLI1 expressions. Methods From 1997 to 2020, we looked at 23 paraffin-embedded recurrent spinal chordoma samples from 23 patients (9 men, 14 women; median age: 63 years). All the patients were treated at the University Medical Center Goettingen in Germany and Azad University of Medical Sciences in Tehran, Iran. This study only included patients who had been diagnosed with conventional chordoma. Results SHH expression (+) and GLI1 expression were discovered in all 23 cases (+) immunohistochemically. GLI1 and SHH levels were markedly increased by recurrent spinal chordoma scores. In the recurrent spinal chordoma, in situ hybridization demonstrated positive responses for PTCH1 and GLI1. Conclusion The Shh sample that represents is believed to play a role in spinal chordoma recurrence.The increased amounts of SHH and GLI1 activity in all chordoma samples, according to the study, indicate an autocrine ligand-dependent activation of the conventional HH signalling cacade. It's hard to rule out a non-canonical or paracrine pathway. Hedgehoginhibitors, such as SHH- and GLl-inhibitors, are believed to be associated in our findings, could be a promising approach for treating recurrent spinal chordomas.

High-dose hypofractionated stereotactic body radiotherapy for spinal chordoma

OBJECTIVE Spinal chordoma is locally aggressive and has a high rate of recurrence, even after en bloc resection. Conventionally fractionated adjuvant radiation leads to suboptimal tumor control, and data regarding hypofractionated regimens are limited. The authors hypothesized that neoadjuvant stereotactic body radiotherapy (SBRT) may overcome its intrinsic radioresistance, improve surgical margins, and allow preservation of critical structures during surgery. The purpose of this study is to review the feasibility and early outcomes of high-dose hypofractionated SBRT, with a focus on neoadjuvant SBRT. METHODS Electronic medical records of patients with spinal chordoma treated using image-guided SBRT between 2009 and 2019 at a single institution were retrospectively reviewed. RESULTS Twenty-eight patients with 30 discrete lesions (24 in the mobile spine) were included. The median follow-up duration was 20.8 months (range 2.3–126.3 months). The median SBRT dose was 40 Gy (range 15–50 Gy) in 5 fractions (range 1–5 fractions). Seventeen patients (74% of those with newly diagnosed lesions) received neoadjuvant SBRT, of whom 15 (88%) underwent planned en bloc resection, all with negative margins. Two patients (12%) developed surgical wound-related complications after neoadjuvant SBRT and surgery, and 4 (two grade 3 and two grade 2) experienced postoperative complications unrelated to the surgical site. Of the remaining patients with newly diagnosed lesions, 5 received adjuvant SBRT for positive or close surgical margins, and 1 received SBRT alone. Seven recurrent lesions were treated with SBRT alone, including 2 after failure of prior conventional radiation. The 2-year overall survival rate was 92% (95% confidence interval [CI] 71%–98%). Patients with newly diagnosed chordoma had longer median survival (not reached) than those with recurrent lesions (27.7 months, p = 0.006). The 2-year local control rate was 96% (95% CI 74%–99%). Among patients with radiotherapy-naïve lesions, no local recurrence was observed with a biologically effective dose ≥ 140 Gy, maximum dose of the planning target volume (PTV) ≥ 47 Gy, mean dose of the PTV ≥ 39 Gy, or minimum dose to 80% of the PTV ≥ 36 Gy (5-fraction equivalent doses). All acute toxicities from SBRT were grade 1–2, and no myelopathy was observed. CONCLUSIONS Neoadjuvant high-dose, hypofractionated SBRT for spinal chordoma is safe and does not increase surgical morbidities. Early outcomes at 2 years are promising, although long-term follow-up is pending.

Incidence, Management, and Outcomes of Adult Spinal Chordoma Patients in the United States

Study Design: Retrospective cohort study. Objective: Spinal chordomas are rare primary malignant neoplasms of the primitive notochord. They are slow growing but locally aggressive lesions that have high rates of recurrence and metastasis after treatment. Gold standard treatment remains en-bloc surgical resection with questionable efficacy of adjuvant therapies such as radiation and chemotherapy. Here we provide a comprehensive analysis of prognostic factors, treatment modalities, and survival outcomes in patients with spinal chordoma. Methods: Patients with diagnosis codes specific for chordoma of spine, sacrum, and coccyx were queried from the National Cancer Database (NCDB) during the years 2004-2016. Outcomes were investigated using Cox univariate and multivariate regression analyses, and survival curves were generated for comparative visualization. Results: 1,548 individuals were identified with a diagnosis of chordoma, 60.9% of which were at the sacrum or coccyx and 39.1% at the spine. The mean overall survival of patients in our cohort was 8.2 years. Increased age, larger tumor size, and presence of metastases were associated with worsened overall survival. 71.2% of patients received surgical intervention and both partial and radical resection were associated with significantly improved overall survival ( P < 0.001). Neither radiotherapy nor chemotherapy administration improved overall survival; however, amongst patients who received radiation, those who received proton-based radiation had significantly improved overall survival compared to traditional radiation. Conclusion: Surgical resection significantly improves overall survival in patients with spinal chordoma. In those patients receiving radiation, those who receive proton-based modalities have improved overall survival. Further studies into proton radiotherapy doses are required.

A Novel Nomogram for Predicting Cancer-Specific Survival in Patients With Spinal Chordoma: A Population-Based Analysis

Background: Chordoma is a rare malignant bone tumor, and the survival prediction for patients with chordoma is difficult. The objective of this study was to construct and validate a nomogram for predicting cancer-specific survival (CSS) in patients with spinal chordoma. Methods: A total of 316 patients with spinal chordoma were identified from the SEER database between 1998 and 2015. The independent prognostic factors for patients with spinal chordoma were determined by univariate and multivariate Cox analyses. The prognostic nomogram was established for patients with spinal chordoma based on independent prognostic factors. Furthermore, we performed internal and external validations for this nomogram. Results: Primary site, disease stage, histological type, surgery, and age were identified as independent prognostic factors for patients with spinal chordoma. A nomogram for predicting CSS in patients with spinal chordoma was constructed based on the above 5 variables. In the training cohort, the area under the curve for predicting 1-, 3-, and 5-year CSS were 0.821, 0.856, and 0.920, respectively. The corresponding area under the curve in the validation cohort were 0.728, 0.804, and 0.839, respectively. The calibration curves of the nomogram showed a high degree of agreement between the predicted and the actual results, and the decision curve analysis further demonstrated the satisfactory clinical utility of the nomogram. Conclusions: The prognostic nomogram provides a considerably more accurate prediction of prognosis for patients with spinal chordoma. Clinicians can use it to categorize patients into different risk groups and make personalized treatment methods.