Combined Transpetrosal Approach: 2-Dimensional Operative Video.

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: This approach is suitable for petroclival lesions medial to V cranial nerve that extend in both middle and posterior fossa. It provides multiple surgical corridors with minimal brain retraction. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Several critical neurovascular structures of the petrous bone are at risk during the approach. Meticulous reading of the preoperative images is fundamental. It is mandatory to perform a thin section computed tomography scan, an MRI, and, in case of petroclival meningiomas, a digital subtraction angiography. ESSENTIAL STEPS OF THE PROCEDURE: In the first illustrative case, we present our current "mini-combined petrosectomy" with minimal drilling of the labyrinth. Positioning, skin incision, and craniotomy are illustrated in the video. Once all the important neurovascular structures are identified, we perform the anterior and the posterior petrosectomy, with preservation of the endolymphatic sac. We continue with dura mater opening and tentorium cutting. After tumor removal, we can appreciate an unique view of ipsilateral and contralateral cranial nerves, as well as pituitary stalk and major arteries. PITFALLS/AVOIDANCE OF COMPLICATIONS: To avoid injuries to the main neurovascular structures, neuronavigation, neuromonitoring, and Doppler can be useful. VARIANTS AND INDICATIONS FOR THEIR USE: The second illustrative case shows an extension of the combined petrosectomy to the anterior fossa, this made possible to perform a transsylvian approach for this giant sphenopetroclival meningioma. The patients consented to the procedure and to the publication of his/her images. Appropriate consent was obtained for the publication of the cadaveric images.

Tailored frontotemporoorbitozygomatic (FTOZ) craniotomy for a large anterior clinoidal meningioma.

Large anterior clinoidal meningiomas are tumors that arise on the anterior clinoid often compressing and encasing the nearby neurovascular structures such as the carotid artery and the optic nerve. These remain as very challenging cases for neurosurgeons because of the issues concerning preservation of critical structures and gross total excision. In this video submission, we will show a case of a large anterior clinoidal meningioma through a tailored frontotemporoorbitozygomotic craniotomy with emphasis on anterior clinoidectomy and the different corridors that can be obtained by this particular approach. The methodical dissection of the tumor and the other critical structures can also be seen.

The mutational landscape of skull base and spinal chordomas and the identification of potential prognostic and theranostic biomarkers.

OBJECTIVE: Chordomas are rare bone neoplasms characterized by a high recurrence rate and no benefit from any approved medical treatment to date. However, the investigation of molecular alterations in chordomas could be essential to prognosticate, guide clinical decision-making, and identify theranostic biomarkers. The aim of this study was to provide a detailed genomic landscape of a homogeneous series of 64 chordoma samples, revealing driver events, theranostic markers, and outcome-related genomic features. METHODS: The authors conducted whole-exome sequencing (WES), targeted next-generation sequencing, and RNA sequencing of 64 skull base and spinal chordoma samples collected between December 2006 and September 2020. Clinical, histological, and radiological data were retrospectively analyzed and correlated to genetic findings. RESULTS: The authors identified homozygous deletions of CDKN2A/2B, PIK3CA mutations, and alterations affecting genes of SWI/SNF chromatin remodeling complexes (PBRM1 and ARID1A) as potential theranostic biomarkers. Using matched germline WES, they observed a higher frequency of a common genetic variant (rs2305089; p.(Gly177Asp)) in TBXT (97.8%, p < 0.001) compared to its distribution in the general population. PIK3CA mutation was identified as an independent biomarker of short progression-free survival (HR 10.68, p = 0.0008). Loss of CDKN2A/2B was more frequently observed in spinal tumors and recurrent tumors. CONCLUSIONS: In the current study, the authors identified driver events such as PBRM1 and PIK3CA mutations, TBXT alterations, or homozygous deletions of CDKN2A/2B, which could, for some, be considered potential theranostic markers and could allow for identifying novel therapeutic approaches. With the aim of a future biomolecular prognostication classification, alterations affecting PIK3CA and CDKN2A/2B could be considered as poor prognostic biomarkers.

Opposed evolution of the osseous and soft parts of progestin-associated osteomeningioma after progestin intake discontinuation.

OBJECTIVE: Numerous studies have confirmed a strong association between progestins and meningiomas and the regression and/or stabilization of meningiomas after discontinuation of treatment. Osteomeningiomas represent a small subgroup of meningiomas that appear to be more common among progestin-related meningiomas. However, the specificity of the behavior of this subset of meningiomas after discontinuation of progestin has not yet been assessed. METHODS: Thirty-six patients (mean age 49.5 years) who presented with at least one progestin-related osteomeningioma (48 tumors total) were identified from a prospectively collected database of patients and had been referred to our department for meningioma and had documented use of cyproterone acetate, nomegestrol acetate, and/or chlormadinone acetate. Hormonal treatment was stopped at the time of diagnosis for all the patients, and the clinical and radiological evolution of this subgroup of tumors was evaluated. RESULTS: For half of the 36 patients, treatment was prescribed for signs of hyperandrogenism, such as hirsutism, alopecia, or acne. Most lesions were spheno-orbital (35.4%) or frontal (31.2%). Although the tissular part of the meningioma shrank in 77.1% of cases, the osseous part exhibited discordant behavior with 81.3% showing volume progression. The combination of estrogens, as well as the prolonged duration of progestin treatment, seems to increase the risk of progression of the osseous part after treatment discontinuation (p = 0.02 and p = 0.028, respectively). No patient required surgical treatment at diagnosis or during the study. CONCLUSIONS: These results show that while the soft intracranial part of progestin-related osteomeningioma tumor is the most likely to regress after treatment discontinuation, the bony part is more likely to increase in volume. These findings suggest the need for careful follow-up of these patients, especially those with tumors near the optical apparatus.

Comparative analysis of histopathological parameters, genome-wide copy number alterations, and variants in genes involved in cell cycle regulation in chordomas of the skull base and sacrum.

Chordomas are rare tumors of the axial skeleton that are refractory to conventional therapy. Few studies have compared the morphological and molecular characteristics of chordomas according to the skull base and sacral locations. Histopathological data and changes revealed by array comparative genomic hybridization (CGH) and next-generation sequencing (NGS) of cell cycle regulation genes were analyzed for 28 skull base (SBCs) and 15 sacral (SC) chordomas. All cases were conventional chordomas. SBCs were significantly more frequent in patients aged <40 years and SCs predominated in patients aged >60 years. Mitotic indices ≥2 mitoses/10 high-power fields were correlated with high degrees of nuclear atypia and Ki67 labeling indices ≥6%. We identified 321 genomic positions, and copy number variation losses were more frequent than gain. Moreover, we report a panel of 85 genetic variants of cell cycle genes and the presence of molecular clusters for chordoma as well in CGH as in NGS. These new data strengthen the view that the chordoma should not be considered as a single molecular entity.

Surgical morbidity of the extradural anterior petrosal approach: the Lariboisière experience.

OBJECTIVE: Concerns about the approach-related morbidity of the extradural anterior petrosal approach (EAPA) have been raised, especially regarding temporal lobe and venous injuries, hearing impairment, facial nerve palsy, cerebrospinal fluid fistula, and seizures. There is lack in the literature of studies with detailed analysis of surgical complications. The authors have presented a large series of patients who were treated with EAPA, focusing on complications and their avoidance. METHODS: The authors carried out a retrospective review of patients who underwent EAPA at their institution between 2012 and 2021. They collected preoperative clinical characteristics, operative reports, operative videos, findings on neuroimaging, histological diagnosis, postoperative course, and clinical status at last follow-up. For pathologies without petrous bone invasion, the amount of petrous apex drilling was calculated and classified as low (< 70% of the volume) or high (≥ 70%). Complications were dichotomized as approach related and resection related. RESULTS: This study included 49 patients: 26 with meningiomas, 10 brainstem cavernomas, 4 chondrosarcomas, 4 chordomas, 2 schwannomas, 1 epidermoid cyst, 1 cholesterol granuloma, and 1 osteoblastoma. The most common approach-related complications were temporal lobe injury (6.1% of patients), seizures (6.1%), pseudomeningocele (6.1%), hearing impairment (4.1%), and dry eye (4.1%). Approach-related complications occurred most commonly in patients with a meningioma (p = 0.02) and Meckel's cave invasion (p = 0.02). Gross-total or near-total resection was correlated with a higher rate of tumor resection-related complications (p = 0.02) but not approach-related complications (p = 0.76). Inferior, lateral, and superior tumoral extension were not correlated with a higher rate of tumor resection-related complications. No correlation was found between high amount of petrous bone drilling and approach- or resection-related complications. CONCLUSIONS: EAPA is a challenging approach that deals with critical neurovascular structures and demands specific skills to be safely performed. Contrary to general belief, its approach-related morbidity seems to be acceptable at dedicated skull base centers. Morbidity can be lowered with careful examination of the preoperative neuroradiological workup, appropriate patient selection, and attention to technical details.

In vivo efficacy assessment of the CDK4/6 inhibitor palbociclib and the PLK1 inhibitor volasertib in human chordoma xenografts.

Background: Management of advanced chordomas remains delicate considering their insensitivity to chemotherapy. Homozygous deletion of the regulatory gene CDKN2A has been described as the most frequent genetic alteration in chordomas and may be considered as a potential theranostic marker. Here, we evaluated the tumor efficacy of the CDK4/6 inhibitor palbociclib, as well as the PLK1 inhibitor volasertib, in three chordoma patient-derived xenograft (PDX) models to validate and identify novel therapeutic approaches. Methods: From our chordoma xenograft panel, we selected three models, two of them harboring a homozygous deletion of CDKN2A/2B genes, and the last one a PBRM1 pathogenic variant (as control). For each model, we tested the palbociclib and volasertib drugs with pharmacodynamic studies together with RT-PCR and RNAseq analyses. Results: For palbociclib, we observed a significant tumor response for one of two models harboring the deletion of CDKN2A/2B (p = 0.02), and no significant tumor response in the PBRM1-mutated PDX; for volasertib, we did not observe any response in the three tested models. RT-PCR and RNAseq analyses showed a correlation between cell cycle markers and responses to palbociclib; finally, RNAseq analyses showed a natural enrichment of the oxidative phosphorylation genes (OxPhos) in the palbociclib-resistant PDX (p = 0.02). Conclusion: CDK4/6 inhibition appears as a promising strategy to manage advanced chordomas harboring a loss of CDKN2A/2B. However, further preclinical studies are strongly requested to confirm it and to understand acquired or de novo resistance to palbociclib, in the peculiar view of a targeting of the oxidative phosphorylation genes.

Management strategies in clival and craniovertebral junction chordomas: a 29-year experience.

OBJECTIVE: Chordomas represent one of the most challenging subsets of skull base and craniovertebral junction (CVJ) tumors to treat. Despite extensive resection followed by proton-beam radiation therapy, the recurrence rate remains high, highlighting the importance of developing efficient treatment strategies. In this study, the authors present their experience in treating clival and CVJ chordomas over a 29-year period. METHODS: The authors conducted a retrospective study of clival and CVJ chordomas that were surgically treated at their institution from 1991 to 2020. This study focuses on three aspects of the management of these tumors: the factors influencing the extent of resection (EOR), the predictors of survival, and the outcomes of the endoscopic endonasal approaches (EEAs) compared with open approaches (OAs). RESULTS: A total of 265 surgical procedures were performed in 210 patients, including 123 OAs (46.4%) and 142 EEAs (53.6%). Tumors that had an intradural extension (p = 0.03), brainstem contact (p = 0.005), cavernous sinus extension (p = 0.004), major artery encasement (p = 0.01), petrous apex extension (p = 0.003), or high volume (p = 0.0003) were significantly associated with a lower EOR. The 5-year progression-free survival (PFS) and overall survival (OS) rates were 52.1% and 75.1%, respectively. Gross-total resection and Ki-67 labeling index < 6% were considered to be independent prognostic factors of longer PFS (p = 0.0005 and p = 0.003, respectively) and OS (p = 0.02 and p = 0.03, respectively). Postoperative radiation therapy correlated independently with a longer PFS (p = 0.006). Previous surgical treatment was associated with a lower EOR (p = 0.01) and a higher rate of CSF leakage after EEAs (p = 0.02) but did not have significantly lower PFS and OS compared with primary surgery. Previously radiation therapy correlated with a worse outcome, with lower PFS and OS (p = 0.001 and p = 0.007, respectively). EEAs were more frequently used in patients with upper and middle clival tumors (p = 0.002 and p < 0.0001, respectively), had a better rate of EOR (p = 0.003), and had a lower risk of de novo neurological deficit (p < 0.0001) compared with OAs. The overall rate of postoperative CSF leakage after EEAs was 14.8%. CONCLUSIONS: This large study showed that gross-total resection should be attempted in a multidisciplinary skull base center before providing radiation therapy. EEAs should be considered as the gold-standard approach for upper/middle clival lesions based on the satisfactory surgical outcome, but OAs remain important tools for large complex chordomas.

Eyebrow incision with a crescent-shaped orbital rim craniotomy for microscopic and endoscopic transorbital approach to the anterior and middle cranial fossa: A cadaveric study and case presentation.

•The transorbital approach combining eyebrow incision and crescent-shaped craniotomy increases the surgical freedom to access the anterior and middle skull-base.•The technic allows the use of both endoscope and microscope.•The concept is at the crossroad between the supraorbital keyhole and endoscopic trans-orbital approach.

Lariboisiere Hospital pre-operative surgical checklist to improve safety during transpetrosal approaches.

BACKGROUND: Transpetrosal approaches are technically complex and require a complete understanding of surgical and radiological anatomy. A careful evaluation of pre-operative magnetic resonance imaging and computed tomography scan is mandatory, because anatomical or pathological variations are common and may increase the risk of complications related with the approach. METHODS: Pre-operative characteristics of venous and petrous bone anatomy were analysed and correlated with intraoperative findings, using injected magnetic resonance imaging and thin-slices computed tomography scan. These data regularly checked before each transpetrosal approach were progressively included in the presented checklist. RESULTS: Transpetrosal approaches have been used in 101 patients. Items included in the checklist were petrous bone pneumatization, angle between petrous apex and clivus, dehiscence of petrous carotid artery, dehiscence of geniculate ganglion, distance between superior semicircular canal and middle fossa floor, distance between cochlea and middle fossa floor, sigmoid sinus dominance, transverse sigmoid sinus junction depth to the outer cortical bone, jugular bulb height (high or low), location of the vein of Labbé, characteristics of superior petrosal vein complex. CONCLUSION: The presented checklist provides a systematic scheme of consultation of characteristic of venous and petrous bone anatomy for transpetrosal approaches. In our experience, the use of this checklist reduces the risk of complications related with approach, by minimizing the neglect of crucial information.

Risk of intracranial meningioma with three potent progestogens: A population-based case-control study.

BACKGROUND AND PURPOSE: A dose-dependent association between the use of cyproterone acetate (CPA) and intracranial meningioma has been identified but data for other potent progestogens are scarce. The association was assessed between intracranial meningioma surgery and exposure to three potent progestogens: CPA (≥25 mg/day), nomegestrol acetate (NOMAC) (3.75-5 mg/day) and chlormadinone acetate (CMA) (2-10 mg/day). METHODS: In this nationwide population-based case-control study, cases underwent surgery for intracranial meningioma in France from 2009 to 2018. They were matched to five control subjects for sex, year of birth and area of residence. Progestogen exposure was defined as progestogen use within the year before surgery for cases or the same date for their controls. RESULTS: In total, 25,216 cases were included (75% women, median age 58 years). Progestogen exposure was noted for 9.9% of cases (2497/25,216) and 1.9% (2382/126,080) of controls, with an odds ratio (OR) of 6.7 (95% confidence interval [CI] 6.3-7.1). The OR was 1.2 (1.0-1.4) for short-term use (<1 year) and 9.5 (8.8-10.2) for prolonged use. A strong association was identified for prolonged use of CPA (OR = 22.7, 95% CI 19.5-26.4), NOMAC (OR = 6.5, 95% CI 5.8-7.2) and CMA (OR = 4.7, 95% CI 4.5-5.3). Progestogen exposure increased the risk of meningioma for all histological grades and anatomical sites, particularly for the anterior and middle skull base: OR = 35.7 (95% CI 26.5-48.2) and 23.9 (95% CI 17.8-32.2) for CPA. The estimated number of attributable cases was 2124 (95% CI 2028-2220) (212/year). CONCLUSION: A strong association between prolonged exposure to potent progestogens and surgery for meningioma was observed. The risk increased from CMA to NOMAC to CPA. Individuals should be informed of this risk.

Dramatic In Vivo Efficacy of the EZH2-Inhibitor Tazemetostat in PBRM1-Mutated Human Chordoma Xenograft.

Chordomas are rare neoplasms characterized by a high recurrence rate and a poor long-term prognosis. Considering their chemo-/radio-resistance, alternative treatment strategies are strongly required, but their development is limited by the paucity of relevant preclinical models. Mutations affecting genes of the SWI/SNF complexes are frequently found in chordomas, suggesting a potential therapeutic effect of epigenetic regulators in this pathology. Twelve PDX models were established and characterized on histological and biomolecular features. Patients whose tumors were able to grow into mice had a statistically significant lower progression-free survival than those whose tumors did not grow after in vivo transplantation (p = 0.007). All PDXs maintained the same histopathological features as patients' tumors. Homozygous deletions of CDKN2A/2B (58.3%) and PBRM1 (25%) variants were the most common genomic alterations found. In the tazemetostat treated PDX model harboring a PBRM1 variant, an overall survival of 100% was observed. Our panel of chordoma PDXs represents a useful preclinical tool for both pharmacologic and biological assessments. The first demonstration of a high antitumor activity of tazemetostat in a PDX model harboring a PBRM1 variant supports further evaluation for EZH2-inhibitors in this subgroup of chordomas.

The mini-combined transpetrosal approach: an anatomical study and comparison with the combined transpetrosal approach.

BACKGROUND: The combined transpetrosal approach (CTPA) is a versatile technique suitable for challenging skull base pathologies. Despite the advantages provided by a wide surgical exposure, the soft tissue trauma, complex and time-consuming bony work, and cosmetic issues make it far from patient expectations. In this study, the authors describe a less invasive modification of the CTPA, the mini-combined transpetrosal approach (mini-CTPA), and perform a quantitative comparison between these two approaches. METHODS: Five human specimens were used for this study. CTPA was performed on one side and mini-CTPA on the opposite side. The surgical freedom, petroclival and brainstem area of exposure, and maneuverability for 6 anatomical targets, provided by the CTPA and mini-CTPA, were calculated and statistically compared. The bony volumes corresponding to each anterior petrosectomy were also measured and compared. Three clinical cases with an operative video are also reported to illustrate the effectiveness of the approach. RESULTS: The question-mark skin incision done along the muscle attachments permits an optimal cosmetic result. Even though the limited incision, the smaller craniotomy, and the less extensive bone drilling of mini-CTPA provide a smaller area of surgical freedom, the areas of exposure of petroclival region and brainstem were not statistically different between the two approaches. The antero-posterior maneuverability for the oculomotor foramen (OF), Meckel's cave (MC) and the REZ of trigeminal nerve, and the supero-inferior maneuverability for OF, MC, Dorello's canal, and REZ of CN VII are significantly reduced by the smaller opening. The bony volume of anterior petrosectomy resulted similar among the approaches. CONCLUSIONS: The mini-CTPA is an interesting alternative to the CTPA, providing comparable surgical exposure both for petroclival region and for brainstem. Although the lesser soft tissue dissection and bony opening decrease the surgical maneuverability, the mini-CTPA may reduce surgical time, potential approach-related morbidities, and improve cosmetic and functional outcomes for the patients.

A new simple and free tubular device for microscopic transcortical approach to deep-seated lesions: technical note and case example.

BACKGROUND: Surgery for deep-seated brain tumors remains challenging. Transcortical approaches often require brain retraction to ensure an adequate surgical corridor, thus possibly leading to brain damage. Various techniques have been developed to minimize brain retraction such as self-retaining retractors, endoscopic approaches, or tubular retractor systems. Even if they evenly distribute the mechanical pressure over the parenchyma, rigid retractors can also cause some degree of brain damage and have significant disadvantages. We propose here a soft cottonoid retractor for microscopic resection of deep-seated and ventricular lesions. METHODS: Through a small corticectomy, a channel route with a blunt cannula is developed until the lesion is reached. Then, a "balloon-like system" made with a surgical glove is progressively inflated, dilatating the surgical corridor. A mini-tubular device, handmade by suturing a surgical cottonoid, is positioned into the corridor, unfolded, and sutured to the edge of the dura, to prevent it from being progressively expelled from the working channel. This allows a good visualization of the lesion and surrounding structures under the microscope. RESULTS: Advantages of this technique are the softness of the tube walls, the absence of rigid arm to hold the tube, and the possibility for the tube to follow the movements of the instruments and to modify its orientation according to the working area. CONCLUSION: This simple and inexpensive tubular working channel for microscopic transcortical approach is a valuable alternative technique to traditional self-retaining retractor and rigid tube for the microsurgical resection of deep-seated brain tumors.

Atypical evolution of meningiomatosis after discontinuation of cyproterone acetate: clinical cases and histomolecular characterization.

PURPOSE: The long-term use of cyproterone acetate (CPA) is associated with an increased risk of developing intracranial meningiomas. CPA discontinuation most often induces a stabilization or regression of the tumor. The underlying biological mechanisms as well as the reasons why some meningiomas still grow after CPA discontinuation remain unknown. We reported a series of patients presenting CPA-induced meningiomatosis with opposed tumor evolutions following CPA discontinuation, highlighting the underlying histological and genetic features. METHODS: Patients presenting several meningiomas with opposite tumor evolution (coexistence of growing and shrinking tumors) following CPA discontinuation were identified. Clinical and radiological data were reviewed. A retrospective volumetric analysis of the meningiomas was performed. All the growing meningiomas were operated. Each operated tumor was characterized by histological and genetic analyses. RESULTS: Four women with multiple meningiomas and opposite tumor volume evolutions after CPA discontinuation were identified. Histopathological analysis characterized the convexity and tentorial tumors which continued to grow after CPA discontinuation as fibroblastic meningiomas. The decreasing skull base tumor was characterized as a fibroblastic meningioma with increased fibrosis and a widespread collagen formation. The two growing skull base meningiomas were identified as meningothelial and transitional meningiomas. The molecular characterization found two NF2 mutations among the growing meningiomas and a PIK3CA mutation in the skull base tumor which decreased. CONCLUSION: To our knowledge, this is the first report describing an atypical tumor evolution of CPA-associated meningiomas after CPA discontinuation. The underlying biological mechanisms explaining this observation and especially the close relationship between mutational landscapes and embryologic origins of the meninges in CPA-related meningiomas as well as their clonal origin require further research.

Intracranial Meningiomas Decrease in Volume on Magnetic Resonance Imaging After Discontinuing Progestin.

BACKGROUND: The behavior of meningiomas under influence of progestin therapy remains unclear. OBJECTIVE: To investigate the relationship between growth kinetics of intracranial meningiomas and usage of the progestin cyproterone acetate (PCA). METHODS: This study prospectively followed 108 women with 262 intracranial meningiomas and documented PCA use. A per-meningioma analysis was conducted. Changes in meningioma volumes over time, and meningioma growth velocities, were measured on magnetic resonance imaging (MRI) after stopping PCA treatment. RESULTS: Mean follow-up time was 30 (standard deviation [SD] 29) mo. Ten (4%) meningiomas were treated surgically at presentation. The other 252 meningiomas were followed after stopping PCA treatment. Overall, followed meningiomas decreased their volumes by 33% on average (SD 28%). A total of 188 (72%) meningiomas decreased, 51 (20%) meningiomas remained stable, and 13 (4%) increased in volume of which 3 (1%) were surgically treated because of radiological progression during follow-up after PCA withdrawal. In total, 239 of 262 (91%) meningiomas regressed or stabilized during follow-up. Subgroup analysis in 7 women with 19 meningiomas with follow-up before and after PCA withdrawal demonstrated that meningioma growth velocity changed statistically significantly (P = .02). Meningiomas grew (average velocity of 0.25 mm3/day) while patients were using PCA and shrank (average velocity of -0.54 mm3/day) after discontinuation of PCA. CONCLUSION: Ninety-one percent of intracranial meningiomas in female patients with long-term PCA use decrease or stabilize on MRI after stopping PCA treatment. Meningioma growth kinetics change significantly from growth during PCA usage to shrinkage after PCA withdrawal.

Tumor Growth Rate as a New Predictor of Progression-Free Survival After Chordoma Surgery.

BACKGROUND: Currently, different postoperative predictors of chordoma recurrence have been identified. Tumor growth rate (TGR) is an image-based calculation that provides quantitative information of tumor's volume changing over time and has been shown to predict progression-free survival (PFS) in other tumor types. OBJECTIVE: To explore the usefulness of TGR as a new preoperative radiological marker for chordoma recurrence. METHODS: A retrospective single-institution study was carried out including patients reflecting these criteria: confirmed diagnosis of chordoma on pathological analysis, no history of previous radiation, and at least 2 preoperative thin-slice magnetic resonance images available to measure TGR. TGR was calculated for all patients, showing the percentage change in tumor size over 1 mo. RESULTS: A total of 32 patients were retained for analysis. Patients with a TGR ≥ 10.12%/m had a statistically significantly lower mean PFS (P < .0001). TGR ≥ 10.12%/m (odds ratio = 26, P = .001) was observed more frequently in recurrent chordoma. In a subgroup analysis, we found that the association of Ki-67 labeling index ≥ 6% and TGR ≥ 10.12%/m was correlated with recurrence (P = .0008). CONCLUSION: TGR may be considered as a preoperative radiological indicator of tumor proliferation and seems to preoperatively identify more aggressive tumors with a higher tendency to recur. Our findings suggest that the therapeutic strategy and clinical-radiological follow-up of patients with chordoma can be adapted also according to this new parameter.