Endoscopic Endonasal Approach for Clival Chordomas in Elderly Patients: Clinical Characteristics, Patient Outcome, and Recurrence Rate.

Introduction The endoscopic endonasal route has demonstrated to be the approach of choice for a large majority of clival chordomas (CCs). However, its results in elderly patients are under-evaluated in the literature. The aim of this study is to assess the surgical outcome for these patients, determining the factors associated with a larger tumor resection in this population. Materials and Methods Our institutional database of CC has been retrospectively reviewed, to identify all cases over 65 years old, operated through an endoscopic endonasal approach (EEA). Preoperative clinical and radiological features were considered, as well as surgical results, morbidity, and patients' outcome at follow-up. Results Out of our series of 143 endoscopic surgical procedures for CC, 34 (23.8%) were in patients older than 65 and 10 in older than 75 (7.0%). Gross tumor removal was achieved in 22 cases (64.7%). Complications consisted of 2 (5.9%) postoperative cerebrospinal leaks, 1 (2.9%) meningitis, 1 (2.9%) permanent cranial nerve VI palsy, 1 (2.9%) pneumonia, and 2 (5.9%) urinary infections. In 39.1% of cases, the preoperative ophthalmoplegia improved or resolved. Twenty-seven patients (79.4%) underwent radiation therapy. At follow-up (37.7 ± 44.9 months), 13 patients (38.2%) showed a recurrence/progression and 13 (38.3%) deceased. Conclusion EEA can be a useful approach in elderlies, balancing the large tumor removal with an acceptable morbidity rate, even if higher than that for general CC population. However, patient selection remains crucial. A multidisciplinary evaluation is important to assess not only their medical conditions, but also their social and familiar conditions.

Not Every Size Fits All: Surgical Corridors for Clival and Cervical Chordomas-A Systematic Review of the Literature and Illustrative Cases.

Introduction. Clival chordomas represent a rare but clinically significant subset of skull base tumors, characterized by a locally aggressive nature and a location in proximity to vital neurovascular structures. Surgical resection, often combined with adjuvant therapies, remains the cornerstone of clival chordoma treatment, and various approaches and techniques have evolved to maximize tumor removal while preserving neurological function. Recent advancements in skull base surgery, imaging, and adjuvant therapies have improved outcomes by reducing morbidity and thus enhancing long-term survival. Methods and Results. We have conducted a systematic review on PubMed/Medline following PRISMA guidelines regarding indications, the extent of resection (EOR), and complication rates. Then, we present three illustrative cases from our personal experience, which started 25 years ago with CVJ instrumentation procedures and 15 years ago with anterior decompressive transmucosal procedures performed with the aid of an operative microscope, an endoscope, and neuroradiological monitoring. Conclusions. Traditionally, the transoral approach (TOA) is the most frequently used corridor for accessing the lower clivus and the anterior craniovertebral junction (CVJ), without the need to mobilize or retract neural structures; however, it is associated with a high rate of complications. The endonasal approach (EEA) provides access to the anterior CVJ as well as to the lower, middle, and superior clivus, decreasing airway and swallowing morbidity, preserving palatal function, decreasing postoperative pain, and reducing the incidence of tracheostomy. The submandibular retropharyngeal approach (SRA) allows unique access to certain cervical chordomas, which is better suited when the lesion is located below the clivus and in the midline.

Degenerative Atypia in Clival Chordoma: Two Case Reports.

In this study, we report surgical management combined with radiotherapy in two patients with typical chordoma. Different types of radiation have varied effects on chordomas when they are radiated. Classical cases display cellular atypia and fibrosis following irradiation, while necrosis and fibrosclerosis are observed after carbon ion therapy, implying that it is possible to control the tumor more effectively using carbon ion therapy with minimal side effects.

Impact of proton versus photon adjuvant radiotherapy on overall survival in the management of skull base and spinal chordomas: a National Cancer Database analysis.

OBJECTIVE: Chordomas are rare tumors that originate from undifferentiated remnants of the notochord. Currently, there are no established guidelines regarding the choice of adjuvant radiation modality for patients surgically treated for chordomas. Using a nationwide, multicenter database, the authors aimed to compare long-term survival outcomes associated with the use of proton or photon adjuvant therapy for the management of chordomas of skull base and spine. METHODS: The National Cancer Database (NCDB) was queried for chordoma cases from 2004 to 2017. Patient, tumor, and treatment characteristics were extracted from the database. The primary outcome was overall survival (OS). Kaplan-Meier survival analyses were conducted to investigate differences in outcome on propensity score-matched cohorts of patients treated with proton or photon adjuvant radiotherapy. RESULTS: Of the 3490 patients available, 424 met the inclusion criteria for this study. In the prematching analysis, patients receiving adjuvant photon therapy were significantly older (median age 57.0 vs 45.0 years, p < 0.001) and were more commonly male (61% vs 43%, p < 0.001) compared with those receiving proton therapy. Races were equally distributed among radiotherapy modalities (p = 0.64). Patients with chordomas of the mobile spine or sacrum were less likely to receive proton compared with photon therapy (37% vs 58%). Patients receiving proton therapy were more often represented among private insurance holders (69% vs 52%, p < 0.001) as well as in the highest income quartile (52% vs 40%, p = 0.008). Patients traveled farther to receive proton, as opposed to photon, therapy (median 59.0 vs 34.9 miles, p < 0.001). On postmatching Kaplan-Meier analysis encompassing all chordoma cases, no difference in OS between photon and proton therapy was revealed (HR 0.75, 95% CI 0.39-1.44; p = 0.39). A Kaplan-Meier analysis only including patients with skull base chordomas reached similar results (HR 0.83, 95% CI 0.31-2.22; p = 0.71). In patients with spine chordomas, however, a significant difference was found, as proton therapy exhibited a superior OS over photon therapy (HR 0.28, 95% CI 0.09-0.81; p = 0.012). CONCLUSIONS: Based on this nationwide analysis, patients with private insurance and higher income were more likely to receive proton adjuvant radiotherapy, while those with spinal or sacral chordomas were less likely to receive this modality. Despite this disparity, an OS benefit was observed in patients with chordomas of the spine and sacrum who received adjuvant proton therapy, in comparison with a matched cohort of patients treated with photon therapy. Conversely, this advantageous outcome was not evident in cases of chordomas located at the skull base.

Balancing benefits and limitations of linear energy transfer optimization in carbon ion radiotherapy for large sacral chordomas.

Background and Purpose: A low linear energy transfer (LET) in the target can reduce the effectiveness of carbon ion radiotherapy (CIRT). This study aimed at exploring benefits and limitations of LET optimization for large sacral chordomas (SC) undergoing CIRT. Materials and Methods: Seventeen cases were used to tune LET-based optimization, and seven to independently test interfraction plan robustness. For each patient, a reference plan was optimized on biologically-weighted dose cost functions. For the first group, 7 LET-optimized plans were obtained by increasing the gross tumor volume (GTV) minimum LETd (minLETd) in the range 37-55 keV/µm, in steps of 3 keV/µm. The optimal LET-optimized plan (LETOPT) was the one maximizing LETd, while adhering to clinical acceptability criteria. Reference and LETOPT plans were compared through dose and LETd metrics (D x , L x to x% volume) for the GTV, clinical target volume (CTV), and organs at risk (OARs). The 7 held-out cases were optimized setting minLETd to the average GTV L98% of the investigation cohort. Both reference and LETOPT plans were recalculated on re-evaluation CTs and compared. Results: GTV L98% increased from (31.8 ± 2.5)keV/µm to (47.6 ± 3.1)keV/µm on the LETOPT plans, while the fraction of GTV receiving over 50 keV/µm increased on average by 36% (p < 0.001), without affecting target coverage goals, or impacting LETd and dose to OARs. The interfraction analysis showed no significant worsening with minLETd set to 48 keV/µm. Conclusion: LETd optimization for large SC could boost the LETd in the GTV without significantly compromising plan quality, potentially improving the therapeutic effects of CIRT for large radioresistant tumors.

Extramedullary Intracranial Plasmacytomas: A Systematic Review of Literature.

Extramedullary plasmacytoma (EMP) is an uncommon disorder characterized by the development of abnormal plasma cell tumors outside the bone marrow. These tumors are typically observed in various locations, including the upper respiratory tract, gastrointestinal tract, and other soft tissues. Among the less explored manifestations of EMP is intracranial EMP, which remains poorly understood due to the limited literature available on the subject. The objective was to comprehend the population characteristics, localization, type, treatment, and outcomes of intracranial EMP. A systematic review of the literature for EMPs was conducted following the Preferred Reporting Items for Systematic Reviews and Meta-Analysis guidelines. The strategy "extramedullary plasmacytoma AND multiple myeloma" was used for the search. The search terms were queried using PubMed, Embase, Scopus, Cochrane, and Web of Science databases. We included only those studies that presented clinical studies with patients diagnosed with intracranial plasmacytomas. In this study, a total of 84 patients from 25 studies were analyzed. The average age of diagnosis was 57.25 years, with a slightly higher proportion of females (57%) compared to male patients (43%). The most common locations of intracranial plasmacytomas were the clivus (29.7%), frontal lobe (18.9%), parietal lobe (8.1%), occipital lobe (6.7%), temporal lobe (6.7%), and sphenoid (4%). Chordoma and meningioma were the most common differential diagnoses encountered during clinical investigations. Treatment modalities included radiotherapy (RT), chemotherapy (QT), surgical resection (SR), and conservative approaches. The most frequent treatment combinations were SR + RT (19%) and RT only (17.8%). Mortality was reported in 48% of the cases, with complete resolution observed in 10 cases and partial resolution in 3 cases. The average follow-up duration was 37.5 months. The clivus is the most frequently reported site of extramedullary intracranial plasmacytoma (EMIP) occurrence, representing 29.7% of cases. Chordomas were commonly observed alongside EMIPs and emerged as the primary differential diagnosis. RT was the predominant treatment modality, with SR considered when feasible. RT alone demonstrated the highest effectiveness in managing EMIPs (30%), while QT as a sole intervention showed lower efficacy. However, a combination of dexamethasone, lenalidomide, and targeted RT displayed promising results, offering improved tumor response and increased safety.

Controversies in neuro-oncology: Focal proton versus photon radiation therapy for adult brain tumors.

Radiation therapy (RT) plays a fundamental role in the treatment of malignant and benign brain tumors. Current state-of-the-art photon- and proton-based RT combines more conformal dose distribution of target volumes and accurate dose delivery while limiting the adverse radiation effects. PubMed was systematically searched from from 2000 to October 2023 to identify studies reporting outcomes related to treatment of central nervous system (CNS)/skull base tumors with PT in adults. Several studies have demonstrated that proton therapy (PT) provides a reduced dose to healthy brain parenchyma compared with photon-based (xRT) radiation techniques. However, whether dosimetric advantages translate into superior clinical outcomes for different adult brain tumors remains an open question. This review aims at critically reviewing the recent studies on PT in adult patients with brain tumors, including glioma, meningiomas, and chordomas, to explore its potential benefits compared with xRT.

A dosiomics approach to treatment outcome modeling in carbon ion radiotherapy for skull base chordomas.

PURPOSE: To investigate the role of dosiomics features extracted from physical dose (DPHYS), RBE-weighted dose (DRBE) and dose-averaged Linear Energy Transfer (LETd), to predict the risk of local recurrence (LR) in skull base chordoma (SBC) treated with Carbon Ion Radiotherapy (CIRT). Thus, define and evaluate dosiomics-driven tumor control probability (TCP) models. MATERIALS AND METHODS: 54 SBC patients were retrospectively selected for this study. A regularized Cox proportional hazard model (r-Cox) and Survival Support Vector Machine (s-SVM) were tuned within a repeated Cross Validation (CV) and patients were stratified in low/high risk of LR. Models' performance was evaluated through Harrell's concordance statistic (C-index), and survival was represented through Kaplan-Meier (KM) curves. A multivariable logistic regression was fit to the selected feature sets to generate a dosiomics-driven TCP model for each map. These were compared to a reference model built with clinical parameters in terms of f-score and accuracy. RESULTS: The LETd maps reached a test C-index of 0.750 and 0.786 with r-Cox and s-SVM, and significantly separated KM curves. DPHYS maps and clinical parameters showed promising CV outcomes with C-index above 0.8, despite a poorer performance on the test set and patients stratification. The LETd-based TCP showed a significatively higher f-score (0.67[0.52-0.70], median[IQR]) compared to the clinical model (0.4[0.32-0.63], p < 0.025), while DPHYS achieved a significatively higher accuracy (DPHYS: 0.73[0.65-0.79], Clinical: 0.6 [0.52-0.72]). CONCLUSION: This analysis supports the role of LETd as relevant source of prognostic factors for LR in SBC treated with CIRT. This is reflected in the TCP modeling, where LETd and DPHYS showed an improved performance with respect to clinical models.

A Bibliometric Analysis of the 20 Most Cited Articles on Sacrococcygeal Chordomas.

This study aims to summarize sacrococcygeal chordoma literature through bibliometric analysis and to offer insights into key studies to guide clinical practices and future research. The Web of Science database was searched using the terms "sacral chordoma", "chordomas of the sacrum", "chordomas of the sacral spine", "chordomas of the sacrococcygeal region", "coccygeal chordoma", and "coccyx chordoma". Articles were analyzed for citation count, authorship, publication date, journal, research area tags, impact factor, and evidence level. The median number of citations was 75 (range: 53-306). The primary publication venue was the International Journal of Radiation Oncology, Biology, Physics. Most works, published between 1999 and 2019, featured a median journal impact factor of 3.8 (range: 2.1-7) and predominantly fell under the research area tag, radiation, nuclear medicine, and imaging. Of these articles, 19 provided clinical data with predominantly level III evidence, and one was a literature review. This review highlights the increasing volume of sacrococcygeal chordoma publications over the past two decades, indicating evolving treatment methods and interdisciplinary patient care. Advances in radiation, particularly intensity-modulated radiation therapy (IMRT) and proton beam therapy, are believed to be propelling research growth, and the lack of level I evidence underscores the need for more rigorous studies to refine treatment protocols for sacrococcygeal chordomas.

KIF15 promotes the development and progression of chordoma via activating PI3K-AKT signalling pathway.

Aims: Despite its implication in various human cancers, the expression and functional significance of Kinesin family member 15 (KIF15) in chordomas remain unexplored. Main methods: The evaluation of KIF15 protein levels was conducted through immunohistochemistry (IHC) staining and Western blot analysis. Cell proliferation was quantified using MTT and CCK8 assays, whereas cell migration was examined using wound healing and Transwell assays. Furthermore, flow cytometric analysis was utilized to assess cell apoptosis and the cell cycle. Additionally, in vivo experiments were performed using a mouse xenograft model. Key findings: Our study revealed significantly higher expression of KIF15 in stage III chordoma tissues compared to stage II tissues. Knockdown of KIF15 led to notable inhibition of cell proliferation and migration, along with enhanced apoptosis and cell cycle arrest. In vivo studies further confirmed the inhibitory effects of KIF15 knockdown on chordoma tumour growth. In terms of mechanism, we identified the involvement of the PI3K-AKT signalling pathway mediated by KIF15 in chordomas. Notably, the anti-tumour effects of KIF15 deficiency on chordomas were partially reversed by the addition of an AKT activator. Significance: KIF15 promotes chordoma development and progression through the activation of the PI3K-AKT signalling pathway. Thus, targeting KIF15 might be a promising therapeutic strategy for treating chordomas.

Sacral-Nerve-Sparing Planning Strategy in Pelvic Sarcomas/Chordomas Treated with Carbon-Ion Radiotherapy.

To minimize radiation-induced lumbosacral neuropathy (RILSN), we employed sacral-nerve-sparing optimized carbon-ion therapy strategy (SNSo-CIRT) in treating 35 patients with pelvic sarcomas/chordomas. Plans were optimized using Local Effect Model-I (LEM-I), prescribed DRBE|LEM-I|D50% (median dose to HD-PTV) = 73.6 (70.4-76.8) Gy (RBE)/16 fractions. Sacral nerves were contoured between L5-S3 levels. DRBE|LEM-I to 5% of sacral nerves-to-spare (outside HD-CTV) (DRBE|LEM-I|D5%) were restricted to <69 Gy (RBE). The median follow-up was 25 months (range of 2-53). Three patients (9%) developed late RILSN (≥G3) after an average period of 8 months post-CIRT. The RILSN-free survival at 2 years was 91% (CI, 81-100). With SNSo-CIRT, DRBE|LEM-I|D5% for sacral nerves-to-spare = 66.9 ± 1.9 Gy (RBE), maintaining DRBE|LEM-I to 98% of HD-CTV (DRBE|LEM-I|D98%) = 70 ± 3.6 Gy (RBE). Two-year OS and LC were 100% and 93% (CI, 84-100), respectively. LETd and DRBE with modified-microdosimetric kinetic model (mMKM) were recomputed retrospectively. DRBE|LEM-I and DRBE|mMKM were similar, but DRBE-filtered-LETd was higher in sacral nerves-to-spare in patients with RILSN than those without. At DRBE|LEM-I cutoff = 64 Gy (RBE), 2-year RILSN-free survival was 100% in patients with <12% of sacral nerves-to-spare voxels receiving LETd > 55 keV/µm than 75% (CI, 54-100) in those with ≥12% of voxels (p < 0.05). DRBE-filtered-LETd holds promise for the SNSo-CIRT strategy but requires longer follow-up for validation.

Unfolding the Art of Methodical Approach for Total Sacrectomy.

BACKGROUND: Total sacrectomy is a technically demanding surgery with substantial risks, including high morbidity and mortality due to the likelihood of exsanguination.1-3 Despite the evolution of surgical techniques,4,5 the incidence of postoperative complications remains significant.1 This study presents a systematic approach to total sacrectomy, with a particular focus on a modified technique for isolating the iliac vessels, aimed at effective management of complex sacrococcygeal masses and the reduction of operative complications. PATIENTS AND METHODS: Employing our approach, a 45-year-old male patient presenting with a sacrococcygeal mass involving the lower S1 bone and sacroiliac joint underwent total sacrectomy. A meticulous preoperative workup, including magnetic resonance imaging (MRI), was followed by precise surgical steps: sigmoid colon and rectal mobilization, isolation of the iliac vessels,2,6 lumbosacral nerve trunk preservation, and strategic anterior and posterior osteotomies. The procedure concluded with reconstruction using mesorectal fat and bilateral gluteus maximus flaps.5-7 RESULTS: The patient's operation was conducted successfully without any perioperative complications, culminating in a chordoma resection with clear margins. Postoperative recovery was swift, allowing for discharge on the seventh day. CONCLUSIONS: The application of our systematic sacrectomy method, with particular emphasis on the isolation of the external iliac veins, significantly minimized intraoperative bleeding risks and other perioperative complications. Our technique offers a reproducible and effective strategy for the surgical management of sacrococcygeal masses.

Planning Strategy to Optimize the Dose-Averaged LET Distribution in Large Pelvic Sarcomas/Chordomas Treated with Carbon-Ion Radiotherapy.

To improve outcomes in large sarcomas/chordomas treated with CIRT, there has been recent interest in LET optimization. We evaluated 22 pelvic sarcoma/chordoma patients treated with CIRT [large: HD-CTV ≥ 250 cm3 (n = 9), small: HD-CTV < 250 cm3 (n = 13)], DRBE|LEM-I = 73.6 (70.4-73.6) Gy (RBE)/16 fractions, using the local effect model-I (LEM-I) optimization and modified-microdosimetric kinetic model (mMKM) recomputation. We observed that to improve high-LETd distribution in large tumors, at least 27 cm3 (low-LETd region) of HD-CTV should receive LETd of ≥33 keV/µm (p < 0.05). Hence, LETd optimization using 'distal patching' was explored in a treatment planning setting (not implemented clinically yet). Distal-patching structures were created to stop beams 1-2 cm beyond the HD-PTV-midplane. These plans were reoptimized and DRBE|LEM-I, DRBE|mMKM, and LETd were recomputed. Distal patching increased (a) LETd50% in HD-CTV (from 38 ± 3.4 keV/µm to 47 ± 8.1 keV/µm), (b) LETdmin in low-LETd regions of the HD-CTV (from 32 ± 2.3 keV/µm to 36.2 ± 3.6 keV/µm), (c) the GTV fraction receiving LETd of ≥50 keV/µm, (from <10% to >50%) and (d) the high-LETd component in the central region of the GTV, without significant compromise in DRBE distribution. However, distal patching is sensitive to setup/range uncertainties, and efforts to ascertain robustness are underway, before routine clinical implementation.

Treatment of Clival Chordomas: A 20-Year Experience and Systematic Literature Review.

Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research. A total of 42 patients underwent 85 surgeries; median follow-up was 15.8 years, overall survival rate was 49.9% at 10 years; meanwhile, progression-free survival was 26.6% at 10 years. A significantly improved survival was observed in younger patients (<50 years), in tumors with Ki67 ≤ 5% and when adjuvant radiotherapy was performed. To conclude, clival chordomas are aggressive tumors in which surgery and radiotherapy play a fundamental role while molecular targeted drugs still have an ancillary position. Recognizing risk factors for recurrence and performing a molecular characterization of more aggressive lesions may be the key to future effective treatment.

Anatomical Considerations and Plastic Surgery Reconstruction Options of Sacral Chordoma Resection.

Introduction Chordomas are slow-growing malignant bone tumors arising from remnant embryonic notochord cells with predilection for the sacrum. They rarely metastasize, and early surgical resection with clear margins is the treatment of choice followed by plastic surgery reconstruction supplemented with adjuvant radiotherapy based on the local treatment protocol or in cases with a contaminated surgical field. Aim The aim of the present study is to present our experience in surgical management of sacral chordomas and propose a surgical reconstruction algorithm considering anatomical parameters after partial or total sacrectomy. Materials and methods  Twenty-seven patients with sacral chordomas were treated in our Orthopaedic Surgery Department between January 1997 and September 2022, and 10 of them had plastic surgery reconstruction. Patients were divided into groups based on the type of sacrectomy, sacrum anatomical vascular or neural variations, partial or total, and the type of soft tissue reconstruction. The postoperative complications and the functional outcomes in each patient were assessed. Results  Bilateral gluteal advancement flaps or gluteal perforator flaps are the first choice in patients with partial sacrectomy, intact gluteal vessels, and without preoperative radiotherapy followed by transpelvic vertical rectus abdominis myocutaneous flap or free flaps in those patients with near total sacrectomy and preoperative radiation therapy. Conclusion  There are four reliable options for patients after sacral chordoma resection: direct closure, bilateral gluteal advancement flaps, transpelvic vertical rectus abdominis myocutaneous flaps, and free flaps. Each time, tumor-free margins and a good reconstructive plan according to the defect and patient characteristics are mandatory.

The critical roles of lnc-GLYATL2-2/PD-L1 axis in immune microenvironment and the clinical value of intracranial chordomas.

Intracranial chordomas (ICs) are associated with a poor prognosis due to low total resection rates and high recurrence rates. However, the role of immunotherapy in ICs remains unknown. RNA sequencing and immunohistochemical staining were performed on IC tissues and normal tissues, and the long noncoding RNA (lncRNA) lnc-GLYATL2-2 was identified. The results indicated that high expression of lnc-GLYATL2-2 was positively correlated with the tumor-infiltrating lymphocyte (TIL) markers CD4 and Foxp3, negatively correlated with CD8, and positively correlated with the expression of the immune checkpoint molecules programmed death receptor-1 (PD-1) and programmed death ligand 1 (PD-L1). Additionally, Kaplan-Meier and univariate or multivariate Cox regression analyses revealed the predictive value of lnc-GLYATL2-2 for survival based on clinical data from patients with ICs. A high expression level of lnc-GLYATL2-2 is potentially correlated with a suppressive tumor immune microenvironment and adverse clinical outcomes in IC patients. Mechanistically, the upregulation of lnc-GLYATL2-2 can result in increased cytoplasmic levels of ELAVL1, leading to enhanced binding to the 3'-UTR of PD-L1 mRNA and maintenance of its stability. In contrast, lnc-GLYATL2-2 can directly interact with the PD-L1 protein to prevent degradation, thereby promoting high levels of PD-L1 expression simultaneously at the transcriptional and translational levels in chordoma cells. These results provide a new perspective on the diagnosis and prognosis of ICs and provide theoretical evidence for immunotherapy in patients with ICs.

Lumbopelvic shortening and local host bone-to-host bone reconstruction: a surgical method for lumbopelvic fusion following total sacrectomy.

PURPOSE: Primary sacral tumors are rare, representing fewer than 7% of spinal neoplasms. Following total sacrectomy, lumbopelvic instrumentation and fusion carries a high risk of non-union with no current consensus on fixation techniques to augment bony defects. We aim to describe the outcome of a reconstruction technique following total sacrectomy whereby lumbopelvic shortening is performed and the posterior pelvic ring is compressed to enable contact with the native L5 vertebra. METHODS: Retrospective chart review of 2 patients with 2 and 7 years post-operative follow-up. A review of hospital records including clinical assessments, complications, pathology and imaging reports. RESULTS: Patient 1 was a 17-years-old male with recurrent sacral chondrosarcoma, who presented with lumbosacral neuropathic pain and radiculopathy after failed intralesional surgery. Patient 2 was a 51-years-old male with chronic low back pain caused by a large low-grade chondroid sacral chordoma. Reconstruction technique involved mobilizing the L5 vertebra into the pelvis and pelvic ring closure to obtain host-bone-to-bone contact, eliminating the need for alternative grafts. Post-operative complications included superficial abdominal wound drainage, lower limb DVT, pulmonary emboli and deep pelvic infection. Serial CT scans demonstrated bony fusion in both patients. Neither patients had evidence of tumor recurrence and were able to ambulate at recent follow-up. Imaging demonstrated changed acetabular version of - 4.6/- 8.1 and - 14.4/- 14.8 (patient 1/2, R/L, respectively). CONCLUSION: Primary lumbopelvic shortening represents an alternative local autograft reconstructive technique for management of large sacral defects following total sacrectomy. This technique obviates the additional morbidity and surgical cost associated with the use of previously described techniques.

[Chordoma-An update]. / Chordome ­ Ein Update.

Chordomas are rare malignant tumors of the axial skeleton with notochordal differentiation. From a morphological point of view, chordomas display a broad spectrum ranging from the classical, conventional form not otherwise specified (NOS) to forms with hepatoid or renal carcinoma-like differentiation or even poorly or dedifferentiated variants. The detection of brachyury is highly characteristic, though not exclusive. The morphological differential diagnosis from a benign notochordal tumor (BNCT) requires integration of imaging since BNCT is limited to the vertebral bodies and is not osteolytic. Targeted therapy is a current research focus and cell lines as in vitro models are a precondition for the establishment and validation of this approach.

Multimodal Intraoperative Image-Driven Surgery for Skull Base Chordomas and Chondrosarcomas.

Given the difficulty and importance of achieving maximal resection in chordomas and chondrosarcomas, all available tools offered by modern neurosurgery are to be deployed for planning and resection of these complex lesions. As demonstrated by the review of our series of skull base chordoma and chondrosarcoma resections in the Advanced Multimodality Image-Guided Operating (AMIGO) suite, as well as by the recently published literature, we describe the use of advanced multimodality intraoperative imaging and neuronavigation as pivotal to successful radical resection of these skull base lesions while preventing and managing eventual complications.