Clinical characteristics and predisposing factors of lung metastasis in sacral chordoma: a cross-sectional cohort study of 221 cases.

Introduction: Limited studies are available on the topic of lung metastasis in sacral chordoma. The primary objective of this study was to investigate the prevalence, characteristics, associated factors, and prognosis of lung metastasis in sacral chordoma. Methods: A total of 221 cases with primary sacral chordoma, all of whom underwent surgical resection at our center, were included in this study. Comprehensive demographic information, imaging findings, and oncological evaluations were collected and thoroughly analyzed. The diagnosis of lung metastasis in the majority of cases was established through radiographic examinations. Results: The prevalence of lung metastasis in the cohort was 19.5%, with the lung emerging as the predominant site of distant metastasis. Recurrent chordoma cases exhibited a significantly higher lung metastasis rate in comparison to newly diagnosed chordoma cases (33.33% and 12.76%, p=0.0005). Patients with lung metastasis had a larger tumor size, a higher proportion of previous sacral chordoma surgeries and a greater likelihood of postoperative recurrence. Associated factors of lung metastasis were tumor size, postoperative recurrence and radiotherapy. Patients with lung metastasis exhibited decreased median overall survival (91 vs. 144 months for those without lung metastasis, p<0.05) and recurrence-free survival (27 vs. 68 months, p<0.001) times. Discussion: Lung is the most common site of distant metastasis in sacral chordoma with an incidence rate nearly 20%. Larger tumor size and postoperative recurrence are risk factors for lung metastasis while radiotherapy is a protective factor. Occurrence of lung metastasis in sacral chordoma is a negative prognostic factor.

How to Prevent Local Recurrence of Sacral Chordoma Treated with Carbon-Ion Radiotherapy: An Analysis of the Risk Factors of Local Failure and an Adequate Disease Margin.

INTRODUCTION: Recent reports have described the usefulness of carbon ion radiotherapy (CIRT) for inoperable sacral chordomas. However, its long-term local control rate needs to be improved. The present study identified the risk factors that affect the local relapse of sacral chordomas and the appropriate margins from the tumors. METHODS: Forty-nine patients with sacral chordoma treated with CIRT between 2011 and 2022 were retrospectively analyzed. Factors predicting the risk of local recurrence were evaluated, including age, sex, tumor size, muscle invaded with tumor, and surgery before CIRT. To determine the appropriate margin, the distance between the clinical target volume (CTV) and the out-field recurrent lesions was analyzed. RESULTS: The patients included 37 males and 12 females with a mean age of 67.1 years. A multivariate analysis showed that a tumor size >8 cm and invasion into the gluteus maximus muscle were significant risk factors with hazard ratios of 5.56 and 15.20 (p = 0.02 and 0.01), respectively. Out-field recurrence occurred in 13 cases, with 6, 3, and 4 relapses occurring in the muscle, bone, and both, respectively. The tumor occurred within 20 mm from the CTV in 60% of relapses in the muscles. CONCLUSION: The current study presented novel findings on CIRT for sacral chordomas, although there were several limitations, such as a short follow-up period to investigate slow-growth tumors and a small number of tumor specimens owing to inoperative cases. A tumor size >8 cm and invasion into the gluteus maximus muscle were shown to be risk factors for recurrence in the treatment of sacral chordoma with CIRT. Our findings further suggest that an additional 2-cm margin from the CTV in the muscle fiber direction is recommended during CIRT.

Two-stage surgery for large sacrococcygeal chordomas: How I do it.

Sacrococcygeal chordoma is a malignant, slow-growing, and locally aggressive bone tumor. A wide surgical margin is recommended to prevent local recurrence and metastasis. This disease tends to cause massive defects when rectal resection and sacrectomy are required. Therefore, soft tissue reconstruction is required and a pedicled vertical rectus abdominis muscle flap (VRAM) is a viable option. Important anatomical landmarks, advantages and limitations are discussed and the procedure is described step by step. This case report presents a two-stage operation with an anterior rectal resection and VRAM flap harvest followed by a complementary posterior approach with sacrectomy and soft tissue reconstruction: approach and results. The wound completely healed in six weeks. Three years after surgery, no local recurrence or distal metastasis was detected. This two-stage strategy presents a viable and safe option for large sacrococcygeal chordomas.

Chordoma: Genetics and Contemporary Management.

Chordomas, arising from notochord remnants, are rare neoplasms with aggressive growth patterns despite their histologically low-grade nature. This review explores their embryological origins, molecular markers like brachyury, and genetic alterations driving pathogenesis. Diagnosis relies on advanced imaging and biopsy confirmation due to overlapping features with chondrosarcoma. The WHO classification distinguishes conventional, dedifferentiated, and poorly differentiated chordomas, each with distinct prognostic implications. Recent genomic analyses uncovered recurrent mutations in PI3K signaling pathways and chromatin remodeling genes, informing prognostic models. Surgery remains the cornerstone of treatment, though adjuvant radiation complements surgical resection. Although chordomas are generally considered refractory to medical therapy, emerging targeted molecular strategies show potential promise in ongoing trials. This review aims to provide a concise yet comprehensive overview of chordomas, guiding clinicians in diagnosis, treatment, and prognostication for improved patient outcomes.

Challenges of enbloc mid-sacrectomy as redo surgery for sacral chordoma: a case report.

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.

Dermoscopy of hypopigmented macules unveiling genetic diagnosis of tuberous sclerosis complex type 2 in an infant presenting with sacral chordoma.

A 2-month-old male with surgically resected sacral chordoma presented with multiple hypopigmented macules showing characteristic patchy, sharply demarcated areas of pigment network on dermoscopy. These dermoscopic findings were suggestive of the ash-leaf macules of tuberous sclerosis over other common hypopigmented macules in neonates. Chordomas presenting in early childhood in the sacral location have been reported as a rare manifestation of tuberous sclerosis complex. The combination of these findings led to a diagnosis of tuberous sclerosis, confirmed with the finding of a heterozygous TSC2 gene deletion; treatment with sirolimus resulted in regression of cardiac rhabdomyomas and hypopigmented macules.

Sacral Chordoma Presenting as Back Pain in the Chiropractic Clinic: A Case Report.

Cases of lumbar and gluteal pain are commonly encountered in chiropractic clinics, with a broad differential diagnosis primarily centered on musculoskeletal conditions. This report presents the second documented case of sacral chordoma diagnosed at a chiropractic clinic and emphasizes the importance of considering alternative diagnoses and interdisciplinary collaboration in patient care. A 42-year-old man presented to a chiropractic clinic with complaints of lumbar and gluteal pain. The initial conservative management based on a presumptive musculoskeletal diagnosis was ineffective. Suspicion of an alternative etiology prompted a referral for imaging, which revealed a sacral chordoma. An interdisciplinary collaboration involving orthopedic surgeons, oncologists, radiologists, and other healthcare professionals was initiated to optimize the treatment outcomes of this rare and aggressive tumor. This case report underscores the importance of maintaining a high index of suspicion in cases of musculoskeletal presentations in chiropractic clinics and the critical role of advanced imaging in establishing a definitive diagnosis. Interdisciplinary collaboration is essential for managing complex conditions such as sacral chordomas, ensuring the delivery of the highest quality of care, and optimizing patient outcomes. Chiropractors play a crucial role in identifying, referring, and contributing to the management of patients with complex presentations as part of a comprehensive multidisciplinary treatment plan.

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

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.

Sacral chordoma: an unusual cause of neurogenic bladder - a case report.

Sacral chordoma is a rare tumor arising from notochord remnants. Sacral pain is the most common sign. The authors report the case of a sacral chordoma diagnosed in a urology department. It was a rare cause of erectile dysfunction and it was unusually revealed by lower urinary tract symptoms (LUTSs). Case Presentation: A 67-year-old man had severe dysuria associated with erectile dysfunction. On digital rectal examination, we palpated a mass protruding through the posterior wall of the rectum. The gluteal region is infiltrated by a firm mass. MRI revealed an osteolytic sacral tumor. A percutaneous biopsy of the tumor was performed. A pathological examination confirmed the diagnosis of sacral chordoma. The patient had palliative radiotherapy. One year after radiotherapy, he had rectal stenosis associated with severe constipation. Clinical Discussion: Sacral chordoma is a rare malignant tumor. Sacralgia is the most common sign. LUTS and erectile dysfunction were rarely reported in patients affected by this bone tumor. MRI is very suggestive of this pathology. Diagnostic confirmation requires pathological examination. The curative management consists of a large resection of the tumor. For inextricable tumors, palliative radiotherapy can be carried out. The prognosis of sacral chordoma is defined by a high recurrence potential and a low metastatic risk. Conclusions: Sacral chordoma is a rare bone tumor. It is an unusual cause for LUTS and erectile dysfunction. Complete tumor excision is the only curative treatment. Recurrence after treatment is the main challenge for surgeons.

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.

Successful mechanical thrombectomy and stent exclusion of sacral chordoma tumor thrombus.

A 77-year-old man with history of sacral chordoma and pulmonary embolism presented to the emergency room with a 1-day history of diffuse left flank and lower extremity swelling. The patient was found to have thrombus in the left common and external iliac veins. The patient was brought to Interventional Radiology for mechanical thrombectomy using the Inari ClotTriever and a sample of extracted thrombus was sent to pathology. Analysis on the sample was positive for sacral chordoma, consistent with tumor thrombus. The patient returned after 6 weeks with similar symptoms and repeat mechanical thrombectomy was performed with the Inari ClotTriever and stent placement through the left common and external iliac vein with an Ovation iX stent graft. The patient remained asymptomatic following the second procedure at repeat follow-up at 6 weeks.

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

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).

The Surgical Approach Combined With Minimally Invasive Surgery for Sacral Chordoma.

Sacral chordomas are malignant, locally aggressive, and rare tumors. Its presentation can be diverse on the entire spine, being more frequent in the sacrococcygeal region. The main treatment is complete surgical resection, which can be performed using different approaches depending on the case. We present the case of a 44-year-old woman with a history of a complex adnexal mass, with an imaging finding of a presacral mass displacing the uterus and rectum, with a histological report of an image-guided biopsy suggestive of a soft-tissue myoepithelioma-like tumor, which was managed with a combined approach: anterior transabdominal laparoscopic and posterior approach, achieving complete tumor resection, without postoperative complications and with the benefits of minimally invasive surgery. The pathology report of the surgical piece was compatible with sacral chordoma.

Dosimetric effect of variable rectum and sigmoid colon filling during carbon ion radiotherapy to sacral chordoma.

PURPOSE: Carbon ion radiotherapy (CIRT) is sensitive to anatomical density variations. We examined the dosimetric effect of variable intestinal filling condition during CIRT to ten sacral chordoma patients. METHODS: For each patient, eight virtual computed tomography scans (vCTs) were generated by varying the density distribution within the rectum and the sigmoid in the planning computed tomography (pCT) with a density override approach mimicking a heterogeneous combination of gas and feces. Totally full and empty intestinal preparations were modelled. In addition, five different intestinal filling conditions were modelled by a mixed density pattern derived from two combined and weighted Gaussian distributions simulating gas and feces respectively. Finally, a patient-specific mixing proportion was estimated by evaluating the daily amount of gas detected in the cone beam computed tomography (CBCT). Dose distribution was recalculated on each vCT and dose volume histograms (DVHs) were examined. RESULTS: No target coverage degradation was observed at different vCTs. Rectum and sigma dose degradation ranged respectively between: [-6.7; 21.6]GyE and [-0.7; 15.4]GyE for D50%; [-377.4; 1197.9] and [-95.2; 1027.5] for AUC; [-1.2; 10.7]GyE and [-2.6; 21.5]GyE for D1%. CONCLUSIONS: Variation of intestinal density can greatly influence the penetration depth of charged particle and might compromise dose distribution. In particular cases, with large clinical target volume in very close proximity to rectum and sigmoid colon, it is appropriate to evaluate the amount of gas present in the daily CBCT images even if it is totally included in the reference planning structures.

How LEM-based RBE and dose-averaged LET affected clinical outcomes of sacral chordoma patients treated with carbon ion radiotherapy.

PURPOSE/OBJECTIVE: To understand the role of relative biological effectiveness (RBE) and dose-averaged linear energy transfer (LETd) distributions in the treatment of sacral chordoma (SC) patients with carbon ion radiotherapy (CIRT). MATERIAL/METHODS: Clinical plans of 50 SC patients consecutively treated before August 2018 with a local effect model-based optimization were recalculated with the modified microdosimetric kinetic RBE model (mMKM). Twenty-six patients were classified as progressive disease and the relapse volume was contoured on the corresponding follow-up diagnostic sequence. The remaining 24 patients populated the control group. Target prescription dose (DRBE|50%), near-to-minimum- (DRBE|95%) and near-to-maximum- (DRBE|2%) doses were compared between the two cohorts in both RBE systems. LETd distribution was evaluated for in-field relapsed cases with respect to the control group. RESULTS: Target DMKM|50% and DMKM|95% were respectively 10% and 18% lower than what we aimed at. Dosimetric evaluators showed no significant difference, in neither of the RBE frameworks, between relapsed and control sets. Half of the relapse volumes were located in a well-covered high dose region. On average, over these cases, median target LETd was significantly lower than the control cohort mean value (27 vs 30 keV/µm). Most notably, the volume receiving dose from high-LET particles (>50 keV/µm) lay substantially below recently reported data in the literature. CONCLUSION: A combined multi model RBE- and LET-based optimization could play a key role in the enhancement of the therapeutic ratio of CIRT for large radioresistant tumors such as sacral chordomas.

Effect of hyperthermia and proton beam radiation as a novel approach in chordoma cells death and its clinical implication to treat chordoma.

PURPOSE: Chordoma is a locally aggressive tumor that most commonly affects the base of the skull/clivus, cervical, and sacral spine. Conventional radiotherapy (RT), cannot be safely increased further to improve disease control due to the risk of toxicity to the surrounding critical structures. Tumor-targeted hyperthermia (HT) combined with Proton Beam Radiation Therapy (PBRT) is known to act as a potent radiosensitizer in cancer control. In this study, we investigated whether PBRT efficacy for chordoma can be enhanced in combination with HT as a radiosensitizer. MATERIAL AND METHODS: Human chordoma cell lines, U-CH2 and Mug-chor1 were treated in vitro with HT followed by PBRT with variable doses. The colony-forming assay was performed, and dose-response was characterized by linear-quadratic model fits. HSP-70 and Brachyury (TBXT) biomarkers for chordoma aggression levels were quantified by western blot analysis. Gene microarray analysis was performed by U133 Arrays. Pathway Analysis was also performed using IPA bioinformatic software. RESULTS: Our findings in both U-CH2 and Mug-Chor1 cell lines demonstrate that hyperthermia followed by PBRT has an enhanced cell killing effect when compared with PBRT-alone (p < .01). Western blot analysis showed HT decreased the expression of Brachyury protein (p < .05), which is considered a biomarker for chordoma tumor aggression. HT with PBRT also exhibited an RT-dose-dependent decrease of Brachyury expression (p < .05). We also observed enhanced HSP-70 expression due to HT, RT, and HT + RT combined in both cell lines. Interestingly, genomic data showed 344 genes expressed by the treatment of HT + RT compared to HT (68 genes) or RT (112 genes) as individual treatment. We also identified activation of death receptor and apoptotic pathway in HT + RT treated cells. CONCLUSION: We found that Hyperthermia (HT) combined with Proton Beam Radiation (PBRT) could significantly increase chordoma cell death by activating the death receptor pathway and apoptosis which has the promise to treat metastatic chordoma.

Clinical Outcome of Sacral Chordoma Patients Treated with Pencil Beam Scanning Proton Therapy.

AIMS: Sacral chordomas are locally aggressive, radio-resistant tumours. Proton therapy has the potential to deliver high radiation doses, which may improve the therapeutic ratio when compared with conventional radiotherapy. We assessed tumour control and radiation-induced toxicity in a cohort of sacral chordoma patients treated with definitive or postoperative pencil beam scanning proton therapy. METHODS AND MATERIALS: Sixty patients with histologically proven sacral chordoma treated between November 1997 and October 2018 at the Paul Scherrer Institute with postoperative (n = 50) or definitive proton therapy (n = 10) were retrospectively analysed. Only 10 (17%) patients received combined photon radiotherapy and proton therapy. Survival rates were calculated using the Kaplan-Meier actuarial method. The Log-rank test was used to compare different functions for local control, freedom from distant recurrence and overall survival. Acute and late toxicity were assessed according to the Common Terminology Criteria for Adverse Events (CTCAE) v5.0. RESULTS: The median follow-up was 48 months (range 4-186). Local recurrence occurred in 20 (33%) patients. The 4-year local control, freedom from distant recurrence and overall survival rates were 77%, 89% and 85%, respectively. On univariate analysis, subtotal resection/biopsy (P = 0.02), tumour extension restricted to bone (P = 0.01) and gross tumour volume >130 ml (P = 0.04) were significant predictors for local recurrence. On multivariate analysis, tumour extension restricted to bone (P = 0.004) and gross total resection (P = 0.02) remained independent favourable prognostic factors for local recurrence. Twenty-four (40%), 28 (47%) and eight (11%) patients experienced acute grade 1, 2 and 3 toxicities, respectively. The 4-year late toxicity-free survival was 91%. Two patients developed secondary malignancies to the bladder 3-7 years after proton therapy. CONCLUSIONS: Our data indicate that pencil beam scanning proton therapy for sacral chordomas is both safe and effective. Gross total resection, tumour volume <130 ml and tumour restricted to the bone are favourable prognostic factors for local tumour control.

Sacral chordoma with incidental rectal adenocarcinoma: a case report.

BACKGROUND: We 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 PRESENTATION: This 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. CONCLUSION: To 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.

Rectal dose-sparing effect with bioabsorbable spacer placement in carbon ion radiotherapy for sacral chordoma: dosimetric comparison of a simulation study.

It is difficult to treat patients with an inoperable sarcoma adjacent to the gastrointestinal (GI) tract using carbon ion radiotherapy (C-ion RT), owing to the possible development of serious GI toxicities. In such cases, spacer placement may be useful in physically separating the tumor and the GI tract. We aimed to evaluate the usefulness of spacer placement by conducting a simulation study of dosimetric comparison in a patient with sacral chordoma adjacent to the rectum treated with C-ion RT. The sacral chordoma was located in the third to fourth sacral spinal segments, in extensive contact with and compressing the rectum. Conventional C-ion RT was not indicated because the rectal dose would exceed the tolerance dose. Because we chose spacer placement surgery to physically separate the tumor and the rectum before C-ion RT, bioabsorbable spacer sheets were inserted by open surgery. After spacer placement, 67.2 Gy [relative biological effectiveness (RBE)] of C-ion RT was administered. The thickness of the spacer was stable at 13-14 mm during C-ion RT. Comparing the dose-volume histogram (DVH) parameters, Dmax for the rectum was reduced from 67 Gy (RBE) in the no spacer plan (simulation plan) to 45 Gy (RBE) in the spacer placement plan (actual plan) when a prescribed dose was administered to the tumor. Spacer placement was advantageous for irradiating the tumor and the rectum, demonstrated using the DVH parameter analysis.

Partial sacrectomy with patient-specific osteotomy guides.

BACKGROUND: Chordomas are rare, locally aggressive, malignant tumors. Surgical resection with sufficient margins defines the outcome. However, the necessity for wide margins often leads to sacrifice of important neurological structures. 3D-printed osteotomy guides are a promising solution for precise execution of surgical resection. We present probably the first sacrococcygeal chordoma resection with 3D-printed guides. METHODS: The case of a 49-year-old woman with a sacrococcygeal chordoma, resected with help of 3-D pre-operative planning and patient-specific 3D-printed osteotomy guides, is reported in detail. RESULTS: A sufficient tumor excision could be performed successfully while sparing nerve root S4. The planed margin has been exactly maintained, as confirmed by histology. The patient demonstrated an excellent clinical outcome with no evidence of local recurrence. CONCLUSIONS: 3-D pre-operative planning and patient-specific osteotomy guides can be used for planning and performing en-bloc surgical resection of sacral chordomas.