Pre-Sacral Chordomas, about Two Cases and Review of the Literature

The World Health Organization (WHO) defines chordoma as a malignant tumor of intermediate or low grade, developing at the expense of embryonic remnants of the notochord. These tumors represent 8.4% of all malignant bone tumors. We report 2 cases collected in the medical oncology department at the CHU Hassan II in Fes. In whom the clinical examination revealed a mass and/or signs of loco-regional compression. The tumor was located in the sacrum in both patients. The diagnosis was based on the data of the anatomopathological examination showing the association of a lobulated architecture with classic physaliphore cells and a mucoid intercellular substance. Immunohistochemistry was performed for both patients. The principle of therapeutic management was based on radiotherapy and targeted therapy since surgery was difficult given the local tumor extension.

Expanded Endoscopic Endonasal Approach (EEEA) for Clival Chordomas

Objective: To evaluate the role of expanded endoscopic endonasal approach in removal of clival chordomas. Patients & Methods: Nine patients with clival chordomas were operated upon in Cairo University hospital from September 2015 to September 2018 using the EEEA a recurrent case and seven new cases were involved in these study and ten operations were done. All patients had preoperative neurological and radiological examination. The study was focusing on the approach, efficacy of tumor removal, reconstruction of the base and complications related to this approach. Results: Nine patients were operated in this study in which ten operations were done. It included six males (66.6%) and three females (33.3%) with age ranging from 4 years to 63 years with average age 40.7 years. Headache and diplopia were the most common symptoms found in six patients (66.6%). Brainstem affection was found in two patients (22.2%). Lower cranial nerves affection was found in two patients (22.2%). One case developed CSF leakage postoperatively (11.1%). Two patients underwent tracheostomy. We achieve total removal in four patients (44.4%), near total removal in one patient (11.1%) and subtotal tumor resection in four patients (44.4%). Conclusion: EEEA for clival chordomas is safe and effective approach regarding the results of the incidence of complications, and the percentage of tumor resection.

Preliminary Result of Hyperfractionated High-Dose Proton Beam Radiotherapy for Pediatric Skull Base Chordomas

Objective: Proton beam therapy (PBT) may provide good local control for skull base chordoma and reduced toxicities, especially for pediatric patients. Methods: We evaluated the efficacy and safety of hyperfractionated high-dose PBT in6 pediatric patients with newly-diagnosed skull basechordoma who were treated with PBT at our institute from 2011 to 2015. The patients were 5 males and one female, and the median age was 9 years old (range: 5 - 13). All patients received surgery before PBT. The median period between surgery and PBT was 57 days (range: 34 - 129 days). The treatment dose was 78.4 GyE in 56 fractions (twice per day). Results: All patients received PBT without severe acute toxicity. The median follow-up period was 27 months (range: 21 - 71 months). At the last follow-up, all patients were alive and all tumors were well controlled. Acute and late toxicities were generally acceptable, with only grade 1 and 2 events. Late toxicities included growth hormone abnormality and cortical hormone abnormality. One patient needed growth hormone and cortical hormone replacement therapy. Conclusion: Although the number of pediatric patients was small, our overall findings in the 6 cases indicate that hyperfractionated high-dose PBT is safe and effective for pediatric patients with skull base chordoma.

Chordomas and Their Consideration in the Radiographic Differential of Extra-Axial Lesions of the Central Nervous

Due to the rare occurrence of chordomas extra-axially, these lesions have not earned a great deal of consideration in the clinical and radiographic differential diagnoses of extra-axial paraspinal lesions. We describe a case of a patient with a surgically resected extra-axial chordoma and review the radiologic characteristics of chordomas as an entity in the spectrum of extra-axial lesions. A 47-year-old man presented to our institution with four months of intermittent parethesias, pain, and subjective weakness in the left leg. MR imaging of the lumbar spine demonstrated a large heterogeneously- enhancing paraspinal lesion extending from the left L3-4 neural foramen into the psoas muscle. The patient underwent a CT-guided needle biopsy demonstrating features consistent with a chordoma. He then underwent a left retroperitoneal approach and en bloc resection of the lesion from the vertebral column. Pathology confirmed chordoma as the diagnosis. EACs are a rare but important consideration in the diagnosis of extra-axial lesions of the central nervous system.

Various Aetiologies of Non-Traumatic Coccydynia Cause Pain in the Posterior Sacrococcygeal Leg of the Pelvic Tripod: A Burden on the Healthcare Sector

Introduction: Coccydynia, television disease, and coccygodynia are the different names given to this disabling disease, which can become chronic. It was described by Simson in 1859. Coccydynia means pain at the end of the vertebral column. Non-traumatic coccydynia is a diagnosis, which is never straightforward like traumatic coccydynia because the onset is unclear, and both the patient and the unaware clinician face many challenges in treating it on time and with accuracy. Coccyx was likened to a cuckoo bird’s beak as a curved bone of fused 3 to 5 vertebrae with remnant disc material in some rare cases, unfused segments, linear scoliosis or subluxations and deformities. Stress X-rays of the coccyx in the antero-posterior and lateral views in standing and sitting reveal the “Dynamic Instability” due to congenital coccygeal morphological, pathological and mechanical variations. Material and Methods: This is a complex study having retrograde data collected from online publications from various databases, like PubMed, Embase, and Cochrane Library and also antegrade data collected from 100 patients with their consent from patients in Adam and Eve Specialised Medical Centre-based at Abu Dhabi, UAE and data was processed in the research centre of Krushi Orthopaedic Welfare Society based in India between 2014-2024 following all guidelines of Helsinki and approved by the ethics board of Krushi Orthopaedic Welfare Society. Clinical Presentation: The coccyx is painful, with aches, spasms, and an inability to sit. This affects daily activities without any particular date of onset. The onset remains insidious for the non-traumatic variety of coccydynia. Aetiology and Patho Anatomy: Non-traumatic coccydynia can be caused by a myriad of reasons, like congenital morphological variations, acquired dynamic instabilities, and hidden trauma remaining quiescent to re-surface as a strain-induced pain. Radiological Presentations: Unless clarity is focused on these coccygeal views, the errors of the unevacuated rectum, non-dynamic standing views, improper X-ray exposure and refuge by insurance companies to approve the much needed but multiple views in radiological investigation (Stress X-ray), MRI scan, lack of awareness by the clinician, all lead to missed diagnosis with its repercussions as congenital variations in morphology, acquired changes in structure/mobility, pathologies like tumours like congenital teratoma & adult onset chordoma, Tarlov cysts, pilonidal sinus or infections—even tuberculosis, dural syndrome, stiff coccyx due to ankylosing spondylitis and many others like relation to neurosis have all been documented. Treatment options are outside the scope of this research topic, as only the differential diagnosis is being stressed here, so that the clinician and the patient do not overlook the varying aetiology, which is the first step to timely and appropriate treatment. Conclusion: Level 3 evidence is available pointing towards many aetiologies causing non-traumatic coccydynia, and in this study of 100 patients by Krushi O W S, a non-profit organisation, the results were as follows: 1) Coccydynia is more common in Type II coccyx and bony spicules. 2) Coccydynia is more prevalent when the sacrococcygeal joints are not fused. 3) Coccydynia is more prevalent when there is subluxation at the intercoccygeal joints. 4) Coccydynia is more when the sacral angle is lower. 5) Coccydynia is associated with higher sacrococcygeal curved length. 6) Coccydynia is associated with a lower sacrococcygeal curvature index. 7) Gender variations: The coccygeal curvature index was lower in females with coccydynia;the intercoccygeal angle was lower in males. 8) Both obese and thin individuals can get affected due to different weight-bearing mechanics in play.

Chordomas of the upper cervical spine： clinical characteristics and surgical management of a series of 21 patients

Background Chordomas of the upper cervical spine are rare and present unique surgical challenge.This study aimed to describe the clinical characteristics and surgical management of patients with chordomas of the upper cervical spine.Methods Twenty-one patients with chordomas of the upper cervical spine who were treated in Peking University Third Hospital from January 1999 to October 2012 were retrospectively analyzed.Survival was calculated by the Kaplan-Meier method and was compared between groups using the log-rank test.Results The postoperative diagnosis was classical chordoma in 20 cases and chondroid chordoma in one case.The mean operative time was 9.5 hours （range 6-17 hours）,and the mean blood loss was 2 812 ml （range 700-4 800 ml）.There were two postoperative deaths.Unilateral vertebral artery ligation was performed in six patients,cervical nerve roots were cut in six patients,and the external branch of the superior laryngeal nerve was repaired after being cut in one case.Two patients developed postoperative velopharyngeal incompetence,and loosening of the occipitocervical screws was observed in one patient.The recurrence rate was 66.7% （10/15） after a mean follow-up period of 46.8 months （range 14-150 months）.The 5-and 10-year overall survival rates were （39.8±13.1）% and （31.9±12.7）%,respectively.There was a significant difference in survival rate between patients who underwent surgery and those who did not.Conclusion In spite of the high rates of recurrence and complications after surgical treatment of chordomas of the upper cervical spine,intralesional resection combined with adjuvant radiotherapy remains the optimal treatment to prolong survival.

Computer Navigation-aided Resection of Sacral Chordomas

Background： Resection of sacral chordomas is challenging. The anatomy is complex, and there are often no bony landmarks to guide the resection. Achieving adequate surgical margins is, therefore, difficult, and the recurrence rate is high. Use of computer navigation may allow optimal preoperative planning and improve precision in tumor resection. The purpose of this study was to evaluate the safety and feasibility of computer navigation-aided resection of sacral chordomas. Methods： Between 2007 and 2013, a total of 26 patients with sacral chordoma underwent computer navigation-aided surgery were included and followed for a minimum of 18 months. There were 21 primary cases and 5 recurrent cases, with a mean age of 55.8 years old （range： 35 84 years old）. Tumors were located above the level of the $3 neural foramen in 23 patients and below the level of the $3 neural foramen in 3 patients. Three-dimensional images were reconstructed with a computed tomography-based navigation system combined with the magnetic resonance images using the navigation software. Tumors were resected via a posterior approach assisted by the computer navigation. Mean follow-up was 38.6 months （range： 18-84 months）. Results： Mean operative time was 307 min. Mean intraoperative blood loss was 3065 ml. For computer navigation, the mean registration deviation during surgery was 1.7 ram. There were 18 wide resections, 4 marginal resections, and 4 intralesional resections. All patients were alive at the final follow-up, with 2 （7.7%） exhibiting tumor recurrence. The other 24 patients were tumor-free. The mean Musculoskeletal Tumor Society Score was 27.3 （range： 19-30）. Conclusions： Computer-assisted navigation can be safely applied to the resection of the sacral chordomas, allowing execution of preoperative plans, and achieving good oncological outcomes. Nevertheless, this needs to be accomplished by surgeons with adequate experience and skill.

Skull base chordomas review of current treatment paradigms

Background:Chordomas are locally invasive neoplasms,arising from notochordal remnants and can appear anywhere along the axial skeleton.Local recurrences are common,and distant metastases may occur years after the initial presentation.Methods:Literature review of current treatment strategies for chordomas of the skull base.Results:Surgery is the mainstay of treatment and complete resection has paramount importance for prognosis.When complete resection is not achieved recurrent disease is common.The anatomical complexity of the skull base makes resection complex.Endonasal endoscopic approaches to the clivus has become increasingly favored in recent years although addressing reconstruction of the skull base to prevent CSF leak may be challenging.Evidence suggests that radiotherapy should not be considered as a primary single modality when trying to achieve cure of the disease.Nonetheless,immediate post-operative radiotherapy improves survival.Many strategies have been suggested to preserve sensitive vital structures in the skull base during treatment but as for survival there is no evidence of advantage when comparing adjuvant therapy with photon radiotherapy,gamma knife surgery,proton beam therapy,and carbon ion radiation therapy.There is no evidence to support cytotoxic chemotherapy in the treatment of chordomas but targeted therapies have started to show promise.Several optional molecular targets exist.Brachyury is overexpressed in 95%of chordomas but not in other mesenchymal neoplasms.However,its precise role in chordoma pathogenesis is currently unclear,and its cellular location in the nucleus makes it difficult to target.The inhibition of brachyury in chordoma cell lines induces growth arrest and apoptosis.This does not have clinical application to date.There are retrospective results with different molecular targeted therapies for advanced chordomas with some effectiveness.Conclusion:Despite improvements made in the past 10 years in our knowledge of chordoma biology,available therapies still offer a limited benefit.There is an unmet need for new therapeutic options for patients with advanced disease.Therefore,patients with advanced disease should be encouraged to participate in clinical trials when and where available.

An overview of the role of cancer stem cells in spine tumors with a special focus on chordoma

Primary malignant tumors of the spine are relatively rare, less than 5% of all spinal column tumors. However, these lesions are often among the most difficult to treat and encompass challenging pathologies such as chordoma and a variety of invasive sarcomas. The mechanisms of tumor recurrence after surgical intervention, as well as resistance to radiation and chemotherapy, remain a pervasive and costly problem. Recent evidence has emerged supporting the hypothesis that solid tumors contain a sub-population of cancer cells that possess characteristics normally associated with stem cells. Particularly, the potential for long-term proliferation appears to be restricted to subpopulations of cancer stem cells(CSCs) functionally defined by their capacity to self-renew and give rise to differentiated cells that phenotypically recapitulate the original tumor, thereby causing relapse and patient death. These cancer stem cells present a unique opportunity to better understand the biology of solid tumors in general, as well as targets for future therapeutics. The general objective of the current study is to discuss the fundamental concepts for understanding the role of CSCs with respect to chemoresistance, radioresistance, special cell surface markers, cancer recurrence and metastasis intumors of the osseous spine. This discussion is followed by a specific review of what is known about the role of CSCs in chordoma, the most common primary malignant osseous tumor of the spine.

Critical evaluation of contemporary management in a new Pelvic Exenteration Unit: The first 25 consecutive cases

AIM To critically appraise short-term outcomes in patientstreated in a new Pelvic Exenteration(PE) Unit.METHODS This retrospective observational study was conducted by analysing prospectively collected data for the first 25 patients(16 males, 9 females) who underwent PE for advanced pelvic tumours in our PE Unit between January 2012 and October 2016. Data evaluated included age, co-morbidities, American Society of Anesthesiologists(ASA) score, Eastern Cooperative Oncology Group(ECOG) status, preoperative adjuvant treatment, intra-operative blood loss, procedural duration, perioperative adverse event, lengths of intensive care unit(ICU) stay and hospital stay, and oncological outcome. Quantitative data were summarized as percentage or median and range, and statistically assessed by the χ~2 test or Fisher's exact test, as applicable.RESULTS All 25 patients received comprehensive preoperative assessment via our dedicated multidisciplinary team approach. Long-course neoadjuvant chemoradiotherapy was provided, if indicated. The median age of the patients was 61.9-year-old. The median ASA and ECOG scores were 2 and 0, respectively. The indications for PE were local y invasive rectal adenocarcinoma(n = 13), advanced colonic adenocarcinoma(n = 5), recurrent cervical carcinoma(n = 3) and malignant sacral chordoma(n = 3). The procedures comprised 10 total PEs, 4 anterior PEs, 7 posterior PEs and 4 isolated lateral PEs. The median follow-up period was 17.6 mo. The median operative time was 11.5 h. The median volume of blood loss was 3306 mL, and the median volume of red cell transfusion was 1475 mL. The median lengths of ICU stay and of hospital stay were 1 d and 21 d, respectively. There was no case of mortality related to surgery. There were a total of 20 surgical morbidities, which occurred in 12 patients. The majority of the complications were grade 2 Clavien-Dindo. Only 2 patients experienced grade 3 Clavien-Dindo complications, and both required procedural interventions. One patient experienced grade 4a Clavien-Dindo complication, requiring temporary renal dialysis without long-term disability. The R0 resection rate was 64%. There were 7 post-exenteration recurrences during the follow-up period. No statistically significant relationship was found among histological origin of tumour, microscopic resection margin status and postoperative recurrence(P = 0.67). Four patients died from sequelae of recurrent disease during follow-up.CONCLUSION By utilizing modern assessment and surgical techniques, our PE Unit can manage complex pelvic cancers with acceptable morbidities, zero-rate mortality and equivalent oncologic outcomes.

Recurrent cervical chordoma:A case illustration

A case is described of multi-recurrent cervical chordoma in a man over a 5 year period. The clinical features were of progressive spinal cord compression. The authors report a chordoma at C4 that recurred 3 times in five years. The patient underwent four operations and suffered distant metastases. This case confirms that thorough resection of the tumor during the first surgery and post- operative adjuvant treatment are the best assurance of a good prognosis with a chordoma. Multiple surgeries can stimulate biological activity of a chordoma.and make its recurrence and distant metastases much more likely. The authors discuss the diagnosis, surgical treatment and the relationship between the histopathological changes and malignancy of a spinal chordoma after four operations. To our knowledge, this represents the first report of a 4th surgery for cervical chordoma.

鼻咽型脊索瘤1例

1临床资料患者,男,21岁,因体检发现鼻咽肿物2 d入院。2 d前因咽部不适到外院查纤维喉镜示鼻咽部新生物,无鼻塞、流涕及血涕,无头痛、头晕,无嗅觉减退,无发热,无视物模糊或复视,无耳鸣、耳痛。鼻内镜检查示:鼻咽顶壁肿胀隆起,血管扩张,表面光滑,似囊肿样物。鼻咽MRI示:鼻咽顶后壁黏膜下结节状病灶,考虑囊肿可能性大,双侧咽旁及颈部未见明确肿大淋巴结(图1)。

Multilevel oblique corpectomies as an effective surgical option to treat cervical chordoma in a young girl

Chordomas are malignant tumors arising from notochordal remnants. They are the most frequent tumors of the spine after plasmacytomas. Only 6% of chordomas are localized to the cervical level. In young patients, chordomas are rare and unpredictable. Despite this, the treatment of choice remains the total resection, as much as possible, followed by proton beam radiation. This case was managed using a precarotid and retrocarotid approach at the same time. The tumor was completely resected with the edges free from disease. The cervical spine was stabilized with an anterior plating C2-C4. Eighteen months after surgery the patient is still free from illness. Multilevel oblique corpectomies are an available and safe option for the treatment of upper cervical chordomas.

Chordoma of petrosal mastoid region:A case report

BACKGROUND Chordoma is a rare low-grade malignant tumor originating from embryonic notochordal tissue mainly occurring in the axial bone,mostly in the sphenooccipital junction and sacrococcyx,which accounts for approximately 1%of all malignant bone tumors and 0.1%–0.2%of intracranial tumors.Chordoma in the petrous mastoid region is rare.CASE SUMMARY We describe a 36-year-old male patient with chordoma in the left petrous mastoid region.The main clinical manifestations were pain and discomfort,which lasted for 2 years.Magnetic resonance imaging showed a lobulated mass in the left petrous mastoid with an unclear boundary and obvious enhancement.The tumor was completely removed after surgical treatment,and a histological examination confirmed that the tumor was a chordoma.During 5 years of follow-up,no clinical or radiological evidence of recurrence or metastasis was found.CONCLUSION Chordoma in the petrosal mastoid region is rare but should be included in differential diagnosis of petrosal mastoid tumors.

Sellar Chordoma Presenting as Pseudo-macroprolactinoma with Unilateral Third Cranial Nerve Palsy

We described a 61-year-old female with a sellar chordoma, which presented as pseudo-macroprolactinoma with unilateral third cranial nerve palsy. Physical examination revealed that her right upper lid could not be raised by itself, right eyeball movement limited to the abduction direction, right pupil dilated to 4.5 mm with negative reaction to light, and hemianopsia in bitemporal sides. CT scanning showed a hyperdense lesion at sellar region without bone destruction. Magnetic resonance imaging （MRI） revealed the tumor was 2.3 cmx2.8 cmx2.6 cm, with iso-intensity on TlWl, hyper-intensity on T2WI and heterogeneous enhancement on contrast imaging. Endocrine examination showed her serum prolactin level increased to 2,032.49 mlU/ml. The tumor was sub-totally resected via pterional craniotomy under microscope and was histologically proven to be a chordoma. Postoperatively, she recovered uneventfully but ptosis and hemianopsia remained at the 6th month.

IMMUNOHISTOCHEMICAL STUDY OF SIXTEEN CASES OF CHORDOMA

Sixteen cases of chordoms were studied with various polycolonal and monoilonal antibodies and ABC technic.such as antibodies S-100 protein, neurou specific enodas(NSE ), lysozyme, α1- AT, Myoglobin, desmin, keratin and cytokeratin. Two embryonic notochords and three chondrosarcomas were studied as control. Most of chordomas revealed positive results with NSE ( 12/16 ). Des ( 12 / 16 ) . α1-AT ( 11/ 16 ), MG( 11/16 ) . S-100( 10 /16)and CK ( 8 / 16 ), while K and Lys were negative in all( 16 )cases. It denotes that chordoma has the potentiality for both mesodermal and ectodermal differentiation. Lys was positive in all 3 chondrosarcomas while the CK was negative. So that Lys can be considered as an useful antibody to distinguish chordoma from chondrosarcoma, and CK is somewhat useful for this differential diagnosis too.

Chordoma Sociodemographic, Clinical and Therapeutic Aspects in National Institute of Oncology Rabat Morocco: A Report of 9 Cases

Introduction: Chordoma is a rare bone tumor, which develops mainly from the sacrum, the base of the skull, or the spine. Surgery + radiotherapy (if necessary) is the standard treatment. Data on chordoma are scarce in this region, and thus, here we summarized 9 patients with this tumor whom we treated in this institute. Material: Nine chordoma patients were summarized, who were treated in National Institute of Oncology in Rabat between 2013 and 2018. We retrieved data from medical charts and analyzed the clinical characteristics of this tumor. Results: The average age was 49 years (range: 29 - 72), with male: female of 3:6. The manifestation-diagnosis time was 4 months (range: 2 - 14). Regarding the tumor location, lumbosacral spine;5, the skull;4. Mass was evident in 6. Signs of locoregional compressions (paraparesis or tetraparesis) were observed in 3. As for treatment, a partial tumor excision was performed in 8, with 3 patients undergoing a wide excision. Radiotherapy was done;3 patients with a dose of 46 Gy, 3 patients with 66 Gy, 1 patient with 50 Gy, and 1 patient with a 16 Gy gamma radio-knife in a single session. 4 patients with a dose of 46 Gy, 2 patients with 60 Gy, and 1 patient with a 16 Gy gamma radio-knife in a single session. Of 9, 4 patients had good locoregional control whereas 5 patients had local recurrence. Conclusion: Chordoma is a predominantly local aggressive tumor with low metastatic potential. The surgical excision remains the main prognostic factor. Advances in radiotherapy may improve local control. These data are of use in management of this tumor in Rabat (Morocco).

Surgical Excision of Clivus Chordoma with the Use of Coblator—A Case Report

Chordomas are dysembryogenic tumors originating from the notochordal process [1] [2]. They are aggressive tumours with unique diagnostic and management challenges. Primary therapy is complete surgical removal of the tumour as much as possible. The likelihood of recurrence is high in spite of complete surgical resection. A 52-year-old female patient presented with complaints of decreased vision in right eye, nasal bleeding, nasal blockage and difficulties in swallowing. CT scan and nasal biopsy were performed which confirmed the diagnosis of clivus chordoma. The CT scan showed extension into nasopharynx, nasal cavity and oropharynx pushing onto the soft palate. Surgical excision of the mass was performed with coblator by both intraoral and intra nasal approach [3]. On follow-up, nasal endoscopy and CT were done;the patient was relieved of the symptoms and was clinically better.

Chordoma in Nasopharynx in a 70-Year-Old Female: A Rare Occurence

The nasopharynx is an unusual site for extraosseous chordoma. The characteristic histology and immunohistochemistry confirmed the diagnosis. The behaviour of this tumour is locally aggressive. We report a case of nasopharyngeal chordoma in an elderly patient with literature review.

颅底脊索瘤的诊治进展

脊索瘤来源于脊索胚胎残余组织产生的原发性骨性肿瘤，是一种少见的慢性进展的低度恶性肿瘤，占全部原发恶性肿瘤的3％～4％，居原发性恶性骨肿瘤第4位。有约1／3起源于颅底部，其余可起源于脊柱，骶尾部或中线以外的位置，其中颅底脊索瘤占所有颅脑肿瘤发病率的0．1％～0．7％，发生于任何年龄，