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.

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.

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.

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.

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.

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.

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.

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.

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.

Transrectal EUS-guided FNA biopsy of a presacral chordoma-report of a case and review of the literature

Chordomas are rare tumors which originate from the remnants of the notochord. These tumors are locally aggressive and have a predilection for the ends of the axial skeleton. An important prerequisite for optimal management of these tumors is a correct preoperative diagnosis. The present case is the first report of the use of endoscopic ultrasound to obtain transrectal fine needle aspiration biopsy of a presacral chordoma. A review of the prior computer tomography (CT) scans allowed us to calculate the tumor volume doubling time (18.3 mo). Transrectal biopsy of chordomas is controversial, however we believe that such concerns are not justified.

Clinical Value of Interventional Embolization for Sacral Chordoma before Surgical Treatment

Objetive:To evaluate the efficacy of preoperative transcatheter arterial embolization in the treatment of sacral chordoma,and to explore the choice of embolization timing.Methods:32 patients underwent the posterior approach after TAE of the main arteries that supplied the sacral chordoma.Intraoperative bleeding amount of each patient was recorded and compared between-group differences at different operation times.Results:After embolization,12 patients were received resection within 24 hours(group A),that of 10 cases between 24~48h(group B),of another 10 cases between 48~72h(group C).All of the 32 tumors were removed intact with intraoperative bleeding about(894±199)ml,without any shock or death,nor injuries on abdominal organs such as rectum and ureter.There was no statistical significance in tumor size among group A,B and C(P>0.05).Data gave statistical significance in intraoperative blood loss between group A and B(P<0.01),there was no statistical differences between group B and C(P>0.05),in spite of group B slightly less than group C.Conclusion:Preoperative TAE for excising the sacral tumor can significantly decrease intraoperative blood loss,make the surgical field clear,and facilitate the maximal removal of the sacral chordoma.It would be best to select the embolization timing within 24 hours before surgical operation.

内镜下经鼻或经口入路颅底脊索瘤切除术后生活质量与临床和影像学特征的相关分析

目的探讨内镜下经鼻或经口入路颅底脊索瘤切除术后生活质量与临床和影像学特征的相关性。方法回顾总结2016年3月至2022年3月在天津市环湖医院行内镜下经鼻或经口入路手术的14例颅底脊索瘤患者的临床和影像学特点,采用36条简明健康状况调查表(SF‑36)评价手术后生活质量,采用Point‑Biserial相关分析和偏相关分析探究SF‑36评分及其各维度评分与临床和影像学特征的相关性。结果相关分析显示,SF‑36评分中躯体健康总分之躯体疼痛维度评分与头痛(r=‑0.998,P=0.043)、复视(r=‑0.999,P=0.032)、视力障碍(r=‑0.998,P=0.037)、海绵窦症状(r=‑0.997,P=0.047)、肿瘤次全切除或大部切除(r=‑0.998,P=0.038)呈负相关,与术后放射治疗(r=0.998,P=0.038)、全斜坡型肿瘤(r=0.998,P=0.036)、肿瘤体积>20 cm3(r=0.999,P=0.035)呈正相关。结论脊索瘤患者可存在术前头痛、复视、视力障碍、海绵窦症状以及术中肿瘤次全切除或大部切除的特征,可降低内镜下经鼻或经口入路手术后生活质量,临床对此类患者应予以关注。

SMARCB1/INI1缺失的低分化脊索瘤的临床病理特征

目的探讨SMARCB1/INI1缺失的低分化脊索瘤(poorly differentiated chordoma,PDC)的临床病理学特征。方法收集10例PDC临床资料,采用免疫组化EnVision两步法检测CK、vimentin、INI1和Brachyury等表达,分析PDC临床病理及预后特征。结果10例中男性5例,女性5例,发病年龄1~7岁(平均发病年龄及中位发病年龄均为4岁);10例发生在斜坡并均有骨质侵犯,其中3例累及颈椎(30%)。肿瘤组织形态上均表现为片状或巢团状生长,肿瘤细胞呈上皮样,细胞核呈圆形或卵圆形,异型性明显,可见核仁,有丝分裂活跃,间质内可见少许淋巴细胞浸润;免疫表型:10例肿瘤细胞均表达CK、vimentin、EMA和Brachyury,SMARCB1/INI1均表达缺失。随访及预后:5例肿瘤复发(中位无进展时间为4个月);7例死亡(中位生存期为5个月)。结论SMARCB1/INI1缺失的PDC相对罕见,预后较差,诊断具有挑战性,认识该肿瘤的临床病理学特征对诊断及治疗具有重要意义。

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.

Intradural spinal seeding and fatal progression of a sacrococcygeal chordoma： a case report

Chordoma as a rare malignant tumor arising from remnants of the fetal notochord accounts for 1%-4%of primary bone tumors. It is usually predominant in males. Local recurrences are common （44%-78%） and distant metastases may occur years after the initial presentation. The reported rates of metastases range from 10% to 48%, which are usually accompanied by a sacrococcygeal chordoma. Cases of surgical seeding and intradural spinal seeding also have been reported climcally. To our knowledge, the present case is the first one concerning cerebrospinal fluid metastases and fatal progression of a sacrococcygeal chordoma.

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.