Intradural Chordoma Research Articles

Primary Intradural Chordoma along the Cauda Equina

Chordoma is a rare malignancy originating from remnants of the primitive notochord along the midline craniospinal axis, usually seen as an osseous lesion of the skull base, spinal column, and sacrum. Several cases of intracranial intradural chordomas have been reported; however, we present a case of primary intradural chordoma along the cauda equina, which, on literature review, has not been previously described. Although this is an uncommon location for a chordoma, it should be considered in the differential diagnosis of an intradural lumbar mass because chordoma has a high recurrence rate following surgery and may influence a surgeon’s approach to the lesion.

Letter: Chordoma and Ecchordosis Physaliphora: 2 Sides of the Same Coin.

To the Editor: Chordoma and ecchordosis physaliphora (EP) are tumors arising from the notochord's remains along the axial skeleton from the dorsum sella to the sacrococcygeal region. Each year, 1 person in a million is diagnosed with chordoma, but only 45 cases of EP have been reported.1-12 In 1857, Virchow invented the acronym EP for the newly discovered dorsum sella lesion, believing it was cartilage related. A year later, Muller discovered notochord rests in the synchondrosis sphenooccipitalis and hypothesized that these were the source of the EP dorsum sella masses. Ribbert then endorsed this theory.13 Over a century later, Congdon and Wolfe coined the terms benign chordoma and intradural chordoma.14,15 Cha et al justified distinguishing EP from chordoma because they are treated differently and have a different prognosis because one is more aggressive than the other. So far, approximately 45 cases of EP have been diagnosed, with 10 patients displaying symptoms. In 1 of these cases, the patient died as a result.13 Two chief aspects spring to mind when pondering this subject. The first is the manner in which these instances were diagnosed and distinguished from chordoma cases. The second issue stems from the first, namely how to determine the ideal modality of treatment when there is no objective way to distinguish between those pathologies. The articles concur that it is impossible to distinguish between them based on histology or immunohistochemistry staining results because they are derived from the same embryonic notochord. Although certain histopathologic characteristics such as hypocellularity, sparse pleomorphism, and the lack of mitosis may aid in the differentiation of EP from chordoma, they are not considered diagnostic criteria.5,6,16 In addition, it has been stated that an intradural location favors EP over chordoma, yet there are instances of chordoma being an intradural.17 From an imaging point of view, chordoma frequently presents a contrast-enhanced lesion. However, it may occasionally exhibit a mild or nonenhanced lesion similar to EP.18 A study of 28 patients found that 65% of magnetic resonance images of chordoma showed mild or no enhancement.19 Moreover, all reported spinal EPs demonstrated enhanced extradural mass, similar to chordoma lesions.3-6,13 In conclusion, differentiation based on histology, immunohistochemistry staining, radiologic results, or the presence of symptoms is inaccurate. Based on the foregoing, it is impossible, or at least impractical, to distinguish between the 2 terms because they refer to the same ailment. Additional environmental or genetic factors may influence the severity and progression of the disease.20 It would be prudent to consolidate these 2 disorders as part of the spectrum of the same disease, chordoma, and classify it based on severity according to the radiologic findings for size, location, and bone involvement. We hope this paradigm shift will lay the groundwork for novel approaches to chordoma management.

Isolated cervical extraosseous intradural chordoma attached to the C5 nerve root: a case report

BackgroundAs chordomas are slow growing and locally invasive with high recurrence rates, initial recommendations include complete surgical resection with or without radiation therapy. A large proportion of recurrences occur years after initial resection necessitating lengthy follow-up. The novel biomarker brachyury and the repurposing of pharmaceutical products have the potential to substantially impact long-term recurrence rates.Case presentationA 43-year-old woman presented with an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root underwent a C3-5 laminectomy, C3-5 lateral mass screw instrumentation, and mass resection. All symptoms resolved by the 12-month postoperative follow-up visit.ConclusionsThis is the first report of an isolated, cervical extraosseous intradural extramedullary chordoma attached to a nerve root, and this case adds to the previous six Type IV chordomas in the literature. Unfortunately, the very rare form of extraosseous intradural chordoma is poorly understood: the lack of detailed knowledge in how they are differentiated from other forms of chordoma confounds the development of optimal treatment strategies and follow-up guidelines.

Expanded Endoscopic Endonasal Approach for Resection of Intradural Chordoma: Surgical and Anatomic Nuances: 2-Dimensional Operative Video.

The expanded endoscopic endonasal approach (EEA) has been growing as a surgical alternative for the treatment of clival chordomas because of their frequent midline location and bone erosion. The endoscopic transclival approach provides with a safer and more direct anatomic route for tumors located predominantly in the midline contributing to minimize postoperative comorbidities. In this video, we demonstrate the step-by-step technique for resection of such challenging clival pathology. This is an operative video of an extended endoscopic resection of a clival chordoma with stepwise description of the surgical technique. We present the case of a 49-yr-old man in whom, incidentally in the context of low testosterone level, a clival lesion with purely midline location with intradural extension into the ventral brainstem and occupation of the left cerebellopontine angle was discovered. The patient was submitted to an expanded endoscopic transclival approach and a macroscopic gross total resection was successfully achieved. The final pathology was compatible with a conventional chordoma. This video details the surgical anatomy of the clival region to facilitate the identification of surgical landmarks and anatomic boundaries with the goal of avoiding injury to the neurovascular structures involved in this approach. Extended endoscopic transclival surgery is a useful and safer option for the management of midline chordomas because it provides with a dissection corridor free of major neurovascular structures. Endoscopic techniques are associated with good outcomes in terms of macroscopic gross total resection and low surgical risks in these selected tumors.

Intradural cervical chordoma with diffuse spinal leptomeningeal spread: case report and review of the literature

Chordoma is a low-grade malignant bone tumor derived from embryonic notochord remnants along the axial skeleton. About 50% of chordomas occur in the sacral vertebrae and 35% in the skull base. Most chordomas are extradural and cause extensive bone destruction. Intradural spinal tumors without bone involvement are rare. We herein describe the clinical features of a patient with a chordoma as well as the imaging and pathological manifestations of the tumor. We encountered an unusual presentation of a C6 and C7 spinal intradural chordoma in a 23-year-old man. He presented with a 5-day history of discomfort over the lumbosacral region. Magnetic resonance imaging and enhanced scanning of the cervical spine showed an intradural soft tissue mass at C6 and C7 and linear enhancement of the spinal meninges. The tumor was excised because the patient had been previously misdiagnosed with an intraspinal neurogenic tumor with spinal meningitis. Postoperative pathological examination confirmed the diagnosis of chordoma. On postoperative day 7, the patient underwent brain magnetic resonance imaging because of severe headache. The images showed multiple soft tissue nodules in the skull base cistern. To the best of our knowledge, this is the first case report of an entirely extraosseous and spinal intradural chordoma with diffuse spinal leptomeningeal spread. The patient died 2months postoperatively. An intradural spinal chordoma is difficult to distinguish from a neurogenic tumor by imaging. When the lesion is dumbbell-shaped, it is easily misdiagnosed as a schwannoma. In the present case, the tumor was intradural and located at the level of the C6 and C7 vertebrae. Preoperative diagnosis was difficult, and the final diagnosis required pathological examination.

Delayed intraventricular metastasis of clival chordoma.

Sir, Intracranial chordomas are typically clival and extradural in location, yet these tumors can obviously invade the dura. However, primary intradural chordomas without local extension is a rare event with approximately 47 cases reported in literature. Furthermore, all intradural chordoma reports appear to be extraventricular in location.[1,2] In addition, through advances in technology and optics, neuroendoscopy has come to the forefront as a mainstream operative technique. Some tumors are more conducive to endoscopic biopsy or resection than others due to hemorrhagic or consistency. We present this unique case of an intraventricular recurrence of a clival chordoma and successful use of neuroendoscopy to explore and resect the tumor. A 60-year-old male, with a history of clival chordoma resection and adjuvant radiation nearly 10 years prior, presented with an intraventricular lesion on magnetic resonance imaging (MRI) obtained for stroke workup of the left sided weakness. Physical examination showed right third nerve palsy, left facial droop, and left sided weakness. MRI demonstrated an intraventricular enhancing lesion within the right lateral ventricle [Figure ​[Figure1a1a and ​andb].b]. No other evidence of metastasis was identified. The decision was made to endoscopically biopsy and resects the intraventricular lesion via a transcortical-transventricular approach [Figure 1c]. Postoperative MRI demonstrated gross total resection [Figure 1d]. The histopathologic diagnosis of the tumor was chordoma. No postoperative radiation was given. On 6-month follow-up imaging, the lesion has not recurred. Figure 1 (a and b) Preoperative magnetic resonance imaging demonstrates an enhancing lesion, approximately 1.5 cm × 1.0 cm, along the ependymal surface within the right lateral ventricle caudothalamic notch. (c) Intraoperative view of intraventricular ... This case illustrates a unique occurrence of intraventricular recurrence of clival chordoma approximately 10 years after initial resection with good result via an endoscopic technique. Prior reports have typically focused on intradural, extraventricular metastases. A solitary intraventricular chordoma neoplasm is a rare, interesting event. Furthermore, neuroendoscopy is a useful technique for tumor resection and proves viable in the resection of chordomas. Financial support and sponsorship Nil. Conflicts of interest There are no conflicts of interest.

Intradural chordoma presenting with intratumoral bleeding

Intradural clival chordomas are very rare, and only 29 cases have been reported to our knowledge. They arise purely intradurally without bone or dural involvement and may differ from classic clival chordomas in physiopathology and management. We present a 28-year-old woman who presented with intradural clival chordoma and tumoral bleeding. After initial gross macroscopic surgical resection, she presented with tumor recurrence after 2years, again with intratumoral bleeding. Although usually considered to have a more favorable prognosis in comparison to typical chordomas, intradural chordomas appear to behave as typical chordomas. Intratumoral bleeding may be a sign of an aggressive lesion and risk of recurrence. We highlight the differential diagnosis of intrinsic posterior fossa bleeding, especially in young patients. Intradural chordomas may be underdiagnosed and incorrectly treated as other types of parenchymal hemorrhage.

Intracranial hypertension syndrome and multiple cranial neuropathy related to atypical radiologic presentation of an intradural chordoma

Intracranial hypertension syndrome and multiple cranial neuropathy related to atypical radiologic presentation of an intradural chordoma

Intradural clival chordoma: a case report.

Clival chordoma is a rare intracranial neoplasm located in the clivus with bony extension and destruction. It is difficult to resect completely and generally has a poor prognosis. However, intradural clival chordomas have been reported with good surgical outcomes. We present a rare case of intradural chordoma and a review of the literature.

Giant ecchordosis physaliphora in an adolescent girl

The authors describe a rare case of giant ecchordosis physaliphora (EP) in a 16-year-old female patient who presented with diplopia. Magnetic resonance imaging of the brain with Gd contrast revealed a 3.0 × 1.7 × 1.8-cm nonenhancing, extraaxial epidural mass along the dorsal aspect of the clivus that was T2 hyperintense and T1 isointense with intermediate signal on diffusion sequences. Resection via a transnasal transsphenoidal approach to the ventral clival wall resulted in a stable tumor size with no evidence of interval growth after 30 months. Although this case features a strictly extradural EP, this tumor more commonly occurs in the subdural space and requires differentiation from intradural chordoma. Unlike EP, intradural chordoma may enhance with Gd contrast, is more likely to be associated with cranial nerve palsies and brainstem symptoms, and will occasionally have an elevated MIB-1 index. In this paper the authors highlight the different possible midline locations for both EP and chordoma, the difficulty in distinguishing between intradural giant EP and intradural chordoma, and the potential occurrence of these lesions in young people despite their typically slow rate of growth, while also underscoring the need for further investigation into the tumors' cytogenetic behavior.

A case of ecchordosis physaliphora presenting with an abducens nerve palsy: A rare symptomatic case managed with endoscopic endonasal transsphenoidal surgery

Background:Ecchordosis physaliphora (EP) is a benign notochordal remnant that is usually asymptomatic; symptomatic cases are extremely rare. Most of the reported symptomatic cases were managed by resection via craniotomy.Case Description:We report a case of a 20-year-old male presenting with abducens nerve palsy. Magnetic resonance imaging performed on admission demonstrated a mass in the retroclival prepontine location. The patient was treated successfully by endoscopic endonasal trans-sphenoidal surgery (ETSS), his postoperative course was uneventful, and the abducens nerve palsy disappeared.Conclusion:ETSS has advantages not only for treatment but also for differentiation between EP and intradural chordoma. This is the first case of symptomatic EP successfully treated solely by ETSS.

Intracranial intradural chordoma presenting with intraventricular hemorrhage

Chordomas constitute 1% of intracranial tumors and are believed o originate from the remnant notochord. These tumors are usually ocated in the extradural bony structures of the base of the skull ecause they arise from the spheno-occipital synchondrosis and lowly erode the surrounding structures. However, some cases of ntradural chordoma have been reported [1,2]. To the best of our nowledge, there is no case report on intraventricular hemorrhage s an initial symptom of intradural chordoma.

Intradural chordoma of the Meckel's cave: A challenging differential diagnosis

Chordomas are midline tumors that arise from embryonic remnants of the notochord and are considered to be malignant tumors because of their tendency to invade and destroy the involved bone. Cases of intradural chordomas without bone involvement have been rarely described with a predilection for prepontine location. The absence of bony invasion renders the complete excision of these tumors more feasible and is related to their better prognosis in comparison to conventional chordomas. Herein we report the first intradural chordoma arising in the Meckel's cave. The intradural location of the lesion, outside midline structures, in the absence of bone infiltration, made the differential diagnosis versus other meningeal lesions such as chordoid meningioma challenging. The intense and strong immunohistochemical expression of pan-cytokeratins, S100, cytokeratin-19 and of the notochordal marker brachyury allowed differential diagnosis toward other tumors showing chordoid morphology. The expression of brachyury, which had not been previously analyzed in intradural chordoma, definitely links the histogenesis of this neoplasia to the notochord, similar to that of conventional chordoma. We also show that, different from conventional chordoma, intradural chordoma does not express the metallo-proteinases (MMPs) -2 and -9, which may account for its indolent biological behavior.

Validity of the COPCORD Core Questionnaire as a Classification Tool for Rheumatic Diseases

Rheumatic diseases are vastly underdiagnosed and undertreated, particularly among minorities and those of low socioeconomic status. The WHO-ILAR Community Oriented Program in the Rheumatic Diseases (COPCORD) advocates screening of musculoskeletal complaints in the community. The objective of this study was to evaluate the performance of the COPCORD Core Questionnaire (CCQ) as a diagnostic tool for rheumatic diseases. We conducted a cross-sectional study designed in parallel with a large COPCORD survey in Mexico. A subsample of 17,566 questionnaires, selected from 4 of the 5 states included in a national COPCORD survey were included in the analysis as a diagnostic test to evaluate sensitivity, specificity, receiver operating characteristics curve (ROC), and positive likelihood ratio (LR+) of the CCQ as a case-detection tool for rheumatic diagnosis and for the most frequent diagnoses identified in the survey, osteoarthritis, regional rheumatic pain syndromes, and rheumatoid arthritis (RA). Logistic regression with the questions with LR+ ≥ 1 was performed to identify the strength of association (OR) for each question. Pain in the last 7 days, high pain score (> 4), and previous diagnosis were the questions with highest LR+ for diagnosis, and for diagnosis of RA treatment with NSAID. The variables that contributed most to the model were pain in the last 7 days (OR 2.0, 95% CI 1.8-2.3), NSAID treatment (OR 3.3, 95% CI 3.0-3.7), a high pain score (OR 1.15, 95% CI 1.13-1.17), and having a previous diagnosis (OR 1.4, 95% CI 1.3-1.6). These 4 questions had R(2) = 0.24, p < 0.01, for detection of any rheumatic diagnosis. The single variable that explains 16% (OR 1.33, 95% CI 1.31-134) of variance was a high pain score in the last 7 days. Some variables were identified in the CCQ that could be combined in a brief version for case detection of rheumatic diseases in community surveys. The validity of this proposal has to be tested against the original version.

Transclival cerebrospinal fluid rhinorrhea as the initial presenting symptom of a tiny intradural chordoma

We report a patient with a tiny intradural clival chordoma, which was identified following presentation with cerebrospinal fluid (CSF) rhinorrhea as the initial symptom. The transclival dural defect and the intradural tumor were successfully localized by both radiological investigation and intraoperative endoscopic inspection. The tumor was totally resected and the CSF fistula was repaired by an endoscopic endonasal approach. The diagnosis, possible mechanisms and management of this rare condition are discussed. The role of endoscopy in identifying and treating the clival CSF rhinorrhea is emphasized. To our knowledge, this is the first report of a clival fistula secondary to a tiny intradural chordoma.

Primary intradural chordoma: report on three cases and review of the literature

Chordomas are rare malignant bone tumors of the skull base or sacrococcygeal region. They derive from notochordal remnants and usually have a chronic progressive course. Even rarer, intradural chordomas with a better biological behavior have also been reported. We present 3 further primary intradural extraosseous chordomas with a favorable clinical outcome. Two patients, a 38-year-old man and a 44-year-old woman, presented with neck pain. In these, intradural extraspinal tumors within intervertebral foramina were found. Both tumors were totally removed and the patients have been free of disease for 7 years and 1 year, respectively. The other patient, a 76-year-old man suffered from an unspecific gait disorder and diplopia as a result of a prepontine space-occupying lesion. In this case, only an incomplete tumor resection was possible but progression has not occurred for 5 years. Paraffin blocks from all cases were examined with classical histopathological stainings and immunohistochemistry for pancytokeratin, CK7, CK8/18, CK19, EMA, CEA, vimentin, S100, aktin, desmin, GFAP, CD117, PDGF-receptor alpha and beta, collagen-type-IV, p63, and Ki67. Fluorescent in situ hybridization was used to exclude EWS translocation. All cases showed the typical histological picture with physaliphorous cells in a myxoid matrix and the characteristic immunohistochemical profile with positivity for vimentin, pancytokeratin, CK19, EMA, and S100. Staining for P63 and type IV collagen was consistently negative. Myxoid extraskeletal chondrosarcoma was excluded by in-situ-hybridization of the EWS gene. Considering our cases in context with so far published literature, we conclude that intradural chordomas are rare and in this location usually have a better prognosis compared to classical intraosseous chordomas.

Intradural clival chordoma: a rare pathological entity

Chordomas constitute about 1% of intracranial tumors. They are generally extradural destroying the bone. Intradural chordomas are very rare. We present one such case of a clival intradural chordoma who presented with a left trigeminal nerve sensory neuropathy. The lesion was totally excised uneventfully via a retromastoid suboccipital approach. There was no recurrence of the lesion at 1-year follow-up. Characteristics of intradural chordoma and its close differential ecchordosis physaliphora are reviewed in this article.

INTRADURAL CLIVAL CHORDOMA AND ECCHORDOSIS PHYSALIPHORA

Purely intradural clival chordomas are rare neoplasms, and only a few cases have been reported. The reported cases present features similar to ecchordosis physaliphora, which is a notochordal remnant. We describe these 2 entities and their differential diagnoses, clinical courses, and management. This is the first reported case to be treated using a neuroendoscopic technique. A 60-year-old man presenting with memory loss underwent magnetic resonance imaging, which revealed an intradural retroclival mass without bone involvement. The patient underwent an endoscopic transsphenoidal-transclival procedure with subtotal removal of the tumor. Histological findings confirmed the diagnosis of a chordoma. Even if some parameters exist for a differential diagnosis, ecchordosis physaliphora and intradural chordoma may represent different aspects of the spectrum of the same pathology. Intradural clival chordomas have a better prognosis with respect to classic chordomas. Therefore, in subtotal removal such as that performed in our case, postoperative radiation therapy should be performed only if a regrowth of the remnant is seen during neuroradiological follow-up.

P.340 Plate-guided distractor for a segmental mandibular defect

To investigate the clinical and pathologic characteristics of primary intradural retroclival chordoma and improve the understanding of this rare disease.A retrospective study was conducted on six cases patients withof an intradural chordoma in the retroclival region who underwent surgery and were confirmed by pathology and imaging. Expression of brachyury, galectin-3, and Ki-67 in paraffin-embedded sections of the specimens was detected by streptavidin-peroxidase immunohistochemistry.Lesions were located in the subdural prepontine cistern. Computed tomography scan showed that the bone of the skull base was not destroyed, and findings on magnetic resonance imaging varied, resulting in a misdiagnosis of 50% of the cases in the preoperative imaging. All six cases were classified by their pathology into the classical subtype. They presented a strong positive staining for brachyury and galectin-3, and the Ki-67 labeling index was between 2.5% and 8.2%. Three cases presented no signs of recurrence or regrowth, whereas in the other three patients recurrence or regrowth occurred at 7∼14 months after initial surgery. Two patients died of this disease.Our study suggests that a positive staining for brachyury, galectin-3, and Ki-67 would be helpful for differential diagnosis, discriminating intradural retroclival chordoma from ecchordosis physaliphora and chordoid meningioma. Our study also shows that within intradural retroclival chordoma, there are significant prognostic differences. Tumors with an abundant blood supply, flake-like cellular arrangement, and a Ki-67 labeling index greater than 5% belong to a rapid-growth type and are prone to short-term recurrence and poorer prognosis.

P.341 Poorly differentiated carcinoma: Regaud's lymphoepithelioma

To investigate the clinical and pathologic characteristics of primary intradural retroclival chordoma and improve the understanding of this rare disease.A retrospective study was conducted on six cases patients withof an intradural chordoma in the retroclival region who underwent surgery and were confirmed by pathology and imaging. Expression of brachyury, galectin-3, and Ki-67 in paraffin-embedded sections of the specimens was detected by streptavidin-peroxidase immunohistochemistry.Lesions were located in the subdural prepontine cistern. Computed tomography scan showed that the bone of the skull base was not destroyed, and findings on magnetic resonance imaging varied, resulting in a misdiagnosis of 50% of the cases in the preoperative imaging. All six cases were classified by their pathology into the classical subtype. They presented a strong positive staining for brachyury and galectin-3, and the Ki-67 labeling index was between 2.5% and 8.2%. Three cases presented no signs of recurrence or regrowth, whereas in the other three patients recurrence or regrowth occurred at 7∼14 months after initial surgery. Two patients died of this disease.Our study suggests that a positive staining for brachyury, galectin-3, and Ki-67 would be helpful for differential diagnosis, discriminating intradural retroclival chordoma from ecchordosis physaliphora and chordoid meningioma. Our study also shows that within intradural retroclival chordoma, there are significant prognostic differences. Tumors with an abundant blood supply, flake-like cellular arrangement, and a Ki-67 labeling index greater than 5% belong to a rapid-growth type and are prone to short-term recurrence and poorer prognosis.