clivus chordoma

INTRODUCTION: Skull base pathologies in the paediatric population are rare and require treatment by multiple qualified specialists. The endoscopic endonasal approach has revolutionized surgical treatment because it is less invasive than existing treatments.The goal of this study was to retrospectively review our experience with the reconstruction of paediatric skull middle base defects and associated complications. MATERIALS AND METHODS: We analysed medical records from patients aged ≤ 18 years who were treated at our centre between 2013 and 2021...

Craniocervical tumors lead to cervical pain, instability, and neurological symptoms, reducing the quality of life. Effective surgical intervention at the craniocervical junction (CCJ) is critical and complex, involving comprehensive approaches and advanced reconstructive techniques. This study, conducted at Mexico City's National Institute of Cancerology, focused on three surgical cases that occurred in 2023 involving tumors at the CCJ: two chordomas and one prostate adenocarcinoma. We utilized a specialized technique: clivus-cervical stabilization reinforced with a polymethylmethacrylate (PMMA)-filled cervical mesh...

BACKGROUND: Chordoma is a rare malignant neoplasm that predominantly arises from the axial skeleton, but can also develop in unusual locations. However, there are also rare cases of "NOS" chordoma involving the oropharyx and epithelial-myoepithelial carcinoma of the parotid gland in the same patient. According to contemporary research, chordoma is a rare malignant neoplasm that arises from the embryonic remnants of the notochord. and typically involves the clivus, sacrococcygeal bones or vertebrae...

INTRODUCTION: Chordomas are rare malignant neoplasms that can originate anywhere along the cerebrospinal axis. However, they are most commonly found in the spine, cranium, and sacrococcygeal region. Chordomas can manifest differently depending on their location and most symptoms are a result of local invasion. We present a rare case of intracranial clival chordoma that manifested as isolated unilateral cranial nerve XII (CN XII) palsy. PRESENTATION: A 53-year-old male presented to the neurosurgical clinic with headaches, dysarthria, and pharyngeal pain...

Chordoma is a rare tumor that arises from chordal tissue during fetal life. Recently, the concept of poorly differentiated chordoma, a subtype of chordoma characterized by loss of SMARCB1 / INI1 with a poorer prognosis than conventional chordomas, was established. It predominantly occurs in children and is rare in adults. Here, we report a rare adult case of poorly differentiated chordoma of the skull base with a unique course that rapidly systemically metastasized and had the shortest survival time of any adult chordoma reported to date...

Ecchordosis physaliphora (EP) is a rare benign lesion arising from embryonic notochordal remnants, typically located in the retroclival region. This case report presents a 46-year-old male patient experiencing intermittent headaches and occipital pain. Imaging revealed a well-defined, smoothly corticated bony lesion on the left side of the clivus, accompanied by a characteristic bony stalk devoid of any aggressive features. A review of the patient's medical records indicated stable imaging findings of the lesion over six years...

BACKGROUND: Non-chordomatous bony tumors of the clivus are extremely rare. Site, extent, and aggressiveness of tumor limits the extent of resection. It poses challenge to the neurosurgeons due to the complexity of anatomy. There is paucity of literature exclusively on non-chordomatous bone tumors of the clivus in young adults. OBJECTIVES: To analyze the clinical presentation, imaging findings, surgical approach, complications, and outcome of primary clival bony tumors in young adults...

BACKGROUND: Multicompartmental lesions of the anterior craniovertebral junction require aggressive management. However, the lesions can be difficult to reach, and the surgical procedure is difficult to understand. The aim of this study was to create a procedural, stepwise microsurgical educational resource for junior trainees to learn the surgical anatomy of the extreme lateral transodontoid approach (ELTOA). METHODS: Ten formalin-fixed, latex-injected cadaveric heads were dissected under an operative microscope...

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: Endoscopic endonasal far-medial approach provides an effective and safe corridor to access the parasagittal structures of the lower clivus such as the medial jugular tubercle (JT) and occipital condyle (OC) for lesions that displace neurovascular structures laterally. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: Parapharyngeal internal carotid arteries (ICAs) run posterolateral to the eustachian tubes and lateral to the OC...

INTRODUCTION: Skull-base chordomas are aggressive tumors with a propensity for recurrence/progression. Even with standard of care (SoC), 5-year recurrence rates are variable (19-54%). This high recurrence/progression rate correlate with increased morbidity and mortality. We sought to analyze a multi-center cohort of skull-base chordomas to identify predictors of progression in patients receiving SoC. METHODS: The [Blinded]-Neurosurgery data registry was queried for skull-base chordomas treated from 2008-2020...

INTRODUCTION: Despite their precarious behavioral classification (benign and low grade on histopathology yet behaviorally malignant), great strides have been taken to improve prognostication and treatment paradigms for patients with skull base chordoma. With respect to surgical techniques, lateral transcranial (TC) approaches have traditionally been used, however endoscopic endonasal approaches (EEA) have been advocated for midline lesions. Nonetheless, due to the rarity of this pathology (0...

BACKGROUND AND OBJECTIVES: The extradural anterior petrosal approach (EAPA) can present a challenge because it deals with critical structures in a narrow, confined corridor. It is associated with several potential approach-related risks including temporal lobe and venous injuries. Tentorial peeling has the potential to largely eliminate these risks during the approach and may offer more options for tailoring the dural opening to the anatomic region that one wants to expose. METHODS: Anatomic dissections of five adult injected non-formalin-fixed cadaveric heads were performed...

OBJECTIVE: The objective of this study was to clarify the detailed clinical course of recurrent clival chordoma and the outcomes of each treatment modality. METHODS: A single-center retrospective analysis was conducted on patients seen for recurrent clival chordoma. The cohort was identified from those who underwent surgery, stereotactic radiosurgery, or proton therapy at the authors' institution between 1990 and 2022. RESULTS: A total of 95 recurrences in 40 patients with a median (interquartile range [IQR]) follow-up of 43 (18-79) months were identified...

Intracranial primary chondrosarcomas are rare, accounting for <0.15% of all intracranial tumors, but exhibit a high risk of recurrence. Due to the rarity of this condition, it has proven difficult to establish efficacy-based treatment guidelines. The present study details a case of clivus chondrosarcoma exhibiting no recurrence following surgical resection using an endoscopic transsphenoidal approach and postoperative adjuvant radiotherapy. A 41-year-old female presented with primary symptoms of left eye esotropia, scotoma of the left nasal visual field and double vision...

Clival chordomas are rare but aggressive skull base tumors that pose significant treatment challenges and portend dismal prognosis. The aim of this study was to highlight the advantages and limitations of available treatments, to furnish prognostic indicators, and to shed light on novel therapeutic strategies. We conducted a retrospective study of clival chordomas that were surgically treated at our institution from 2003 to 2022; for comparison purposes, we provided a systematic review of published surgical series and, finally, we reviewed the most recent advancements in molecular research...

BACKGROUND: Chordomas are rare bone tumors originating from notochord remnants with a predilection for the median axis of the body, primarily in the sacrococcygeal spine, skull base, and cervical spine. This paper presents the first reported case of simultaneous multilevel chordomas with various enhancements. OBSERVATIONS: A 40-year-old woman presented with right-sided otalgia, headache, nasal obstruction, and facial pressure. The patient's head and spinal magnetic resonance imaging (MRI) demonstrated a midline lesion in the clivus, C2, and T1-5 spinal levels...

OBJECTIVE: Chordomas are rare tumors from notochordal remnants and account for 1%-4% of all primary bone malignancies, often arising from the clivus and sacrum. Despite margin-negative resection and postoperative radiotherapy, chordomas often recur. Further, immunohistochemical (IHC) markers have not been assessed as predictive of chordoma recurrence. The authors aimed to identify the IHC markers that are predictive of postoperative long-term (≥ 1 year) chordoma recurrence by using trained multiple tree-based machine learning (ML) algorithms...

OBJECTIVE: To compare outcomes of proton radiation therapy (PRT), stereotactic radiosurgery (SRS), and x-ray-based radiation with an SRS boost (XRT+SRS) for newly diagnosed clival chordoma. METHODS: The consecutive patients who had PRT or SRS in our facility were retrospectively reviewed. RESULTS: A total of 59 patients were identified (PRT, 36; SRS, 11; XRT+SRS, 12). The mean age (±SD) was 46 ± 20 years, with 54% being male. The mean tumor diameter (±SD) was 3...

The term ecchordosis physaliphora (EP) has been used historically to describe a benign notochordal remnant with no growth potential, most commonly occuring in the central clivus. Unfortunately, the radiologic appearance of EP overlaps considerably with the appearance of low-grade chordomas, which do have the potential for growth. In this article, we review new pathologic terminology that better describes this family of diseases, and we propose new radiologic terms that better address the uncertainty of the radiologic diagnosis...

INDICATIONS CORRIDOR AND LIMITS OF EXPOSURE: The extreme lateral approach is useful for both extradural and intradural anterior and anterolateral lesions at the lower clivus down to the level of C2. ANATOMIC ESSENTIALS NEED FOR PREOPERATIVE PLANNING AND ASSESSMENT: The patient is evaluated with MRI, computed tomography (CT), and an angiogram. Special attention is given to vascular (vertebral artery course, dominance, tumor feeders) and bony (occipital condyle, jugular tubercle, foramen magnum and extent of bony involvement) anatomy...