clivus chordoma

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...

Chordoma is a slow-growing local invasive tumor with high mortality and recurrence rates after surgical resection. It can affect the clivus and sacrum and rarely involve the lumbar vertebra. There is limited literature research describing lumbar embolization before surgical resection in lumbar chordoma. Thus, this case report describes an atypical patient with chronic lower back pain who presented to the hospital for worsening pain. Radiological images show an aggressive focal lesion at the second lumbar spine extending into the posterior element...

BACKGROUND: Data on clinical outcomes for base of skull (BOS) chordomas in the pediatric population is limited. We report patient outcomes after surgery and proton radiotherapy (PRT). METHODS: Pediatric patients with BOS chordomas were treated with PRT or combined proton/photon approach (proton-based; for most, 80% proton/20%photon) at a single institution from 1981-2021. Endpoints of interest were overall survival (OS), disease-specific survival (DSS), progression-free survival (PFS), freedom from local recurrence (LC), and freedom from distant failure (DC)...

Clival chordomas are locally invasive midline skull base tumors arising from remnants of the primitive notochord. Intracranial vasculature and cranial nerve involvement of tumors in the paraclival region necessitates image guidance that provides accurate real-time feedback during resection. Several intraoperative image guidance modalities have been introduced as adjuncts to endoscopic endonasal surgery, including stereotactic neuronavigation, intraoperative ultrasound, intraoperative MRI, and intraoperative CT...

BACKGROUND: Ectopic pituitary adenoma (EPA) is defined as a special type of pituitary adenoma that originates outside of the sellar region, is extra- or intra-cranially located, and without connection to normal pituitary tissue. EPA is extremely rare, with most cases presented as case reports or small case series. Due to nonspecific symptoms and laboratory indicators, the preoperative diagnosis, treatment and management for EPA remain challenging. CASE PRESENTATION: Here, we report the imaging phenotype and pathological findings of a case of invasive EPA in a 47-year-old woman...

BACKGROUND: Surgical management of skull base chordomas has changed significantly in the past 2 decades, most notably with use of the endoscopic endonasal approach (EEA), although high quality outcome data using these modern approaches remain scarce. OBJECTIVE: To evaluate outcomes in a large series of patients treated by a single surgeon, using primarily the EEA. METHODS: Between 2006 and 2020, 68 patients with skull base chordoma underwent resection using mostly the EEA...

Leiomyomas are common lesions that are usually located in the genitourinary and gastrointestinal tracts. Primary leiomyomas at the skull base are uncommon. They are composed of well-differentiated smooth muscle cells without cellular atypia. The diagnosis of a leiomyoma has to be confirmed by immunohistochemistry. The tumor tissue is immunoreactive for SMA, S100 and cytokeratin. Leiomyomas mainly occur in immunocompromised patients. Most tumor tissues are positive for EBV. The presented case is that of a 56-year-old immunocompetent woman with a tumor on the clivus...

INTRODUCTION: Chordoma is a rare, aggressive tumor that is believed to originate from notochord remnants. It can occur anywhere from the clivus to the sacrum and often recurs even after resection and radiotherapy. We present a unique case that initially suggested a different pathology based on imaging and presentation but was found to be a chordoma on gross and pathological analysis. CASE PRESENTATION: An 11-year-old girl presented outpatient for scoliosis evaluation and was found to have what appeared to be a right L4 peripheral nerve sheath tumor on MRI, causing dextroconvex scoliosis...

OBJECTIVE: To assess the early outcomes of two types of proton therapy: passive scattering proton therapy (PSPT) and pencil beam proton therapy (PBPT). METHODS: The consecutive patients who had surgery in our facility were retrospectively reviewed. RESULTS: Thirty-two patients were identified (PBPT 22 patients [69%]). The mean (±standard deviation [SD]) tumor size was 3.8±1.8 cm, and the most common location was the upper clivus (41%)...

Chordoma is a rare malignant bone tumor that mainly occurs in the sacrum and the clivus/skull base. Surgical resection is the treatment of choice for chordoma, but the local recurrence rate is high with unsatisfactory prognosis. Compared with other common tumors, there is not much research and individualized treatment for chordoma, partly due to the rarity of the disease and the lack of appropriate disease models, which delay the discovery of therapeutic strategies. Recent advances in modern techniques have enabled gaining a better understanding of a number of rare diseases, including chordoma...

Chordomas are aggressive and invasive tumors that are notoriously famous for their recurrence and metastasis. They present with diverse manifestations, usually with lower cranial nerve involvement. Here, we present the first reported case of chondroid chordoma with femur metastasis. A 71-year-old lady presented to us with a headache and diplopia. MRI of the brain revealed an enhanced broad and destructive mass in the infrasellar region with complete destruction of the clivus, right cavernous sinus. She underwent multiple surgeries along with gamma knife and proton therapy...

Chordomas are a locally invasive, low-grade, CNS malignancy that are primarily found in the skull base, spine, and sacrum. They are thought to be derived from notochordal remnants and remain a significant clinical challenge due to their local invasiveness, resistance to chemoradiation, and difficulty in achieving a complete resection. Adjuvant therapy such as proton beam therapy is critical in preventing recurrence in patients who are at high risk, however this treatment is associated with increased risk of complication...

The management of clival chordoma in our group shifted around 2013 to mostly endoscopic, and proton beam was introduced for our multidisciplinary team. Consecutive patients who had surgical resection from 1987 to 2021 were reviewed. A total of 58 patients (39 patients after 2013) were analyzed. The mean tumor size was 3.7 cm, and the most common location was the upper clivus (43%). Compared to before 2013, after 2013, the endoscopic endonasal approach was more common (90%, p &lt; 0.001), and more gross or near total resections (64%, p = 0...

•EEA represents an ideal approach for loco-regional recurrent CCs.•EEA is well tolerated, with preservation of patients QoL.•EEA can be considered for patients with perspectives of adjuvant therapies.•Otherwise, EEA can be considered only in selected cases with a palliative aim.