En-bloc resection of a chordoma in L3 by a combined open posterior and less invasive retroperitoneal approach: technical description and case report.

INTRODUCTION: To fulfill oncological criteria, extensive open anterior and posterior approaches are usually performed in the lumbar spine to obtain an appropriate en-bloc spondylectomy. It is commonly accepted that the price of a tumor-free margin includes such extensive incisions and soft-tissue damage, with consequent relevant blood loss and possible postoperative complications as delayed wound healing. In this article, a case of chordoma in L3 is presented, submitted to an oncologically appropriate en-bloc resection performed by an open posterior approach combined with a mini-retroperitoneal approach. The successful oncologic procedure was combined with a short and uneventful postoperative course. MATERIALS AND METHODS: The authors present the surgical technique and the possible challenges of minimally invasive anterior oncologic surgery as a contribution to a limited literature. RESULTS: Up to date, palliative care of single metastases has been the main setting in which anterior, minimally invasive surgery has been performed in the lumbar spine. The authors explained how, in selected cases, this approach can be performed in combination with an open posterior access for an oncologically appropriate treatment of a primary malignant tumor. CONCLUSION: Anterior, minimally invasive surgery can have a role in selected patients with primary malignant tumors of the lumbar spine. The surgical team should have extensive training both in oncologic and minimally invasive surgery.

Surgical Management of Skull Base and Spine Chordomas.

OPINION STATEMENT: Management of chordoma along the cranial-spinal axis is a major challenge for both skull base and spinal surgeons. Although chordoma remains a rare tumor, occurring in approximately 1 per 1 million individuals, its treatment poses several challenges. These tumors are generally poorly responsive to radiation and chemotherapy, leading to surgical resection as the mainstay of treatment. Due to anatomic constraints and unique challenges associated with each primary site of disease, gross total resection is often not feasible and is associated with high rates of morbidity. Additionally, chordoma is associated with high rates of recurrence due to the tumor's aggressive biologic features, and postoperative radiation is increasingly incorporated as a treatment option for these patients. Despite these challenges, modern-day surgical techniques in both skull base and spinal surgery have facilitated improved patient outcomes. For example, endoscopic endonasal techniques have become the mainstay in resection of skull base chordomas, improving the ability to achieve gross total resection, while reducing associated morbidity of open transfacial techniques. Resection of spinal chordomas has been facilitated by emerging techniques in preoperative imaging, intraoperative navigation, spinal reconstruction, and radiotherapy. Taken collectively, the treatment of chordoma affecting the skull base and spinal requires a multidisciplinary team of surgeons, radiation oncologists, and medical oncologists who specialize in the treatment of this challenging disease.

Aggressive rhabdoid meningioma with osseous, papillary and chordoma-like appearance.

Meningiomas are the most common primary intracranial tumors. They are usually benign and slowly growing; however, they may show histologically malignant features categorizing them into grade II or III of World Health Organization (WHO) classification. Rhabdoid meningioma (RM) is an uncommon meningioma variant categorized as WHO grade III. The clinical course of RM is determined by local recurrences, invasion of adjacent brain and/or dura, widespread leptomeningeal dissemination, remote metastases and fatal clinical outcome. Herein we report a case with recurrent aggressive left occipital parasagittal region RM in which the patient initially declined radiation treatment. The tumor was resected four times in 5 years. Histopathological examination revealed a rhabdoid meningioma with metaplastic, papillary and chordoid differentiation. Six months after her fourth operation the patient died of progressive disease. RM is a rare subtype of malignant meningioma and the role of different adjuvant therapeutic options are still unknown. Clinical presentation, radiological features and pathologic findings of this uncommon tumor are discussed.

Chordoma of the thoracic spine in an 89-year-old.

STUDY DESIGN: Case report and literature review. OBJECTIVE: Report the highly unusual presentation of thoracic spine chordoma in an 89-year-old and review existing literature as it impacts treatment in the elderly. Chordomas are infrequent tumors of the spine that commonly present during mid-adulthood at the spheno-occipital or sacrococcygeal junctions. The mobile spine is affected in 10-15% of cases but chordomas are extremely rare in the thoracic spine. Chordoma rarely enters the differential diagnosis of spinal tumors in elderly patients, for whom metastases and multiple myeloma are by far the most common. METHODS: A case report is detailed of an 89-year-old male presenting with incapacitating pain and early signs of thoracic myelopathy. A lytic, expanding lesion of the T10 vertebral body with epidural spinal cord compression was identified. In the absence of evidence of other primary tumor, a CT-guided needle biopsy revealed chordoma. A literature review of reported thoracic spine chordomas was also performed. RESULTS: An intralesional posterolateral resection and reconstruction was performed with good results and no recurrence at 13 months' follow-up. 30 reports of thoracic spine chordomas were identified in the literature since 1902. Mean age of presentation at 35.7 years is earlier than for most chordomas. The oldest previously reported patient was 68-year-old. Neurological impairment at presentation is rare with the usual presenting symptom being pain. Response to radiation and chemotherapy is limited. 5-year survival rates range from 50 to 60%. CONCLUSION: Chordoma is a highly uncommon epidural neoplasm of the thoracic spine and is vanishingly rare in elderly patients. This report documents the oldest reported patient with thoracic spine chordoma at 89-year-old. Their locally aggressive behavior typically prompts consideration of aggressive surgical resection. When tailored to the individual patient, such procedures are feasible without excessive morbidity even in elderly patients.

Surgical results of an endoscopic endonasal approach for clival chordomas.

BACKGROUND: The surgical approaches for clival chordomas remain controversial, although the extent of resection is one of the most important factors for long survival rates. Recently an endoscopic endonasal approach in good collaboration with otolaryngologists has attracted major attention as a surgical approach for clival chordomas. We describe our experience with the endoscopic endonasal approach and provide a review of the literature. METHODS: Between 2008 and 2011, six operations were performed via the endoscopic endonasal approach for clivus chordomas. The mean tumor size was 35 mm in diameter. The tumor location was mainly from the upper to middle clivus. The tumor extended into the cavernous sinus in five cases and intradurally in three cases. A binostril approach was performed in four cases, while a one nostril approach was performed in two cases. RESULTS: Gross total removal was achieved in three cases. The analysis of cases with incomplete resection suggested that residual tumors were observed epidurally and subdurally. The residual on the epidura was observed from the posterior clinoid to the posterior compartment of the cavernous sinus. On the other hand, the residual on the subdural was observed behind the upper part of the pituitary gland. There was no postoperative cerebrospinal fluid (CSF) leakage using vascularized nasoseptal flaps in any of the cases. CONCLUSIONS: The endoscopic endonasal transclival approach allows an appropriate extent of resection with acceptable complication rates in comparison with other approaches. In our series, the accomplishment of gross total removal was associated with the relationship between the tumors and surrounding structures, such as the pituitary gland and the cavernous portion of the intracranial carotid artery (ICA).

Management of Chordoma of the Sacrum and Mobile Spine.

➢: Chordomas account for 1% to 4% of primary tumors of the spine and sacrum. ➢: En bloc resection is the preferred surgical treatment for the management of chordomas. ➢: Proton beam radiation is increasingly being used as a postoperative radiation modality for the treatment of chordomas.

Efficacy of epidermal growth factor receptor targeting in advanced chordoma: case report and literature review.

BACKGROUND: Chordomas are very rare low-grade malignant bone tumors that arise from the embryonic rests of the notochord. They are characterized by slow growth and long history with frequent local relapses, and sometimes metastases. While chemotherapy is not efficient, imatinib has shown antitumor activity. CASE PRESENTATION: We report on a 76-year-old patient with EGFR-overexpressing advanced chordoma that progressed on imatinib and subsequently responded to erlotinib during 12 months. CONCLUSIONS: We report the fourth case of advanced chordoma treated with an EGFR inhibitor. We also review the literature concerning the rationale and potential of EGFR targeting in chordoma.

Extensive lumbar chordoma and unique reconstructive approach.

Chordomas are rare, malignant, low grade, primary bone tumours arising from notochord remnants. They are rarely located in the lumbar spine and consequently, the literature is lacking about the condition. There are very few case reports on the technical aspects of resection of extensive lesions such as our case. Treatment can be potentially curative only if aggressive surgical resection is utilised. We describe a case of an extensive lumbar chordoma, and demonstrate the possibility of performing a staged anterior and posterior total tumour resection with planned transgression in conjunction with an expandable cage, vascularised rib, fibula and latissimus dorsi grafts for the treatment and reconstruction of extensive spinal disease with this condition.

Dumbbell-shaped thoracic chondroid chordoma mimicking a neurinoma.

A 31-year-old woman was admitted to the hospital with paraparesis and pyramidal signs. Magnetic resonance imaging disclosed a homogeneously enhanced mass occupying the spinal canal at the T1-T2 level and extending to the apical pleural space through the right intervertebral foramen. Surgical resection was achieved using a laminectomy and complete facetectomy. Histological examination indicated chondroid chordoma. The patient received postoperative proton radio-therapy. Chondroid chordoma is a subtype of chordomas with better prognosis, and it may appear as a dumbbell-shaped tumor as previously described in classical chordomas.

Resection of a retropharyngeal craniovertebral junction chordoma through a posterior cervical approach.

STUDY DESIGN: This illustrative case report is designed to provide technical data regarding the use of a posterior approach to resect a retropharyngeal chordoma involving the craniovertebral junction. OBJECTIVE: The objective of this report is to emphasize the utility of the posterior approach when treating anterior tumors of the craniovertebral junction. SUMMARY OF BACKGROUND DATA: Traditionally, a transoral transpharyngeal or extended anterior approach was used to resect anterior tumors of the craniovertebral junction. These approaches have several limitations unique to these exposures, limitations not applicable to a posterior midline cervical approach. METHODS: A case report is provided that illustrates the use of a posterior cervical approach used to resect a retropharyngeal craniovertebral junction chordoma. RESULTS: Gross total resection of a retropharyngeal chordoma was achieved using a posterior cervical approach. Although local tumor recurrence did occur, this was resected and adjuvant radiotherapy prescribed. This resulted in an ongoing 4-year recurrence free survival. CONCLUSIONS: The posterior cervical midline exposure could be used to dissect and remove anterior retropharyngeal tumors, with minimal morbidity.

Upper cervical spine chordoma of C2-C3.

Chordoma is a rare low-grade malignant neoplasm derived from the remnants of the embryonic notochord. This locally invasive neoplasm is subject to recurrence after treatment. The median survival time is estimated to be 6.3 years. Various treatment approaches have been attempted, including radical excision, radiotherapy and chemotherapy. Treatment outcome is significantly influenced by the size and site of the chordoma. Recently, Imatinib, a molecular-targeted agent, has been shown to have antitumor activity in chordoma. Proton radiotherapy, stereotactic radiotherapy and intensity-modulated radiotherapy have also been used. Surgical treatment is still the primary choice for chordoma. It has become more aggressive in recent years, evolving from intralesional or partial excision to en bloc resection. However, upper cervical localizations make such en bloc resection in most cases not possible. We present and discuss the therapeutic challenges of a young female with large retropharyngeal chordoma who presented to our institution after conventional photon beam radiotherapy. This C2/3 tumor was classified IB according to the Enneking classification. It distributed to layers A-D and sectors 1-6 according to the Weinstein Boriani Biagini Classification. The left vertebral artery (VA) was encapsulated and displaced. One stage intralesional extracapsular tumor excision and reconstruction was achieved by combined bilateral high anterior cervical approaches and posterior approach. No recurrence or metastasis was observed 3 years after the operation. She returned to her previous occupation as office worker.

A brief review of chordomas: pathogenesis, prognostic factors and therapeutic targets.

Chordomas are rare but locally aggressive cancer, which originate from primitive notochord remnants. Guidelines have recently been proposed to include the option of choosing chordomas in different locations. However, there is still a great challenge in the modern management of chordomas, primarily due to the high recurrence rate and poor prognosis. On this basis, there is a high demand for new therapeutic approaches and reliable prognostic factors. Recent progress in studying the molecular basis of this specific type of cancer has deepened the understanding of this mechanism, which overall facilitates the discovery of specific biomarkers or indicators of the disease. It also gives rise to potential targeted therapies against chordomas as evidenced by the fact that some RTK inhibitors in a clinical context have been evaluated in relation to chordomas. This article summarizes these achievements including the studies relative to pathogenesis, prognostic factors, and targeted therapies for chordomas. The theme of existing problems is also mentioned, which would facilitate general future efforts in this field.

Cranial Chordoma: A New Preoperative Grading System.

BACKGROUND: Chordomas are rare but challenging neoplasms involving the skull base. A preoperative grading system will be useful to identify both areas for treatment and risk factors, and correlate to the degree of resection, complications, and recurrence. OBJECTIVE: To propose a new grading system for cranial chordomas designed by the senior author. Its purpose is to enable comparison of different tumors with a similar pathology to clivus chordoma, and statistically correlate with postoperative outcomes. METHODS: The numerical grading system included tumor size, site of the tumor, vascular encasement, intradural extension, brainstem invasion, and recurrence of the tumor either after surgery or radiotherapy with a range of 2 to 25 points; it was used in 42 patients with cranial chordoma. The grading system was correlated with number of operations for resection, degree of resection, number and type of complications, recurrence, and survival. RESULTS: We found 3 groups: low-risk 0 to 7 points, intermediate-risk 8 to 12 points, and high-risk ≥13 points in the grading system. The 3 groups were correlated with the following: extent of resection (partial, subtotal, or complete; P < .002); number of operative stages to achieve removal (P < .014); tumor recurrence (P = .03); postoperative Karnofsky Performance Status (P < .001); and with successful outcome (P = .005). The grading system itself correlated with the outcome (P = .005). CONCLUSION: The proposed chordoma grading system can help surgeons to predict the difficulty of the case and know which areas of the skull base will need attention to plan further therapy.

Clinical experience of intracranial chordoma - A systematic review and meta-analysis of the literature.

Chordomas are rare neoplasms of the axial skeleton, with a predilection for the clival region. The lack of data about intracranial chordomas has led to inconsistent reporting of their clinical behavior and management principles. We performed a systematic review and meta-analysis with the aim of reporting on clinical behavior and management in the literature. We performed an electronic search of the MEDLINE, EMBASE, LILACS, Web of Science, Cochrane, and CINAHL databases. We included studies of patients with intracranial chordoma that had undergone surgical management with 5-year data available. The weighted mean estimate of 5-year OS was 77.4% (95% CI, 72.9-81.7%) whilst the 5-year PFS estimate was 49.4% (95% CI, 41.6-57.3%). A wide variety of surgical approaches were utilised, with transcranial approaches being the commonest. Surgeons achieved gross total resection in 29.4% of cases (95% CI, 17.6-43.0%), Sub-total resection in 60.9% of cases (95% CI, 40.2-79.8%) and partial resection in 19.7% (95% CI; 11.9 to 28.9%). The total complication rate was 28.5% (95% CI, 18.4-39.8%), with the commonest being new cranial nerve palsy (weighted mean 11.1%, 95% CI, 7.11-15.7%). Our review demonstrates that a wide range of management options exist in the literature. These should be tailored to each patient based on factors including tumoral location, local involvement and capabilities of the surgical team. The outcomes of chordoma management have largely been stable with no improvement despite improvements in modern surgical techniques.

Intradural cranial chordoma: a rare presentation of an uncommon tumor. Surgical experience and review of the literature.

OBJECT: Intracranial intradural chordomas are extremely rare lesions, and only a few cases have been reported in the neurosurgical literature. The authors performed a retrospective analysis of cases treated at their institutions. They present an illustrative surgical scenario and discuss the published literature, pathogenesis, and histopathological features as well as available follow-up data on the clinical behavior of these intradural lesions. METHODS: The authors reviewed clinical, neuroimaging, operative and follow-up data from a series of 79 chordomas treated at their institutions over the last 17 years. They found that the tumors were confined exclusively to the intradural compartment in only three cases. Staining for MIB-1 was performed to support the differential diagnosis between ecchordosis physaliphora and intradural chordoma, and in all three patients the neoplastic nature of the lesions was confirmed. None of these three cases showed recurrence of the lesion at midterm follow up. CONCLUSIONS: Complete resection, followed by close clinical and neuroradiological follow up, is warranted in cases of intradural cranial chordoma.

Surgical Treatment of Skull Base Tumors, Extending to Craniovertebral Junction.

OBJECTIVE: To conduct a comparative analysis of 2 groups of patients with skull base chordomas extending onto the craniovertebral junction, who underwent surgical treatment using extracranial approaches with and without craniocervical fusion. METHODS: The study group included 29 patients with skull base chordomas, extending to the craniovertebral junction, who were operated on from 2000 to 2015. The patients underwent the following surgical treatment: posterior craniocervical fusion followed by tumor removal using transoral and combined transoral and transnasal approaches. The reference group included 21 patients with the same disease, who underwent tumor removal surgery using the transoral approach without craniocervical fusion. RESULTS: In the study group, in 27.5% of all cases (8 patients), the tumors were removed radically; in 65.5% of all cases (19 patients), the tumors were removed subtotally; and in 7% of all cases (2 patients), the tumors were removed partially. In the reference group, the extent of surgical radicality was as follows: radical, 0%; subtotal, 19% (4 cases); partial, 81% (17 cases). CONCLUSIONS: Use of the proposed surgical technique in clinical practice may help increase the radicality of tumor excisions, decrease the number of postoperative complications, accelerate the rehabilitation process, and increase the quality of life in patients with skull base tumors extending to the craniovertebral junction.

Endoscopic Endonasal Surgery for Tumors of the Cavernous Sinus: A Series of 234 Patients.

BACKGROUND: Cavernous sinus (CS) tumors often are considered inoperable. We present our experience with endoscopic endonasal surgery (EES) and compare the outcomes for different tumor. METHODS: EES (medial or lateral approach) was used in 234 patients with CS tumors. The cohort included 175 (75%) pituitary adenomas and 59 (25%) nonadenomatous lesions. RESULTS: Presenting symptoms were significantly different between the 2 groups, with cranial neuropathies occurring mainly in nonadenomas (P < 0.0001). The overall gross total tumor resection rate from the CS was 37.3% (37.1% in adenomas, 38.1% in non-adenomas). In total, preexisting cranial nerve (CN) dysfunction improved in 56.4% of the patients. After treatment completion (including radiation of residual tumor), 83.3% of acromegalic patients, 50% of prolactinomas and 33.3% of Cushing's disease, were in remission. Visual loss improved in 86.8% of adenomas and in 70.8% of nonadenomas. Intracavernous CN palsies improved in 77.3% of adenomas and 42.4% of nonadenomas. New permanent CN palsies occurred in 7 nonadenomas, which is significantly greater than in adenomas (P = 0.007). The leak rate of cerebrospinal fluid was 6.3% for adenomas and 11.9% for nonadenomas. Four patients suffered an internal carotid artery injury with no neurologic sequelae in 3 cases and 1 death (0.4%). CONCLUSIONS: EES provides an easily accessible midline corridor to the CS with equivalent or superior results to transcranial approaches in the management of select tumors. Symptomatology due to CS invasion is more likely to improve in pituitary adenomas and the rate of surgical complications is greater in nonadenomas. Using a team approach, the overall mortality due to vascular injury is low.

Extra-axial chordoma.

A chordoma which occurs as a primary tumour outside the axial skeleton is known as an extra-axial chordoma, parachordoma or chordoma periphericum. It is extremely rare and therefore survival, recurrence and the rates of metastasis are not known. Whilst few recurrences have been described, the extra-axial chordoma has the potential for late recurrence at up to 12 years. Metastases are even less frequent. We report the case of a 56-year-old woman who developed an extra-axial chordoma of the right thoracic wall in close relationship with the tenth rib. The tumour was completely removed and the prognosis is excellent.