Tag: Chordoma

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Endoscopic Endonasal Transclival Approach to the Jugular Tubercle

Neurosurgery 71[ONS Suppl 1]:ons146–ons159, 2012

The jugular tubercle is a rounded bony prominence that arises from the inferolateral margin of the clivus. In a previous publication, we described the surgical anatomy of the expanded endonasal approach to the jugular tubercle.

OBJECTIVE: To illustrate the translation of laboratory work to the operating room describing the anatomic and technical nuances of the endonasal approach to the jugular tubercle.

METHODS: We review the relevant surgical anatomy needed to perform an endonasal approach to the jugular tubercle, and we select 4 different lesions to illustrate the application of our laboratory findings.

RESULTS: In the first case, exposure and partial drilling of the jugular tubercle was critical to gain an adequate corridor to the meningioma, particularly to its inferolateral margin. This allowed for early devascularization, safe extracapsular dissection, and preservation of surrounding neurovascular structures. In addition, the jugular tubercle was hyperostotic and its resection, along with generous dural removal, provided a grade I Simpson tumor resection. In the second (chondrosarcoma) and third (chordoma) cases, the jugular tubercle was infiltrated by tumor, and consequently its complete resection was essential to achieve total tumor removal. In the last case, an unusual adrenocorticotropic hormone-secreting adenoma recurrence at the jugular tubercle region, the technical modification of the transclival approach presented here was successfully applied to achieve complete resection and Cushing disease remission.

CONCLUSION: The transjugular tubercle variant of the expanded endonasal transclival approach allows for direct access to ventrolateral lesions in the inferior clival/petroclival region with no cerebral or cerebellar retraction, or cranial nerve manipulation during the approach.

 

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Role of Cancer Stem Cells in Spine Tumors

Neurosurgery 71:117–125, 2012 DOI: 10.1227/NEU.0b013e3182532e71

The management of spinal column tumors continues to be a challenge for clinicians. The mechanisms of tumor recurrence after surgical intervention as well as resistance to radiation and chemotherapy continue to be elucidated. Furthermore, the pathophysiology of metastatic spread remains an area of active investigation.

There is a growing body of evidence pointing to the existence of a subset of tumor cells with high tumorigenic potential in many spine cancers that exhibit characteristics similar to those of stem cells. The ability to self-renew and differentiate into multiple lineages is the hallmark of stem cells, and tumor cells that exhibit these characteristics have been described as cancer stem cells (CSCs).

The mechanisms that allow nonmalignant stem cells to promote normal developmental programming by way of enhanced proliferation, promotion of angiogenesis, and increased motility may be used by CSCs to fuel carcinogenesis.

The purpose of this review is to discuss what is known about the role of CSCs in tumors of the osseous spine. First, this article reviews the fundamental concepts critical to understanding the role of CSCs with respect to chemoresistance, radioresistance, and metastatic disease. This discussion is followed by a review of what is known about the role of CSCs in the most common primary tumors of the osseous spine.

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En Bloc Resection of Sacral Chordomas Aided by Frameless Stereotactic Image Guidance

Neurosurgery 70[ONS Suppl 1]:ons82–ons88, 2012 DOI: 10.1227/NEU.0b013e31822dd958 

The most important predictor of survival for patients with sacral chordomas is an initial en bloc resection with negative margins. However, obtaining negative margins can be technically challenging. Intraoperative navigation may be helpful in attempting an excision with negative margins.

OBJECTIVE: This is the first report of partial sacrectomy guided by frameless stereotactic navigation.

METHODS: Three patients with a mean age of 58.7 years underwent en bloc resection of sacral chordomas aided by image guidance. Intraoperatively, the reference arc was clamped to the spinous process of L5 and the bony landmarks of S1 were used for registration. Subsequently, the drill was registered, allowing the osteotomy trajectory to be visualized in real time with reference to the patients’ anatomy and tumor location.

RESULTS: None of the patients had any intraoperative or postoperative complications. Two patients with smaller tumors (5 cm) had negative margins, whereas the third patient with an 11.5 cm tumor had marginal margins. With an average follow-up of 44 months, none of the patients have had a recurrence of the tumor.

CONCLUSION: The use of frameless stereotaxy during the en bloc resection of sacral tumors is safe and feasible. Frameless stereotactic navigation was a useful adjunct to preoperative imaging and to the surgeon’s anatomic knowledge. Image guidance was used during the osteotomies to decrease the likelihood of injury to vital adjacent structures or violation of the tumor capsule and to increase the likelihood that the appropriate surrounding tissue was resected to attempt a wide or marginal resection.


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Primary Vertebral Tumors: A Review of Epidemiologic, Histological and Imaging Findings, Part II: Locally Aggressive and Malignant Tumors

Neurosurgery 70:211–219, 2012 DOI: 10.1227/NEU.0b013e31822d5f17

This second part of a comprehensive review of primary vertebral tumors focuses on locally aggressive and malignant tumors. As discussed in the earlier part of the review, both benign and malignant types of these tumors affect the adult and the pediatric population, and an understanding of their subtleties may increase their effective resection. In this review, we discuss the epidemiologic, histological, and imaging features of the most common locally aggressive primary vertebral tumors (chordoma and giantcell tumor) and malignant tumors (chondrosarcoma, Ewing sarcoma, multiple myeloma or plasmacytoma, and osteosarcoma). The figures used for illustration are from operative patients of the senior authors (Z.L.G. and J.H.C.). Taken together, parts 1 and 2 of this article provide a thorough and illustrative review of primary vertebral tumors.

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Chordomas of the skull base and cervical spine: clinical outcomes associated with a multimodal surgical resection combined with proton-beam radiation in 40 patients

Neurosurg Rev DOI 10.1007/s10143-011-0334-5

Previous studies of chordoma have focused on either surgery, radiotherapy, or particular tumor locations. This paper reviewed the outcomes of surgery and proton radiotherapy with various tumor locations.

Between 2001 and 2008, 40 patients with chordomas of the skull base and cervical spine had surgery at our hospital. Most patients received proton therapy. Their clinical course was reviewed. Age, sex, tumor location, timing of surgery, extent of resection, and chondroid appearance were evaluated in regard to the progression-free survival (PFS) and overall survival (OS). The primary surgery (PS) group was analyzed independently. The extensive resection rate was 42.5%. Permanent neurological morbidity was seen in 3.8%. Radiotherapy was performed in 75% and the mean dose was 68.9 cobalt gray equivalents. The median followup was 56.5 months. The 5-year PFS and OS rates were 70% and 83.4%, respectively. Metastasis was seen in 12.5%. The tumor location at the cranio-cervical junction (CCJ) was associated with a lower PFS (P=0.007). In the PS group, a younger age and the CCJ location were related to a lower PFS (P=0.008 and P<0.001, respectively). The CCJ location was also related to a lower OS (P=0.043) and it was more common in young patients (P=0.002). Among the survivors, the median of the last Karnofsky Performance Scale score was 80 with 25.7% of patients experiencing an increase and 11.4% experiencing a decrease.

Multimodal surgery and proton therapy thus improved the chordoma treatment. The CCJ location and a younger age are risks for disease progression.

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Clival chordomas: clinical management, results, and complications in 71 patients

J Neurosurg 113:1059–1071, 2010. DOI: 10.3171/2009.9.JNS08596

Chordomas are rare malignant neoplasms arising predominantly at the sacrum and skull base. They are uniformly lethal unless treated with aggressive resection and proton beam irradiation. The authors present results of the surgical management of a large number of patients with clivus chordomas. Factors that influence the surgeon’s ability to achieve radical tumor resection are also evaluated.

Methods. Between 1991 and 2005, 71 patients with clivus chordomas underwent surgery. The average followup was 66 months (median 60 months, range 3–189 months). Sixty-five patients had complete records that were analyzed in the present report. Thirty-five percent of them had undergone surgery before being treated by the authors. They were evaluated with MR imaging and CT scanning and underwent surgery utilizing a variety of skull base techniques aimed at achieving radical excision. Many also underwent postoperative radiation, usually in the form of proton beam therapy. The patients were followed up with serial imaging at regular intervals as well as with neurological evaluation.

Results. Radical tumor resection was achieved in 58% of the group. The overall 5-year survival rate was 75%. Radical resection had a positive impact on survival. The ability to achieve radical resection was dependent on the preoperative tumor volume and the number of anatomical areas involved by the tumor. Cranial nerve impairment and CSF leakage were the most frequent postoperative complications.

Conclusions. Radical excision is the ideal surgical goal in the treatment of clival chordomas and can be achieved with reasonable risks. Several different surgical approaches may be necessary to accomplish this.

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Prognostic factors for long-term outcome of patients with surgical resection of skull base chordomas—106 cases review in one institution

Neurosurg Rev (2010) 33:451–456. DOI 10.1007/s10143-010-0273-6

Skull base chordoma are still challenging. Between May 1993 and June 2005, 106 consecutive patients with skull base chordoma underwent surgical removal at Skull Base Division of Neurosurgery, Beijing Tiantan Hospital, China. Retrospective analysis included medical charts and images.

The age of the patients ranged from 7 to 65 years old, with an average age of 35.6 years. Sixty patients were male; the other 46 were female (1.3:1). Follow up data were available in 79 cases ranging from 10 to 158 months (average 63.9 months) after operation.

The prognostic factors for recurrence and survival were analyzed with Kaplan‐Meier, Cox regression and t‐test. Overall, 1, 3, 5 and 10 years survival rates were 87.2%, 79.4%, 67.6% and 59.5% respectively. One, 3, 5 and 10 year recurrent rates were 19.1%, 34.7%, 52.9% and 88.3%, respectively.

The long term outcome of the skull base chordomas is poor. The previous radiotherapy or surgery, dedifferentiated pathology, and less tumor resection are risk factors for longterm survival and recurrence (p<0.05). Although there is no statistic significant role of tumor adherent to vital structure for outcome (p=0.051), it can not exclude its importance for favorable outcome.

Gender, age, tumor size and staging are not independent risk factors for outcome. Surgical technique leading to radical tumor resection with less morbidity is advocatory and beneficial for patients with skull base chordoma with long term outcome, if the tumor could be exposed and resected completely, the recurrence rate was very low for most benign chordomas.

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Endoscopic endonasal transclival resection of chordomas: operative technique, clinical outcome, and review of the literature

J Neurosurg 112:1061–1069, 2010. DOI: 10.3171/2009.7.JNS081504

Transcranial approaches to clival chordomas provide a circuitous route to the site of origin of the tumor often involving extensive bone drilling and brain retraction, which places critical neurovascular structures between the surgeon and pathology. For certain chordomas, the endonasal endoscopic transclival approach is a novel minimal access, but it is an equally aggressive alternative providing the most direct route to the tumor epicenter.

Methods. The authors present a consecutive series of patients undergoing endonasal endoscopic resection of clival chordomas. Extent of resection was determined by postoperative volumetric MR imaging and divided into > 95% and < 95%.

Results. Seven patients underwent 10 operations. Preoperative cranial neuropathies were present in 4. The mean patient age was 52.0 years. The mean tumor volume was 34.9 cm3. Intraoperative lumbar drainage was used in 1 patient, and the tumors extended intradurally in 3. One patient underwent 2 intentionally palliative procedures for subtotal debulking. Greater than 95% resection was achieved in 7 of 8 operations in which radical resection was the goal (87%). All tumors with volumes < 50 cm3 had > 95% resection (p = 0.05). The overall mean follow-up was 18.0 months. Cranial neuropathies resolved in all 3 patients with cranial nerve VI palsies. One patient with recurrent nasopharyngeal chordoma died of disease progression; another experienced 2 recurrences before receiving radiation therapy. All surviving patients remain progression free. There were no intraoperative complications; however, 1 patient developed a pulmonary embolus postoperatively. There were no postoperative CSF leaks.

Conclusions. The endonasal endoscopic transclival approach represents a less invasive and more direct approach than a transcranial approach to treat certain moderate-sized midline skull base chordomas. Longer follow-up is necessary to determine comparability to transcranial approaches for long-term control. Large tumors with significant extension lateral to the carotid artery may not be suitable for this approach.

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Outcome of 132 Operations in 97 Patients With Chordomas of the Craniocervical Junction and Upper Cervical Spine

Neurosurgery 66:59-65, 2010 DOI: 10.1227/01.NEU.0000362000.35742.3D

OBJECTIVE: To study the outcomes of surgery for chordomas of the craniocervical junction and upper cervical spine as well as complication rates, survival, and associated adverse factors.

METHODS: Retrospective review of patients (1982-2007) at 2 European centers who underwent transoral, transfacial, transmandibular, and anterior cervical approaches for excision of chordomas of the craniocervical junction and cervical spine. The χ2 test and Fisher exact test were used to determine significant adverse factors (P < .05), and log-rank survival analysis was used to compare outcome in different groups.

RESULTS: One hundred thirty-two operations were performed in 97 patients. The most common operations were transoral surgeries and maxillotomies. After surgery, neck pain was the same or better in 98.1% of patients. Of the 18.6% of patients who presented with myelopathy, 27.8% improved, 44.4% remained unchanged, and 27.8% deteriorated. Major complication rates were velopharyngeal incompetence, 3.1%; vertebral artery stroke, 1%; wound infection, 3.1%; dysphagia, 3.1%; failure of fixation, 2.1%; sepsis, 3.1%; meningitis, 3.1%; and cerebrospinal fluid leakage, 6.2%. Five- and 10-year overall survivals were 55% and 36%, respectively. Patients who presented to our units for revision surgery, after prior attempts at resection elsewhere, were associated with a worse survival than patients who underwent de novo surgery.

CONCLUSION: We present, to our knowledge, the largest published series of chordomas at the craniocervical junction. Complication rates for these major operations can be minimized at specialist centers, with careful patient selection and counseling. As complete or as radical an operation as possible should be performed at first presentation; the best chance for the patient is the first chance.

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