Sergei Terterov

Predictors of recurrence following resection of intracranial chordomas

Management of intracranial chordomas remains challenging, despite improvements in microsurgical techniques and radiotherapy. Here, we analyzed the prognostic factors associated with improved rates of tumor control in patients with intracranial chordomas, who received either gross (GTR) or subtotal resections (STR). A retrospective review was performed to identify all patients who were undergoing resection of their intracranial chordomas at the Ronald Reagan University of California Los Angeles Medical Center from 1990 to 2011. In total, 57 patients undergoing 81 resections were included. There were 24 females and 33 males with a mean age of 44.6 years, and the mean tumor diameter was 3.36 cm. The extent of resection was not associated with recurrence. For all 81 operations, the 1 and 5 year progression free survival (PFS) was 87.5 and 40.4%, and 88.0 and 33.6% for STR and GTR, respectively (p=0.90). Adjuvant radiotherapy was associated with improved rates of PFS (hazard ratio [HR] 0.20; p=0.009). Additionally, age >45 years (HR 5.88; p=0.01) and the presence of visual deficits (HR 7.59; p=0.03) were associated with worse rates of tumor control. Tumor size, sex, tumor histology, and recurrent tumors were not predictors of recurrence. Younger age, lack of visual symptoms on presentation and adjuvant radiotherapy were associated with improved rates of tumor control following surgery. However, GTR and STR produced comparable rates of tumor control. The surgical management of intracranial chordomas should take a conservative approach, with the aim of maximal but safe cytoreductive resection with adjuvant radiation therapy, and a major focus on quality of life.

Adjuvant Stereotactic Radiosurgery and Radiation Therapy for the Treatment of Intracranial Chordomas.

Objective Chordomas are locally aggressive, highly recurrent tumors requiring adjuvant radiotherapy following resection for successful management. We retrospectively reviewed patients treated for intracranial chordomas with adjuvant stereotactic radiosurgery (SRS) and stereotactic radiation therapy (SRT). Methods A total of 57 patients underwent 83 treatments at the UCLA Medical Center between February 1990 and August 2011. Mean follow-up was 57.8 months. Mean tumor diameter was 3.36 cm. Overall, 8 and 34 patients received adjuvant SRS and SRT, and the mean maximal dose of radiation therapy was 1783.3 cGy and 6339 cGy, respectively. Results Overall rate of recurrence was 51.8%, and 1- and 5-year progression-free survival (PFS) was 88.2% and 35.2%, respectively. Gross total resection was achieved in 30.9% of patients. Adjuvant radiotherapy improved outcomes following subtotal resection (5-year PFS 62.5% versus 20.1%; p = 0.036). SRS and SRT produced comparable rates of tumor control (p = 0.28). Higher dose SRT (> 6,000 cGy) (p = 0.013) and younger age (< 45 years) (p = 0.03) was associated with improved rates of tumor control. Conclusion Adjuvant radiotherapy is critical following subtotal resection of intracranial chordomas. Adjuvant SRT and SRS were safe and improved PFS following subtotal resection. Higher total doses of SRT and younger patient age were associated with improved rates of tumor control.

Postcraniotomy superficial temporal artery pseudoaneurysm in the setting of triple H therapy: A case report and literature review

Background: Superficial temporal artery (STA) pseudoaneurysm after a craniotomy is very rare with only five cases reported in the literature, none manifesting in the setting of cerebral vasospasm treatment with triple H therapy. Case Description: A 31-year-old male was admitted after a syncopal episode. Imaging documented a ruptured anterior communicating artery aneurysm. He was taken to the operating room for aneurysm clipping, but the procedure was aborted due to intraoperative aneurysm re-rupture, at which point the patient underwent emergent coil embolization of the aneurysm. The postoperative course was complicated by severe cerebral vasospasm requiring prolonged triple H therapy. On postoperative day 22, a growing left temporal mass with a bruit was noted. The suspected diagnosis of STA pseudoaneurysm was confirmed by femoral angiography, and it was treated with coils and Onyx embolization. Conclusion: We report the first case of a postcraniotomy STA pseudoaneurysm in the setting of induced hypertension for the treatment of cerebral vasospasm. Endovascular embolization is a viable option for the treatment of an STA pseudoaneurysm.

Neurenteric cyst of the conus medullaris

Background: Neurenteric cysts (NECs) are rare developmental malformations of the central nervous system (CNS) which originate as benign congenital lesions. They originate from developmental foregut precursors, and are presumed to be the result of abnormal partitioning of the embryonic notochord plate. Such NECs predominantly arise in the cervical region in patients around 6 years of age or in their twenties or thirties. Notably, NECs of the conus medullaris are exceedingly rare, especially in patients of advanced age. Case Description: A 70-year-old male presented with bilateral upper thigh and leg pain of over 20 years duration. His pain worsened over the past 3 years, and he sought surgical management. Although his neurological exam was normal, the lumbar magnetic resonance imaging revealed an intradural, nonenhancing, thin-walled, cystic lesion at L1/conus medullaris. The lesion was successfully resected without any adverse sequelae. Conclusions: NECs are rare congenital legions that involve the spine. Here, an L1 intradural extramedullay neuroenteric cyst of the conus medullaris was resected without complications.

Simultaneous cerebrospinal fluid and hematologic metastases in a high-grade ependymoma

Background: Ependymomas are relatively uncommon tumors that constitute about 7% of all primary intracranial neoplasms. Among these, high-grade ependymomas are locally aggressive and recur most commonly at the primary site following resection. Ependymomas are also known to be the one glial neoplasm that tends to frequently metastasize inside and outside the central nervous system (CNS) that complicates workup and management. Metastasis due to surgical manipulation is common and neurosurgeons should be well-versed in the most effective methods to remove these tumors in order to avoid such metastases. Case Description: Here, we report a case of a 28-year-old female who initially presented with a parenchymal World Health Organization (WHO) grade III anaplastic ependymoma of the occipital lobe without metastasis. After multiple resections, the patient showed no evidence of disease recurrence for 2 years. During follow-up, new metastasis to the frontal lobe as well as to the lung were discovered 2 years after the initial surgery, without recurrence at the tumors primary site. Conclusions: While uncommon, this case demonstrates the possibility for ependymomas to metastasize via cerebrospinal fluid to other locations within the CNS and hematologically to extraneural locations without recurring locally.

Surgical nuances of partial sacrectomy for chordoma

Background: Sacral chordomas are rare, slow growing, locally aggressive tumors. Unfortunately, aggressive surgical resection is often associated with increased neurological morbidity. Methods: This technical note focuses on the utilization of partial sacrectomy for the resection of complex spinal chordomas. Results: The case presented documents the potential range of postoperative morbidity seen in patients undergoing partial sacrectomy for chordomas. Despite iatrogenic morbidity and tumor recurrence, with the cooperation of medical and surgical spine specialists, majority of patients can achieve good long-term outcomes. Conclusions: Sacral chordomas are rare lesions and pose a therapeutic challenge for spinal surgeons and oncologists. En-bloc surgical resection (e.g., partial sacrectomy) is the treatment of choice for these lesions, and the cooperation between subspecialists can lead to good neurologic outcomes, particularly if gross total resection is achieved.

Adult sacrococcygeal teratoma with coccygectomy: A case report with a review of the literature

Background: Saccrococcygeal teratomas (SCT) are derived from embryonic germ cell layers. They frequently present at the base of the coccyx within the pelvis. While these tumors are common in children, they are exceedingly rare in adults. In adults, a majority of these tumors are intrapelvic and associated with a low risk of malignant transformation. Therefore, this contributes to a good prognosis following resection of mostly benign lesions. Case Description: An adult female with chronic pelvic pain presented with a sacral teratoma. She failed conservative treatment and underwent a coccygectomy with an en-bloc excision of the tumor. Microscopic histological analysis showed no evidence of immature or malignant elements, confirming the diagnosis of a mature, benign, cystic SCT. Conclusions: Mature SCTs in adults are rare malignant lesions. In this case, the patient was cured following primary surgical excision requiring en-bloc coccygectomy.

Surgical nuances of circumferential lumbar spondylectomy: A case report and short literature review

Background: Chordomas are uncommon malignant bone tumors that are often minimally symptomatic for several years. By the time they are diagnosed, these lesions are typically large, involve major neural, bony, and vascular structures, and are no longer readily resectable. This leads to a high recurrence rate. Case Description: In this case report, we present a 67-year-old male with nonmechanical axial back pain, neurogenic claudication, and a large mass centered at the L3 level on magnetic resonance imaging consistent with a locally invasive chordoma. The patient underwent surgical resection that required a complete lumbar spondylectomy utilizing a three-stage approach, leading to incomplete tumor excision. The patients residual postoperative symptoms included paresthesias/numbness in the right anterior thigh and a partial (4/5) right-sided foot drop. At the time of discharge, there were plans for future proton beam therapy. Conclusions: Because of their relative resistance to chemotherapeutic agents, the optimal surgical management of chordomas is gross total en-bloc excision. Unfortunately, this is rarely feasible.

Poster 393 C5 Palsy After Cervical Laminectomy and Fusion: 2 Case Reports

Disclosures: Alexander Tucker: I Have No Relevant Financial Relationships To Disclose Case/Program Description: Proximal upper extremity weakness is a poorly understood complication of cervical spine surgery. The cause of this complication is unknown, although most patients recover completely over weeks to months with conservative treatment. We present two cases of postoperative, transient C5 palsy after laminectomy and fusion. Electrodiagnostic (EDX) studies performed after surgery revealed active denervation of the deltoid and biceps muscles on the symptomatic side, however the rhomboid muscles appeared unaffected. Setting: Tertiary care hospital. Results: By 6 months postoperatively, both patients had clinically improved strength in all previously affected muscle groups. We suggest that the current models of postoperative C5 palsy pathogenesis may be inadequate to explain our findings and propose an alternative theory of watershed ischemia distal to the origin of the dorsal scapular nerve as a contributing factor to the etiology of this surgical complication. Discussion: The cases described above illustrate postoperative C5 palsy, a notorious and under-recognized complication of cervical decompressive surgery, that was diagnosed clinically and correlated with EDX evidence. This description is the first to present the results of preoperative and postoperative electrophysiological studies; confirming deltoid and biceps weakness with rhomboid sparing. Conclusions: Serial EDX studies, as were performed on the patients presented here, may be used to assist with diagnosis, confirmation of involved root levels, prognostication, to follow recovery or to offer clues into the pathophysiology of the condition. Level of Evidence: Level V