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Chang-Sheng Hsieh, Sang-Ho Lee, Hyung Chang Lee, Hyeong-Seok Oh, Byeong-Wook Hwang, Sang-Joon Park, and Jian-Han Chen

Congenital hypoplasia of the spinal pedicle is a rare condition. Previously reported cases were treated conservatively or with posterior instrumented fusion. However, the absence or hypoplasia of the lumbar pedicle may increase the difficulty of pedicle screw fixation and fusion. Herein, the authors describe 2 cases of rare adult congenital hypoplasia of the right lumbar pedicles associated with spondylolisthesis. The patients underwent anterior lumbar interbody fusion with a stand-alone cage as well as percutaneous pedicle screw fixation. This method was used to avoid the difficulties associated with pedicle screw fixation and to attain solid fusion. Both patients achieved satisfactory outcomes after a minimum of 2 years of follow-up. This method may be an alternative for patients with congenital hypoplasia of the lumbar spinal pedicle.

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Myung-Jin Park, In-Chul Park, Jin-Heang Hur, Mi-Suk Kim, Hyung-Chan Lee, Sang-Hyeok Woo, Kyung-Hee Lee, Chang-Hun Rhee, Seok-Il Hong, and Seung-Hoon Lee

Object. Expression of matrix metalloproteinases (MMPs) has been postulated to play a central role in brain tumor invasion; however, its underlying mechanism is not yet fully understood. In the present study, by assessing the effect of a specific p38 mitogen-activated protein kinase (MAPK) inhibitor, SB203580, on the secretion of MMPs and in vitro invasion of various glioma cells, the authors attempt to define the role of the p38 MAPK pathway in the regulation of MMPs and tissue inhibitors of metalloproteinases (TIMPs) activated by phorbol ester (phorbol-12-myristate-13-acetate [PMA]) in the D54 human glioblastoma cell line.

Methods. The activation of MAPKs was determined using Western blot analysis after addition of phospho-specific antibodies against these kinases, the status of MMPs and TIMPs was analyzed using gelatin zymography and Western blot analysis, and the invasion rate of D54 cells and other glioma cells was analyzed using a modified Boyden chamber assay. Treatment of D54 cells with PMA activated two distinct MAPKs, extracellular signal-regulated kinase (ERK) 1/2 and p38 MAPK, but not c-Jun N-terminal kinase/stress-activated protein kinase. Induction of MMP-9 production and MMP-2 activation by PMA were blocked by SB203580, a specific inhibitor of p38 MAPK, but not by PD98059, a specific inhibitor of ERK 1/2. In addition, PMA-induced downregulation of TIMP-1 and TIMP-2 secretion and upregulation of the membrane type 1 MMP, a major activator of MMP-2 on the cell surface, were reversed by SB203580 in these cells; the PMA-induced increase of invasion in vitro decreased when SB203580 was added to the top compartment of a modified Boyden chamber; and the inhibitor also reduced the MMP secretion and PMA-induced in vitro invasion in various glioma cell lines.

Conclusions. These results indicate that activation of p38 MAPK by PMA plays a central role in the regulation of MMPs and TIMPs in D54 cells, which has a major influence in tumor invasion and metastasis. Furthermore, inhibition of p38 MAPK by SB203580 blocked the secretion of MMPs and in vitro invasion of various glioma cells, underscoring a possible role of p38 MAPK inhibitors as antiinvasive and/or antimetastatic agents of malignant gliomas.

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Ju Hyung Moon, Won Seok Chang, Hyun Ho Jung, Kyu Sung Lee, Yong Gou Park, and Jong Hee Chang

Object

The aim of this study was to evaluate the tumor control rate and functional outcomes after Gamma Knife surgery (GKS) among patients with a facial nerve schwannoma.

Methods

The authors reviewed the radiological data and clinical records for 14 patients who had consecutively undergone GKS for a facial nerve schwannoma. Before GKS, 12 patients had facial palsy, 7 patients had hearing disturbance, and 5 patients had undergone partial or subtotal tumor resection. The mean and median tumor volumes were 3707 mm3 and 3000 mm3, respectively (range 117–10,100 mm3). The mean tumor margin dose was 13.2 Gy (range 12–15 Gy), and the mean maximum tumor dose was 26.4 Gy (range 24–30 Gy). The mean follow-up period was 80.7 months (range 2–170 months).

Results

Control of tumor growth was achieved in all 12 (100%) patients who were followed up for longer than 2 years. After GKS, facial nerve function improved in 2 patients, remained unchanged in 9 patients, and worsened in 3 patients. All patients who had had serviceable hearing at the preliminary examination maintained their hearing at a useful level after GKS. Other than mild tinnitus reported by 3 patients, no other major complications developed.

Conclusions

GKS for facial nerve schwannomas resulted in excellent tumor control rates and functional outcomes. GKS might be a good primary treatment option for patients with a small- to medium-sized facial nerve schwannoma when facial nerve function and hearing are relatively preserved.

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Kyoung Su Sung, Ju Hyung Moon, Eui Hyun Kim, Seok-Gu Kang, Se Hoon Kim, Chang-Ok Suh, Sun Ho Kim, Kyu-Sung Lee, Won Seok Chang, and Jong Hee Chang

OBJECTIVE

Solitary fibrous tumor/hemangiopericytoma (SFT/HPC) is a new combined entity for which a soft-tissue–type grading system, ranging from grades I to III, has been introduced in the 2016 WHO classification of tumors of the CNS. The results of the treatment of this new disease entity require evaluation.

METHODS

The authors retrospectively reevaluated the pathological findings and medical records of patients with SFT/HPC. This study included 60 patients (27 men and 33 women, median age 42.5 years, range 13–69 years) treated at Severance Hospital between February 1981 and February 2016. Four, 40, and 16 patients were categorized as having SFT/HPC grades I, II, and III, respectively. Among these patients, SFTs diagnosed in 7 patients were regraded as grades I (n = 4), II (n = 2), and III (n = 1).

RESULTS

The median overall survival (OS) was 73.2 months (range 1.4–275.7 months), and the progression-free survival (PFS) after the first operation was 53.8 months (range 1.4–217.7 months). Six patients (10%) showed extracranial metastasis during a median period of 103.7 months (range 31.9–182.3 months). Nineteen patients (31.7%) presented with tumor recurrences. The patients in the grade III group had shorter PFS and OS, as well as a shorter period to extracranial metastasis, compared with patients in the grade II group. In the grade II group, patients who underwent gross-total resection showed longer PFS than those who underwent subtotal resection; however, there was no difference in OS. Patients who underwent adjuvant radiation therapy (RT) after surgery had longer PFS compared with that of patients who did not undergo adjuvant RT.

CONCLUSIONS

The SFT/HPC grade I group showed a relatively benign course compared with those of the other groups. The grade III group presented a course with a more aggressive nature than that of the grade II group. In the grade II group, the extent of resection and adjuvant RT was significantly associated with longer PFS. The long-term follow-up and periodic systemic evaluation are mandatory to detect systemic metastasis.

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Hyeoncheol Hwang, Kyu-Chang Wang, Moon Suk Bang, Hyung-Ik Shin, Seung-Ki Kim, Ji Hoon Phi, Ji Yeoun Lee, Jinwoo Choi, Seungwoo Cha, and Keewon Kim

OBJECTIVE

The aim of this study was to establish optimal electric stimulation parameters for intraoperatively monitoring the bulbocavernosus reflexes (BCRs) in infants.

METHODS

The authors retrospectively reviewed the medical records of all infants (age < 24 months) who had undergone an untethering operation for tethered cord syndrome between May 2013 and February 2014 at a single institution and whose baseline BCR had been elicited during surgery. Using different combinations of stimulation parameters—number of stimulation pulses: 4 or 8 pulses, interpulse interval: 1, 2, or 5 msec, and polarity of stimulation: biphasic or monophasic—the authors compared the relative mean amplitude of 10 BCR responses (rmaBCRs) to each combination of parameters.

RESULTS

The rmaBCRs were larger with the 8-pulse stimulations than with the 4-pulse stimulations (p < 0.0001). There was a tendency, though not statistically significant, for larger rmaBCRs to be obtained with the longer interpulse interval in the 8-pulse stimulation (p = 0.1289). The biphasic stimulation produced larger rmaBCRs than the monophasic stimulation (p = 0.0005).

CONCLUSIONS

Biphasic 8-pulse stimulations with 5-msec or 2-msec intervals yield the largest BCR responses. Considering that an 8-pulse stimulation with 5-msec intervals may overlap the onset of the BCR, a biphasic 8-pulse stimulation with 2-msec intervals is recommended as the optimal stimulation paradigm to monitor intraoperative BCRs in infants.

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You Gyoung Yi, Keewon Kim, Hyung-Ik Shin, Moon Suk Bang, Hee-Soo Kim, Jinwoo Choi, Kyu-Chang Wang, Seung-Ki Kim, Ji Yeoun Lee, Ji Hoon Phi, and Han Gil Seo

OBJECTIVE

This study aimed to investigate the feasibility and safety of intraoperative motor evoked potential (MEP) monitoring in infants less than 3 months of age.

METHODS

The authors investigated 25 cases in which infants younger than 3 months (mean age 72.8 days, range 39–87) underwent neurosurgery between 2014 and 2017. Myogenic MEPs were obtained through transcranial electrical stimulation. In all cases, surgery was performed under total intravenous anesthesia, maintained with remifentanil and propofol.

RESULTS

MEPs were documented in 24 infants, the sole exception being 1 infant who was lethargic and had 4-limb weakness before surgery. The mean stimulation intensity maintained during monitoring was 596 ± 154 V (range 290–900 V). In 19 of 24 infants MEP signals remained at ≥ 50% of the baseline amplitude throughout the operation. Among 5 cases with a decrease in intraoperative MEP amplitude, the MEP signal was recovered in one during surgery, and in the other case a neurological examination could not be performed after surgery. In the other 3 cases, 2 infants had relevant postoperative weakness and the other did not show postoperative neurological deficits. Postoperative weakness was not observed in any of the 20 infants who had no deterioration (n = 19) or only temporary deterioration (n = 1) in MEP signal during surgery.

CONCLUSIONS

Transcranial electrical MEPs could be implemented during neurosurgery in infants between 1 and 3 months of age. Intraoperative MEP monitoring may be a safe adjunct for neurosurgical procedures in these very young patients.

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Doo-Sik Kong, Stephanie Ming Young, Chang-Ki Hong, Yoon-Duck Kim, Sang Duk Hong, Jung Won Choi, Ho Jun Seol, Jung-Il Lee, Hyung Jin Shin, Do-Hyun Nam, and Kyung In Woo

OBJECTIVE

Cranioorbital tumors are complex lesions that involve the deep orbit, floor of the frontal bone, and lesser and greater wing of the sphenoid bone. The purpose of this study was to describe the clinical and ophthalmological outcomes with an endoscopic transorbital approach (TOA) in the management of cranioorbital tumors involving the deep orbit and intracranial compartment.

METHODS

The authors performed endoscopic TOAs via the superior eyelid crease incision in 18 patients (16 TOA alone and 2 TOA combined with a simultaneous endonasal endoscopic resection) with cranioorbital tumors from September 2016 to November 2017. There were 12 patients with sphenoorbital meningiomas. Other lesions included osteosarcoma, plasmacytoma, sebaceous gland carcinoma, intraconal schwannoma, cystic teratoma, and fibrous dysplasia. Ten patients had primary lesions and 8 patients had recurrent tumors. Thirteen patients had intradural lesions, while 5 had only extradural lesions.

RESULTS

Of 18 patients, 7 patients underwent gross-total resection of the tumor and 7 patients underwent planned near-total resection of the tumor, leaving the cavernous sinus lesion. Subtotal resection was performed in 4 patients with recurrent tumors. There was no postoperative CSF leak requiring reconstruction surgery. Fourteen of 18 patients (77.8%) had preoperative proptosis on the ipsilateral side, and all 14 patients had improvement in exophthalmos; the mean proptosis reduced from 5.7 ± 2.7 mm to 1.5 ± 1.4 mm. However, some residual proptosis was evident in 9 of the 14 (64%). Ten of 18 patients (55.6%) had preoperative optic neuropathy, and 6 of them (60.0%) had improvement; the median best-corrected visual acuity improved from 20/100 to 20/40. Thirteen of 18 patients showed mild ptosis at an immediate postoperative examination, all of whom had a spontaneous and complete recovery of their ptosis during the follow-up period. Three of 7 patients showed improvement in extraocular motility after surgery.

CONCLUSIONS

Endoscopic TOA can be considered as an option in the management of cranioorbital tumors involving complex anatomical areas, with acceptable sequelae and morbidity.