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Doo-Sik Kong, Do-Hyun Nam, Jung-Il Lee, Kwan Park and Jong Hyun Kim

Object

The authors conducted a retrospective study to evaluate the efficacy of Gamma Knife surgery (GKS) followed by radiotherapy for the treatment of unresectable glioblastomas multiforme (GBMs) on patient survival and quality of life.

Methods

A total of 19 patients with unresectable GBMs located in eloquent areas of the brain were eligible for this study. Beginning in January 2002, 10 patients underwent GKS followed by fractionated radiotherapy. Nine patients who had undergone radiotherapy alone after biopsy-proven diagnosis served as the control group. The mean patient ages were 53 years and 56 years, respectively. Preoperative Karnofsky Performance Scale (KPS) scores were 80 (range 60–100) and 90 (range 50–100), respectively. The median margin dose for GKS was 12 Gy (9–16 Gy), and the total dose for radiotherapy was 60 Gy in 30 fractions. The mean follow-up duration was 7.2 months, the median patient survival time was 52 weeks (95% confidence interval [CI] 22–110.6 weeks) in the GKS group, and the median overall survival time was 28 weeks (95% CI 22.5–33.5 weeks) in the control group. The difference was not statistically significant (p = 0.0758). The estimated progression-free survival rate at 3 months was 75% in the GKS group and 45% in the control group (p = 0.082). The posttreatment KPS scores were either unchanged or improved in the GKS group, whereas it deteriorated by 20 or more points in six of nine patients of the control group (p = 0.004).

Conclusions

Gamma Knife surgery prior to radiotherapy may be helpful in preserving patients' daily activities in the adjuvant management of unresectable GBM.

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Doo-Sik Kong, Do-Hyun Nam, Jung-Il Lee, Kwan Park, Jong Hyun Kim and Hyung Jin Shin

Object

It is important to differentiate growing teratoma syndrome (GTS) from tumor recurrence in the setting of an enlarging residual mass present after treatment of intracranial germ cell tumors (GCTs). The aim of this study was to determine the incidence of intracranial GTS and present its clinical manifestations in detail.

Methods

The authors performed a retrospective cohort study of 52 consecutive patients with newly diagnosed intracranial GCTs who presented between January 2000 and December 2006. The records were screened to identify a study cohort in which all patients had regrowing tumor mass despite normalization of tumor markers during or after treatment of GCTs.

Results

In 6 (11.5%) of 52 patients the pathological diagnosis was GTS. The median patient age at diagnosis was 14.5 years (range 2 months–17 years), and the primary tumors included 4 mixed GCTs and 2 immature teratomas. After second-look surgery, histological testing revealed the lesions to be mature teratoma in all patients. Three of 6 patients subsequently underwent radiation therapy and 1 patient received additional chemotherapy for spinal seeding.

Conclusions

In enlarging residual masses after treatment of intracranial GCTs, GTS should be kept in mind in the differential diagnosis of tumor recurrence especially if there is a radiographic mismatch with serum marker test results. If technically feasible, second-look surgery may be necessary for an accurate diagnosis.

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Taek-Kyun Nam, Jung-Il Lee, Young-Jo Jung, Yong-Seok Im, Hee-Ye An, Do-Hyun Nam, Kwan Park and Jong-Hyun Kim

Object. This study was performed to evaluate the role of gamma knife surgery (GKS) in patients with a large number (four or more) of metastatic brain lesions.

Methods. The authors retrospectively reviewed the outcome in 130 patients who underwent GKS for metastatic lesions. Eighty-four patients presented with one to three lesions (Group A) and 46 presented with four or more lesions (Group B). The overall median survival time after GKS was 35 weeks. The median survival time in Group A (48 weeks) was significantly longer (p = 0.005) than the survival time in Group B (26 weeks). The recursive partitioning analysis (RPA) class was the only significant prognostic factor identified in multivariate analysis. The median survival for patients in RPA Classes I, II, and III was 72, 48, and 19 weeks, respectively, in Group A and 36 and 13 weeks for Classes II and III in Group B. The number of lesions, tumor volume, whole brain radiotherapy, primary tumor site, age, and sex did not affect survival significantly.

Conclusions. It is suggested that GKS provides an increase in survival time even in patients with a large number (four or more) of metastatic lesions. Concerning the selection of patients for GKS, RPA class should be considered as the most important factor and multiplicity of the lesions alone should not be a reason for withholding GKS.

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Jung-Il Lee, Do-Hyun Nam, Jong Soo Kim, Seung-Chyul Hong, Hyung-Jin Shin, Kwan Park, Whan Eoh, Yeon-Lim Suh and Jong Hyun Kim

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Kyung Hwan Kim, So Jeong Kang, Jung-Won Choi, Doo-Sik Kong, Ho Jun Seol, Do-Hyun Nam and Jung-Il Lee

OBJECTIVE

This study aimed to verify the effect of proactive Gamma Knife surgery (GKS) in the treatment of asymptomatic meningioma compared with the natural course without any therapeutic intervention.

METHODS

From January 2006 to May 2017, 354 patients newly diagnosed with asymptomatic meningioma were reviewed and categorized into GKS (n = 153) and observation (n = 201) groups. Clinical and radiological progression rates were examined, and changes in volume were analyzed.

RESULTS

Clinical progression (i.e., clinician-judged progression), combining symptomatic progression (n = 43) and clinician-judged increase in size using images routinely acquired (n = 34), occurred in 4 patients (2.6%) and 73 patients (36.3%) in the GKS and observation groups, respectively (p < 0.001). The clinical progression-free survival (PFS) rates in the GKS and observation groups were 98.7% and 64.6%, respectively, at 5 years (p < 0.001), and 92.9% and 42.7%, respectively, at 10 years (p < 0.001). The radiological tumor control rate was 94.1% in the GKS group, and radiological progression was noted in 141 patients (70.1%) in the observation group. The radiological PFS rates in the GKS and observation groups were 94.4% and 38.5%, respectively, at 5 years (p < 0.001), and 88.5% and 7.9%, respectively, at 10 years (p < 0.001). Young age, absence of calcification, peritumoral edema, and high T2 signal intensity were correlated with clinical progression in the observation group. Volumetric analysis showed that untreated tumors gradually increased in size. However, GKS-treated tumors shrank gradually, although transient volume expansion was observed in the first 6 months. Adverse events developed in 26 of the 195 GKS-treated patients (13.3%), including 1 (0.5%) major event requiring microsurgery due to severe edema after GKS. Peritumoral edema was related to the development of adverse events (p = 0.004).

CONCLUSIONS

Asymptomatic meningioma is a benign disease; however, nearly two-thirds of patients experience tumor growth and one-third of untreated patients eventually require neurosurgical interventions during watchful waiting. GKS can control tumors clinically and radiologically with high probability. Although the risk of transient adverse events exists, proactive GKS may be a reasonable treatment option when there are no comorbidities limiting life expectancy.

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Kyung-Il Jo, Yong Seok Im, Doo-Sik Kong, Ho Jun Seol, Do-Hyun Nam, Yoon-Duck Kim and Jung-Il Lee

Object

The goal of this study was to investigate the safety and efficacy of multisession Gamma Knife surgery (GKS) in the treatment of benign orbital tumors.

Methods

Twenty-three patients who retained their vision despite having tumors touching their optic nerve were treated with multisession (4-fraction) GKS. The median tumor volume was 2800 mm3 (range 211–10,800 mm3), and the median cumulative margin dose was 20 Gy (range 18–22 Gy).

Results

The median clinical follow-up duration in these patients was 38 months (range 9–74 months). No patient experienced tumor progression in this study. In particular, a higher degree of tumor shrinkage was found in the 7 patients with cavernous hemangiomas than in patients with other types of lesions (p < 0.05). Of the 23 patients whose preoperative vision was preserved, 11 showed improvement in visual acuity and/or visual field and 12 showed stable visual acuity. No GKS-related adverse events were noted during or after treatment.

Conclusions

Multisession radiosurgery using the Gamma Knife may be a good strategy for tumors in direct contact with the optic nerve. A cumulative margin dose of up to 22 Gy delivered in 4 sessions is safe for preservation of visual function with a high probability of tumor control.

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Seung-Chyul Hong, Kwan-Soo Kang, Dae Won Seo, Seung Bong Hong, Munhyang Lee, Do-Hyun Nam, Jung-Il Lee, Jong Soo Kim, Hyung-Jin Shin, Kwan Park, Whan Eoh, Yeon-Lim Suh and Jong-Hyun Kim

Object. Surgical treatment of cortical dysplasia (CD) together with intractable seizures is challenging because both visualization and localization of the lesion are difficult, correlation with seizure foci requires comprehensive study, and the surgical outcomes reported thus far are unsatisfactory. The authors report their experience in the surgical treatment of CD classified according to a surgical point of view.

Methods. The definition of CD used in this study was a dysplastic lesion visible on magnetic resonance (MR) images or a lesion that, although not visible on MR images, was diagnosed as moderate-to-severe dysplasia by using pathological analysis. During the last 4.5 years, the authors treated 36 patients with intractable epilepsy accompanied by CD. They divided the 36 cases of CD into four characteristic groups: Group A, diffuse bilateral hemispheric dysplasia; Group B, diffuse lobar dysplasia; Group C, focal dysplasia; and Group D, a moderate to severe degree of CD with a normal appearance on MR images. All but one patient in Group C were monitored in the epilepsy monitoring unit by using subdural electrodes for seizure localization and functional mapping.

The incidence of CD among a cohort of 291 patients who had undergone epilepsy surgery at the authors' center during the study period was 12.4%. The mean age of the 36 patients was 21.3 years and the mean age at seizure onset was 8.5 years. The mean follow-up period was 26 months. Twenty-six patients (72.2%) belonged to Engel Class I or II (20 and six, respectively). There were five cases in Group A, nine in Group B, nine in Group C, and 13 in Group D. Patients in Groups A and B were significantly younger at seizure onset and had significantly poorer surgical outcomes compared with patients in Groups C and D (p < 0.05). If outcome is compared on the basis of the extent of removal of CD, patients in whom CD was completely removed had significantly better outcomes than those in whom CD was only partially removed (p < 0.001).

Conclusions. The authors conclude that intractable epilepsy accompanied by CD can be treated surgically using comprehensive preoperative approaches. Deliberate resective procedures aimed at complete removal of dysplastic tissue ensure excellent seizure control without permanent neurological deficit.

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Byung Sup Kim, Yuil Kim, Doo-Sik Kong, Do-Hyun Nam, Jung-Il Lee, Yeon-Lim Suh and Ho Jun Seol

OBJECTIVE

The authors conducted this retrospective study to investigate the clinical outcomes of intracranial solitary fibrous tumor (SFT) and hemangiopericytoma (HPC), defined according to the 2016 WHO classification of central nervous system (CNS) tumors.

METHODS

Histopathologically proven intracranial SFT and HPC cases treated in the period from June 1996 to September 2014 were retrospectively reviewed and analyzed. Two neuropathologists reviewed pathological slides and regraded the specimens according to the 2016 WHO classification. Factors associated with progression-free survival (PFS) and overall survival (OS) were statistically evaluated with uni- and multivariate analyses.

RESULTS

The records of 47 patients—10 with SFT, 33 with HPC, and 4 with anaplastic HPC—were reviewed. A malignant transition from conventional SFT to WHO grade III SFT/HPC was observed in 2 cases, and 13 HPC cases were assigned grade III SFT/HPC. Mean and median follow-ups were 114.6 and 94.7 months, respectively (range 7.1–366.7 months). Gross-total resection (GTR) was significantly associated with longer PFS and OS (p = 0.012 for both), and adjuvant radiation therapy versus no such therapy led to significantly longer PFS (p = 0.018). Extracranial metastases to the liver, bone, lung, spine, and kidney occurred in 10 patients (21.3%). Grade III SFT/HPC was strongly correlated with the development of extracranial metastases (p = 0.031).

CONCLUSIONS

The 2016 WHO classification of CNS tumors reflected the different types of pathological malignant progression and clinical outcomes better than prior classifications. Gross-total resection should be the primary treatment goal in patients with SFT/HPC, regardless of the pathological grade, and radiation can be administered as adjuvant therapy for patients with SFT/HPC that shows an aggressive phenotype or that is not treated with GTR.

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Yong Hwy Kim, Chiman Jeon, Young-Bem Se, Sang Duk Hong, Ho Jun Seol, Jung-II Lee, Chul-Kee Park, Dong Gyu Kim, Hee-Won Jung, Doo Hee Han, Do-Hyun Nam and Doo-Sik Kong

OBJECTIVE

The endoscopic endonasal approach for treating primary skull base malignancies involving the clivus is a formidable task. The authors hypothesized that tumor involvement of nearby critical anatomical structures creates hurdles to endoscopic gross-total resection (GTR). The aim of this study was to retrospectively review the clinical outcomes of patients who underwent an endoscopic endonasal approach to treat primary malignancies involving the clivus and to analyze prognostic factors for GTR.

METHODS

Between January 2009 and November 2015, 42 patients underwent the endoscopic endonasal approach for resection of primary skull base malignancies involving the clivus at 2 independent institutions. Clinical data; tumor locations within the clivus; and anatomical involvement of the cavernous or paraclival internal carotid artery, cisternal trigeminal nerve, hypoglossal canal, and dura mater were investigated to assess the extent of resection. Possible prognostic factors affecting GTR were also analyzed.

RESULTS

Of the 42 patients, 37 were diagnosed with chordomas and 5 were diagnosed with chondrosarcomas. The mean (± SD) preoperative tumor volume was 25.2 ± 30.5 cm3 (range 0.8–166.7 cm3). GTR was achieved in 28 patients (66.7%) and subtotal resection in 14 patients (33.3%). All tumors were classified as upper (n = 17), middle (n = 17), or lower (n = 8) clival tumors based on clival involvement, and as central (24 [57.1%]) or paramedian (18 [42.9%]) based on laterality of the tumor. Univariate analysis identified the tumor laterality (OR 6.25, 95% CI 1.51–25.86; p = 0.011) as significantly predictive of GTR. In addition, the laterality of the tumor was found to be a statistically significant predictor in multivariate analysis (OR 41.16, 95% CI 1.12–1512.65; p = 0.043).

CONCLUSIONS

An endoscopic endonasal approach can provide favorable clinical and surgical outcomes. However, the tumor laterality should be considered as a potential obstacle to total removal.

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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.