Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children under the Age of 3 Years / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

Atypical Teratoid/Rhabdoid Tumor of the Central Nervous System in Children under the Age of 3 Years / Journal of the Korean Cancer Association, 대한암학회지

Purpose@#Atypical teratoid/rhabdoid tumor (ATRT) is a highly aggressive malignancy with peak incidence in children aged less than 3 years. Standard treatment for central nervous system ATRT in children under the age of 3 years have not been established yet. The objective of this study was to analyze characteristics and clinical outcomes of ATRT in children aged less than 3 years. @*Materials and Methods@#A search of medical records from seven centers was performed between January 2005 and December 2016. @*Results@#Forty-three patients were enrolled. With a median follow-up of 90 months, 27 patients (64.3%) showed at least one episode of disease progression (PD). The first date of PD was at 160 days after diagnosis. The 1- and 3-year progression-free survivals (PFS) were 51.2% and 28.5%, respectively. The 1- and 3-year overall survivals were 61.9% and 38.1%, respectively. The 3-year PFS was improved from 0% in pre-2011 to 47.4% in post-2011. Excluding one patient who did not receive any further therapy after surgery, 27 patients died due to PD (n=21), treatment-related toxicity (n=5), or unknown cause (n=1). In univariate analysis, factors associated with higher 3-year PFS were no metastases, diagnosis after 2011, early adjuvant radiotherapy, and high-dose chemotherapy (HDCT). In multivariate analysis, the use of HDCT and adjuvant radiotherapy remained significant prognostic factors for PFS (both p < 0.01). @*Conclusion@#Aggressive therapy including early adjuvant radiotherapy and HDCT could be considered to improve outcomes of ATRT in children under the age of 3 years.

Pediatric Glioma at the Optic Pathway and Thalamus

Gliomas are the most common pediatric tumors of the central nervous system. In this review, we discuss the clinical features, treatment paradigms, and evolving concepts related to two types of pediatric gliomas affecting two main locations: the optic pathway and thalamus. In particular, we discuss recently revised pathologic classification, which adopting molecular parameter. We believe that our review contribute to the readers' better understanding of pediatric glioma because pediatric glioma differs in many ways from adult glioma according to the newest advances in molecular characterization of this tumor. A better understanding of current and evolving issues in pediatric glioma is needed to ensure effective management decision.

Pediatric Glioma at the Optic Pathway and Thalamus

Gliomas are the most common pediatric tumors of the central nervous system. In this review, we discuss the clinical features, treatment paradigms, and evolving concepts related to two types of pediatric gliomas affecting two main locations: the optic pathway and thalamus. In particular, we discuss recently revised pathologic classification, which adopting molecular parameter. We believe that our review contribute to the readers' better understanding of pediatric glioma because pediatric glioma differs in many ways from adult glioma according to the newest advances in molecular characterization of this tumor. A better understanding of current and evolving issues in pediatric glioma is needed to ensure effective management decision.

Advanced MRI for Pediatric Brain Tumors with Emphasis on Clinical Benefits

Conventional anatomic brain MRI is often limited in evaluating pediatric brain tumors, the most common solid tumors and a leading cause of death in children. Advanced brain MRI techniques have great potential to improve diagnostic performance in children with brain tumors and overcome diagnostic pitfalls resulting from diverse tumor pathologies as well as nonspecific or overlapped imaging findings. Advanced MRI techniques used for evaluating pediatric brain tumors include diffusion-weighted imaging, diffusion tensor imaging, functional MRI, perfusion imaging, spectroscopy, susceptibility-weighted imaging, and chemical exchange saturation transfer imaging. Because pediatric brain tumors differ from adult counterparts in various aspects, MRI protocols should be designed to achieve maximal clinical benefits in pediatric brain tumors. In this study, we review advanced MRI techniques and interpretation algorithms for pediatric brain tumors.

Genetic and clinical characteristics of Korean patients with neurofibromatosis type 2

PURPOSE: Neurofibromatosis type 2 (NF2) is characterized by multiple tumors, including vestibular schwannoma (VS) and others affecting cranial and peripheral nerves. NF2 is caused by mutation of the NF2 gene. The mutation spectrum of NF2 has not been characterized in Korean patients. In the current study, the clinical and genetic characteristics of Korean NF2 patients were analyzed. MATERIALS AND METHODS: Twenty-five unrelated Korean families were enrolled according to the Manchester criteria. Genetic analysis was performed by direct sequencing and multiplex ligation-dependent probe amplification methods using genomic DNA from peripheral lymphocytes or tumor tissues. RESULTS: All patients had bilateral/unilateral VS and/or other cranial and peripheral nerve tumors. Two patients were familial cases and the other 24 patients were sporadic. Germline NF2 mutations were detected in peripheral lymphocytes from both familial cases, but only in 26.1% of the 23 sporadic families. Somatic mutations were also found in tumor tissues from two of the sporadic families. These somatic mutations were not found in peripheral lymphocytes. A total of 10 different mutations including 2 novel mutations were found in 40.0% of studied families. Five mutations (50.0%) were located in exon 6 of NF2, the FERM domain coding region. CONCLUSION: Family history was an important factor in identifying germline NF2 mutations. Further study is required to investigate whether exon 6 is a mutation hotspot in Korean NF2 patients and its correlation to phenotypic severity.

Neurocognitive Function and Health-Related Quality of Life in Pediatric Korean Survivors of Medulloblastoma

The neurocognitive function and quality of life of 58 Korean survivors of childhood medulloblastoma were assessed after surgery, cranial radiation and chemotherapy. All patients were evaluated with a battery of neurocognitive function tests and the Pediatric Functional Assessment of Cancer Therapy-Brain Tumor Survivors, which consists of self-report questionnaires on quality of life. The mean full-scale intelligence quotient (IQ), verbal IQ, and performance IQ scores were 90.2, 97.1, and 84.16, respectively. The mean memory quotient (MQ) score was 86.78, which was within 1 standard deviation of the average score of 100. Processing speed, attention, and executive function showed mild to moderate deficits. Intelligence, memory, executive function, visuospatial function, and simple motor function were significantly lower in the patients diagnosed before 8 years of age compared with those diagnosed after 8. The cognitive deficits in the patients diagnosed at younger ages might be related to earlier exposure to craniospinal irradiation and chemotherapy. The patient and parent proxy evaluations of attention, fine motor function, and quality of life did not differ. We found significant neurocognitive changes in a wide range of neurocognitive functional domains in Korean survivors of childhood medulloblastoma. Long-term follow-up studies of survivors of childhood medulloblastoma beginning at the time of their first diagnosis are required to better understand the deficits exhibited by survivors of childhood medulloblastoma, so that intervention strategies and treatment refinements that reduce the long-term neurocognitive decline can be developed.

Prognostic Factors and Therapeutic Outcomes in 22 Patients with Pleomorphic Xanthoastrocytoma

OBJECTIVE: Pleomorphic xanthoastrocytoma (PXA) is a rare primary low-grade astrocytic tumor classified as WHO II. It is generally benign, but disease progression and malignant transformation have been reported. Prognostic factors for PXA and optimal therapies are not well known. METHODS: The study period was January 2000 to March 2012. Data on MR findings, histology, surgical extents and adjuvant therapies were reviewed in twenty-two patients diagnosed with PXA. RESULTS: The frequent symptoms of PXA included seizures, headaches and neurologic deficits. Tumors were most common in the temporal lobe followed by frontal, parietal and occipital lobes. One patient who died from immediate post-operative complications was excluded from the statistical analysis. Of the remaining 21 patients, 3 (14%) died and 7 (33%) showed disease progression. Atypical tumor location (p<0.001), peritumoral edema (p=0.022) and large tumor size (p=0.048) were correlated with disease progression, however, Ki-67 index and necrosis were not statistically significant. Disease progression occurred in three (21%) of 14 patients who underwent GTR, compared with 4 (57%) of 7 patients who did not undergo GTR, however, it was not statistically significant. Ten patients received adjuvant radiotherapy and the tumors were controlled in 5 of these patients. CONCLUSION: The prognosis for PXA is good; in our patients overall survival was 84%, and event-free survival was 59% at 3 years. Atypical tumor location, peritumoral edema and large tumor size are significantly correlated with disease progression. GTR may provide prolonged disease control, and adjuvant radiotherapy may be beneficial, but further study is needed.

Clinical and Neuroimaging Outcomes of Surgically Treated Intracranial Cysts in 110 Children

OBJECTIVE: The indications and optimal surgical treatments for intracranial cysts are controversial. In the present study, we describe long-term clinical and neuroimaging results of surgically treated intracranial cysts in children. The goal of this study is to contribute to the discussion of the debate. METHODS: This study included 110 pediatric patients that underwent surgeries to treat intracranial cysts. Endoscopic cyst fenestrations were performed in 71 cases, while craniotomies and cyst excisions (with or without fenestrations) were performed in 30 patients. Cystoperitoneal shunts were necessary for nine patients. Long-term results were retrospectively assessed with medical and neuroimaging records. RESULTS: Clinical and radiological improvement was reported in 87.3% and 92.8% of cases, respectively, after endoscopic neurosurgery, and in 93.3% and 100% using open microsurgery whereas 88.9% and 85.7% after shunt operation. There were no statistical differences in clinical outcomes (p=0.710) or volume reductions (p=0.177) among the different surgeries. There were no mortalities or permanent morbidities, but complications such as shunt malfunctions, infections, and subdural hematomas were observed in 56% of the patients that had shunt operations. A total of 13 patients (11.8%) underwent additional surgeries due to recurrences or treatment failures. The type of surgery performed did not influence the recurrence rate (p=0.662) or the failure rate (p=0.247). CONCLUSION: Endoscopic neurosurgeries are less invasive than microsurgeries and are at least as effective as open surgeries. Thus, given the advantages and complications of these surgical techniques, we suggest that endoscopic fenestration should be the first treatment attempted in children with intracranial cysts.

Validation of Pediatric Functional Assessment of Cancer Therapy Questionnaire (Version 2.0) in Brain Tumor Survivor Aged 13 Years and Older (Parent Form)(PedsFACT-BrS Parent of Adolescent)

OBJECTIVE: The aim of this study was to evaluate the reliability and validity of the Pediatric Functional Assessment of Cancer Therapy Questionnaire Brain Tumor Survivor (version 2.0) Aged 13 years and older (Parent Form) (pedsFACT-BrS parent of adolescent). METHODS: The pedsFACT-BrS parent of adolescent was translated and cross-culturally adapted into Korean, following standard Functional Assessment of Chronic Illness Therapy (FACIT) methodology. The psychometric properties of the pedsFACT-BrS parent of adolescent were evaluated in 170 brain tumor patient's mothers (mean age=43.38 years). Pretesting was performed in 30 mothers, and the results indicated good symptom coverage and overall comprehensibility. The participants also completed the Child Health Questionnaire Parent Form 50 (CHQ-PF-50), Neuroticism in Eysenck Personality Questionnaire, and Karnofsky score. RESULTS: In validating the pedsFACT-BrS parent of adolescent, we found high internal consistency, with Cronbach's alpha coefficients ranging from 0.76 to 0.94. The assessment of test-retest reliability using intraclass correlation coefficient revealed satisfactory values with ICCs ranging from 0.84 to 0.93. The pedsFACT-BrS for parent of adolescent also demonstrated good convergent and divergent validities when correlated with the Child Health Questionnaire Parent Form 50 (CHQ-PF-50) and the Neuroticism in Eysenck Personality Questionnaire. The pedsFACT-BrS parent of adolescent showed good clinical validity, and effectively differentiated between clinically distinct patient groups according to the type of treatment, tumor location, shunt, and Karnofsky score of parent proxy report. CONCLUSION: We confirmed that this reliable and valid instrument can be used to properly evaluate the quality of life of Korean adolescent brain tumor patients by their parents' proxy report.

Reduced-dose craniospinal radiotherapy followed by high-dose chemotherapy and autologous stem cell rescue for children with newly diagnosed high-risk medulloblastoma or supratentorial primitive neuroectodermal tumor / 대한혈액학회지

BACKGROUND: In this study, we investigated the effects of reduced-dose craniospinal radiotherapy (CSRT) followed by tandem high-dose chemotherapy (HDCT) with autologous stem cell rescue (ASCR) in children with a newly diagnosed high-risk medulloblastoma (MB) or supratentorial primitive neuroectodermal tumor (sPNET). METHODS: Between March 2005 and April 2007, patients older than 3 years with a newly diagnosed high-risk MB or sPNET were enrolled. The patients received two cycles of pre-RT chemotherapy consisting of cisplatin, etoposide, vincristine, and cyclophosphamide (cycle A), and carboplatin, etoposide, vincristine, and ifosphamide (cycle B), followed by CSRT with 23.4 Gy and local RT with 30.6 Gy. After four cycles of post-RT chemotherapy (cycles A, B, A, and B), tandem double HDCT with ASCR was performed. RESULTS: A total of 13 patients (MB=11, sPNET=2) were enrolled. Of these, one patient progressed, one patient died of septic shock after the second cycle of B, and one patient relapsed after the third cycle of B. The 3-year event-free survival (EFS) rate of the patients intended for HDCT was 76.9%, whereas the 3-year EFS rate of the patients who received HDCT was 100%. No treatment-related mortality occurred during HDCT. CONCLUSION: Although the follow-up period was short and the patient cohort was small in size, the results of this study are encouraging. The limited toxicity and favorable EFS rate observed in children treated with reduced-dose CSRT followed by HDCT and ASCR warrant further exploration in a larger study population.

A Case of Primary Intraspinal Peripheral Primitive Neuroectodermal Tumor (pPNET) Mimicking Guillain-Barre Syndrome

Primary intraspinal peripheral primitive neuroectodermal tumors (pPNET) are extremely rare. We report a 10-month-old girl presented with rapidly progressive paraparesis and raised cerebrospinal fluid protein. A magnetic resonance image demonstrated an intramedullary mass from C3 to T3 level and intradural extramedullary nodules in the lumbosacral area. Surgery was performed with partial tumor removal and histologic examination revealed a small round cell tumor and immunohistochemical characteristics of pPNET. She died due to tumor progression at four months after initial diagnosis. This case has been reported to raise awareness among clinicians to include the possibility of intraspinal tumors in the differential diagnosis of progressive neurological deficits mimicking Guillain-Barre syndrome.

A Case of Spinal Cord Cavernoma Mimicking Transverse Myelitis

A 14-year-old girl presented with recurrent, progressive lower leg weakness and decrease in sensation. When the symptoms first appeared, she was misdiagnosed as transverse myelitis and was treated with methylprednisolone. Eleven months later, when she had the same symptoms, spine magnetic resonance images showed the characteristic findings of spinal cord cavernoma and she underwent surgical intervention. Spinal cord cavernomas are rare lesions. Because of their highly bleeding tendency, acute neurological deterioration occurs secondary to hemorrhage within the spinal cord. In order to prevent intramedullary hemorrhage and to halt the progressive neurological decline, surgical resection is recommended. We experienced a case of spinal cord cavernoma presenting with recurrent transverse myelitis clinically.

Comparative Analysis of Cervical Arthroplasty Using Mobi-C(R) and Anterior Cervical Discectomy and Husion Using the Solis(R) -Cage

OBJECTIVE: Although anterior cervical discectomy and fusion (ACDF) is the standard treatment for degenerative cervical disc disease, concerns regarding adjacent level degeneration and loss of motion have suggested that arthroplasty may be a better alternative. We have compared clinical and radiological results in patients with cervical disc herniations treated with arthroplasty and ACDF. METHODS: We evaluated 53 patients treated for cervical disc herniations with radiculopathy, 21 of whom underwent arthroplasty and 32 of whom underwent ACDF. Clinical results included the Visual Analogue Scale (VAS) score for upper extremity radiculopathy, neck disability index (NDI), duration of hospital stay and convalescence time. All patients were assessed radiologically by measuring cervical lordosis, segmental lordosis and segmental range-of-movement (ROM) of operated and adjacent disc levels. RESULTS: Mean hospital stay (5.62 vs. 6.26 days, p<0.05) and interval between surgery and return to work (1.10 vs. 2.92 weeks, p<0.05) were significantly shorter in the arthroplasty than in the fusion group. Mean NDI and extremity VAS score improved after 12 months in both groups. Although it was not significant, segmental ROM of adjacent levels was higher in the fusion group than in the arthroplasty group. And, segmental motion of operated levels in arthroplasty group maintained more than preoperative value at last follow up. CONCLUSION: Although clinical results were similar in the two groups, postoperative recovery was significantly shorter in the arthroplasty group. Although it was not significant, ROM of adjacent segments was less in the arthroplasty group. Motion of operated levels in arthroplasty group was preserved at last follow up.

The Change of Bladder Function after Neurosurgery in Patients with Lipomyelomeningocele / 대한비뇨기과학회지

PURPOSE: To prevent further neurological deterioration by tethering of the spinal cord, an early surgical procedure for asymptomatic lipomyelomeningocele (LMMC) can be performed. The neurogenic bladder patterns, degree of improvement according to timing of surgery, and the relationship between the change in the upper urinary tract and neurogenic bladder pattern were evaluated. MATERIALS AND METHODS: A retrospective chart review was performed on all 39 patients, before and after primary neurosurgical repair of LMMC, who presented between 1996 and 2003. RESULTS: Preoperative urodynamics (UDS) revealed a neurogenic bladder in 23 patients (59%). Of these 23 patients, 16 had a hyperreflexic and 7 an areflexic bladder. Of the 16 who had a hyperreflexic bladder, 4 and 2 were changed to normal and areflexic bladders, respectively, but 10 remained unchanged postoperatively. In 3 patients repeated UDS was performed, with 1 remaining unchanged, but 2 changed to an areflexic bladder. Of the 7 patients with an areflexic bladder, 1 changed to a hyperreflexic bladder, but 6 remained unchanged postoperatively. All 3 patients who underwent a repeated UDS remained unchanged. Hydronephrosis was noted on ultrasonography in 3 patients. The degree of improvement according to the timing of surgery was not statistically significant. All 16 patients who had a normal bladder remained unchanged postoperatively, and 2 patients who underwent repeated UDS changed to a hyperreflexic bladder. CONCLUSIONS: Even when pre- and postoperative UDS reveal a normal bladder function, long-term follow-up through UDS is still required. The use of routine preoperative and periodic postoperative urodynamic evaluations is important in children with LMMC, but without clinically overt symptoms.

Postoperative Electroencephalogram for Follow up of Pediatric Moyamoya Disease

It is well known that the electroencephalographic finding in patients with moyamoya disease demonstrates the characteristic "re-build up" phenomenon a few minutes after hyperventilation. To evaluate the usefulness of an electroencephalogram (EEG) in the postoperative management of children with moyamoya disease, we studied the presence or absence of improvement in the clinical, single photon emission computed tomography (SPECT) and EEG findings, before and after surgery. Twenty-two patients, who underwent indirect revascularization surgery for moyamoya disease, were included in our study. Clinical improvement was assessed as the disappearance or decrease of a transient ischemic attack or headache. The findings on the EEG and SPECT were considered improved when the re-build up phenomenon was absent and when there was improvement in the cerebrovascular reserve as a result of the acetazolamide challenge test. The statistical correlation analysis for both clinical and EEG improvement were consistent (kappa value=0.409, p< 0.05). However, the result from the clinical and SPECT improvement as well as that from EEG and SPECT improvement were not statistically significant. Our results suggest that EEG can be used as a noninvasive and simple follow-up test for moyamoya disease after indirect revascularization surgery if the hyperventilation procedure is effectively performed during EEG recording.

Prognostic Factors of Moyamoya Disease Evaluated by Activity of Daily Living / 대한뇌혈관외과학회지

BACKGROUNDS: Moyamoya disease is a progressive occlusive cerebrovascular disease which has characteristics of distal ICA stenosis and basal collateral vessels. Various methods of surgical treatment are recommended in the literatures but surgical strategies and outcome are not well established yet. AIMS AND METHODS: The aims of study is to evaluate surgical outcomes of moyamoya disease and to establish surgical indications. Total 155 patients diagnosed with moyamoya disease since 1990, were analyzed retrospectively. Female was more predominant by 1.5 : 1. There were two peaks of age of onset at the 1st decade (39.0%) and 4th (15.9%). Familial occurrence was 6.8% (n=17). The mean duration of follow-up was 41.1 months (12-156 months). Moyamoya syndrome was excluded in this study. Surgical outcomes were measured by grading activity of daily living (ADL) and prognostic factors were analyzed statistically with SAS. RESULT: The most common clinical presentations were transient ischemic attacks (69.1%), followed by cerebral infarction (26.0%) and hemorrhage (4.9%) in pediatric patients, but in adult hemorrhage (49.2%) was the most prevalent. Recurrence of symptoms developed in 8 patients (11.4%) among 70 of cerebral infarction with mean intervals of 30.8 months and 7 patients (21.9%) among 32 of hemorrhage with mean interval of 42.3 months. Forty five (29.0%) of 155 patients showed stenosis of posterior cerebral arteries on cerebral angiography at the age of diagnosis. The surgical treatment were performed at 183 hemispheres of 115 patients, direct bypass surgery was done in 10 hemispheres, indirect bypass surgery in 169 hemispheres (106 EDAMS, 14 EDAS, 40 frontal EDS or burr hole, 6 EDAMS and EDAS, 3 others), combined direct and indirect bypass surgery in 4 hemispheres. The improvement of ADL was not so different between 68 (59.1%) of 115 patients treated with surgery and 23 (57.5%) in 40 patients with conservative care. The initial and final grade of ADL of pediatric patients were better than those of adult (p0.1). Significant prognostic factors affecting outcomes of moyamoya disease age at onset, clinical features, and initial grade of ADL. CONCLUSION: Children with transient ischemic attack were the best, but adults with recurrent hemorrhage were the worst in outcomes. Surgical treatment for moyamoya disease should be carefully tailored according to age of onset and clinical features regardless of surgical methods. Further prospective study is indicated to determine optimal treatment guideline for moyamoya disease.

KSPNO Protocol for Ependymomas / 대한소아혈액종양학회지

No abstract available.

KSPNO Protocol for Glioma / 대한소아혈액종양학회지

No abstract available.

Clinical Experience of Pineal Parenchymal Tumors

OBJECTIVE: Pineal parenchymal tumors (PPT) are rare; therefore, only limited clinical data regarding their behavior is available. The aim of this study is to evaluate the pathologic features, clinical behavior, and response to therapy of these tumors. METHODS: Thirteen patients with biopsy-proven PPT were treated from September 1992 to February 2004 in our hospital. Their medical records and radiologic studies were analyzed retrospectively. The tumors were divided into three groups: pineocytoma, pineoblastoma, and mixed pineocytoma-pineoblastoma or PPT with intermediate differentiation. Initial stages were made by spinal magnetic resonance images and cerebrospinal fluid cytology. Magnetic resonance images were used for defining the treatment response of the patients. RESULTS: The patients were divided into 4 pineocytomas, 5 pineoblastomas, and 4 mixed/intermediate PPT. The patients aged from 2 to 77 years. The patients presented with headache, nausea, vomiting, memory and gait disturbances, and impaired vision. Only one patient showed disseminated disease at initial staging. All patients that had surgery showed improvements of hydrocephalus. Two patients showed complete responses to treatment, six showed partial responses, two showed stable diseases, and two showed disease progression. Eleven patients were alive, but two were dead due to disease progression. CONCLUSION: Obtaining a tissue diagnosis in patients with PPT is important. Although our experience is limited, we consider that multimodality therapies including surgical resection, radiotherapy, chemotherapy, and radiosurgery must be considered for better outcome in treating patients with PPT. More experience is necessary to determine the optimal treatments of PPT.