bluuebunny/crossref_metadata_2025_split huggingface dataset:
ML-14 LOW INVASIVE APPROACH FOR PRIMARY CENTRAL NERVOUS SYSTEM LYMPHOMA TREATMENT INVOLVING RITUXIMAB–METHOTREXATE + PROCARBAZINE + VINCRISTINE AND TRIPLE INTRATHECAL INJECTION
Authors: Ogawa Takahiro , Oowada Kei , Tanba Kazuna , Hirakawa Kouichi 
Published: December 2019

Abstract

INTRODUCTION
Although the treatment outcomes of primary central nervous system lymphoma (PCNSL) with multiple treatment regimens involving high–dose methotrexate (MTX)–based multiagent chemotherapy have improved compared with the outcomes with high–dose methotrexate therapy in recent years, its regimen has not been established. Additionally, it is controversial whether intrathecal (IT) MTX injection can be included in a regimen. Our facilities treat PCNSL with R–MPV (rituximab–methotrexate + procarbazine + vincristine) and triple IT injection (methotrexate + Ara C + prednisolone). Here we report a low invasive approach for PCNSL treatment involving R–MPV therapy and triple IT injection.

CASE DESCRIPTION
A 58–year–old woman complained of prolonged headache and had undergone a medical checkup at our department. Head computed tomography revealed a mass lesion with edema near the right anterior horn of the lateral ventricle. After being hospitalized, biopsy was performed via a small craniotomy for the mass lesion near the right anterior horn of the lateral ventricle, which was suspected to be PCNSL. Multipurpose Head Frame 2 (Mizuho Co., Ltd.) was used for four points of head fixation. Vertek Biopsy Kit (Medtronic Co., Ltd.) was attached to it, and needle biopsy guided by neuronavigation was performed. Finally, biopsy samples were obtained from the lesion. Changes in the entry and target points could be easily made through the operative procedure, which was performed uneventfully. The diagnosis was B–cell–related lymphoma. Subsequently, an Ommaya reservoir was placed via lumbar puncture on postoperative day 7. Finally, the patient was successfully treated with R–MPV therapy and triple IT injection, thereby avoiding repeated lumbar puncture and reduced pain associated with IT injection.

CONCLUSION
We have reported the diagnosis of PCNSL with needle biopsy guided by neuronavigation and its treatment with R–MPV therapy and triple IT injection after lumbar Ommaya reservoir placement. This approach was associated with reduced invasion and pain in the patient.
MNG-01 NATURAL HISTORY OF ASYMPTOMATIC MENINGIOMAS: REVIEW WITH META-ANALYSES
Authors: Nakasu Satoshi , Nakasu Yoko 
Published: December 2019

Abstract
Frequency of meningioma as an incidental finding in neuroimaging study is increasing. It is important to characterize tumors that will grow to be symptomatic in order to select appropriate treatment and radiological follow-up because long-term observation may increase the risk of surgery due to enlargement of the tumors and aging of patients. [Methods]We reviewed 26 studies that analyzed natural courses in asymptomatic or untreated meningiomas. RESULTS: In time-growth rate analysis, nearly 70% of meningiomas showed radiological progression defined by a volume criteria and the rate approached plateau at 5–6 years. About half of incidental meningiomas presented a decelerating growth pattern or no growth while less than one-fourth of them grew exponentially. Growth pattern change could be affected by the length of follow-up period. Radiological progression, growth speed (annual volume change (AVC) or relative growth rate (RGR)) and symptomatic progression had each different factor related to their progression. Younger age, non-calcification and high intensity on T2 weighted image related to radiological progression and rapid growth speed but not to symptomatic progression. Tumors in men and lager size were likely to be symptomatic in meta-analysis. AVC (&gt;= 2.1cm3/year) was the strongest indicator for symptom development. In the group of AVC &gt;=2.1cm3/year, progression free rate was 69.3% at 3 years, and reached to 55.4% at 6 years whereas 100% in slower growth group. [Conclusion]Radiological features may not be useful for prediction of symptom development except for perifocal edema in a long term. This may be due to dynamic change of these radiological markers in a long term. Quantitative tumor size and growth speed especially AVC were important factors for decision of treatment.
MNG-07 A CASE OF TRANSFORMED ANAPLASTIC MENINGIOMA IN 18 YEARS
Authors: Fujii Yu , Ogiwara Toshihiro , Yamazaki Daisuke , Nagamine Kohei , Horiuchi Tetsuyoshi 
Published: December 2019

Abstract

INTRODUCTION
Although 95% of meningiomas are benign tumor (WHO grade I), they rarely exist malignant transformation. It is well known that WHO II or III meningiomas rapidly grow and recurrent frequently. However, there are no common sense about duration of malignant transformation between previous reports. Here we report a case of anaplastic meningioma which showed malignant transformation in 18 years.

CASE
The patient was 77-year-old man. He was pointed out as having a 20-mm mass in the left occipital convexity at brain checkup 19 years before. After 6 months, the mass was totally removed (Simpson grade I) at previous hospital. Histopathological diagnosis revealed meningioma with psammoma body, whose Ki-67 index was below 1 %. Postoperative course was good and periodic follow up was abandoned. The patient presented with consciousness disturbance and right hemiparesis after 18 years from the first operation. CT scan showed a 30-mm mass with intratumoral hemorrhage and perifocal edema in the left occipital lesion. Tumor removal was performed. The border of the tumor was clear and the macroscopic view was compatible with meningioma at intraoperative findings. The tumor was removed as much as possible, but partially remained which were extended to superior sagittal sinus (Simpson grade III). Histopathological findings were anaplastic meningioma with sarcoid change, whose Ki-67 index was 70%. His neurological abnormality improved postoperatively.

DISCUSSION
In the previous report, anaplastic meningioma is a rare tumor, which is 1% of whole meningioma. The rate of malignant transformation is reported 12–38%. The cause of malignant transformation is reported to be radiation therapy, mechanical stimulation by surgical manipulation, viral infection, and loss of chromosome. Although recurrence rate is low at total resection of meningioma, there rarely exists malignant transformation after a long period of time like our case.
BT-05 A CASE OF GLIOMATOSIS CEREBRI WITH TOTAL SPINAL CORD EXTENSION
Authors: Chiba Yasuyoshi , Takemoto Osamu , Yamada Junji , Matsumura Takaki 
Published: December 2019

Abstract

INTRODUCTION
Although gliomatosis cerebri is no longer a pathological diagnostic name, it is still an important disease state as a gliomatosis cerebri growth pattern. Here we report a case of gliomatosis cerebri originating from the left cerebral hemisphere that developed whole spinal cord.

CASE
A 10-year-old boy. He has a history of 13q-syndrome and left retinoblastoma. Left eye enucleation and chemotherapy (modified 98A1) have been performed. MRI in July 2017 showed no abnormalities, but in September 2018, he developed epilepsy. MRI revealed a gliomatosis cerebri that spreads extensively in the left cerebral hemisphere. Biopsy revealed anaplastic astrocytoma (MIB-1 LI; 22%, IDH1 / 2; WT, TERT C228T mutation positive) and IMRT (59.4Gy) and temozolomide (Stupp regimen) were performed in December 2018. In June 2019, neck pain developed. Head and neck MRI revealed that the tumor in the head increased lightly, and there was no suspicion of tumor growth in the brainstem, but the tumor progressed to the entire spinal cord. Therefore, radiation therapy was started from the lower brainstem that had not been irradiated last time to the entire spinal cord, and administration of bevacizumab was started.

DISCUSSION
Based on the single cell origin theory, the left hemisphere tumor and spinal cord tumor should be continuous. Since gliomatosis cerebri is visualized on MRI only after the tumor volume has increased and edema has occurred, it may appear as if there is no tumor in between. The spinal cord MRI was not taken, so it is only speculation, but it seems that tumor cells had probably infiltrated the spinal cord from the beginning, and it seems that it gradually increased because it was not irradiated. Considering the possibility of remote invasion as in this case, it is necessary to consider taking MRI of spinal cord at the first occurrence.
BT-09 CLINICAL AND MOLECULAR GENETIC FEATURE OF CEREBELLAR GLIOBLASTOMA
Authors: Iijima Shohei , Saito Kuniaki , Shimizu Saki , Kobayashi Keiichi , Shimada Daisuke , Matsumoto Yoshie , Shiokawa Yoshiaki , Nagane Motoo 
Published: December 2019

Abstract

INTRODUCTION
Cerebellar glioblastoma (cGBM) is extremely rare, accounting for 0.7–0.9% of all gliomas. Few studies have reported on clinical course, histopathology, and prognosis. In this report, we discussed cases which were diagnosed as cGBM, and were treated in our institute.

Materials and Methods
We retrospectively analyzed 9 cGBMs (age ranged 41 to 85 years, median 69), operated at our institute after 2010 January, and evaluated their &lt;MGMT&gt; promoter methylation, &lt;IDH1&gt; mutation, and Copy Number Variation status detected by methylation-specific PCR (MSP), DNA sequencing or immunohistochemistry, and Multiplex Ligation-dependent Probe Amplification (MLPA), respectively.

RESULTS
All patients underwent resection; 3 gross total resections (GTRs, 33%), 2 subtotal resections, 4 partial resections, with relatively low achievement of GTR. The tumor location predominated in the cerebellar hemisphere (7 patients, 78%) over vermis (2). One patient had brain stem invasion. After surgery, 8 patients received temozolomide (TMZ) and radiotherapy (RT), while did only one RT alone. After recurrence, three patients were treated with bevacizumab monotherapy, and other three received either TMZ and RT, TMZ and ACNU, or TMZ monotherapy. The median progression-free survival (PFS) was 12.0 months, and the median overall survival (OS) was 17.1 months. Five patients (56%) were &lt;MGMT&gt; methylated, whereas all were &lt;IDH1&gt;wild-type. &lt;PTEN&gt; deletion was negative in all patients. &lt;EGFR&gt; amplification and combined &lt;PDGFR&gt; amplification and &lt;CDKN2A&gt; deletion were found in one patient each.

DISCUSSION
Despite the lower rate of GTR, there was a tendency of longer PFS compared to supratentorial GBM (sGBM). The clinical course after recurrence was unfavorable, and OS thereafter was similar to that of sGBM. cGBMs appeared to lack the typical genetic mutations occurred in sGBM, suggesting that cGBMs might be stimulated with different regulatory cellular signals.
CS-02 CLINICAL AND MOLECULAR ANALYSIS OF ASTROBLASTOMAS
Authors: Ichikawa Tomotsugu , Mino Satoshi , Kenmotsu Naoya , Kuramoto Satoshi , Ono Yasuhiro , Kawauchi Masamitsu , Kurozumi Kazuhiko , Date Isao , Shimada Akira , Nakamura Satoko , Yanai Hiroyuki , Hirato Junko , Yokoo Hideaki 
Published: December 2019

Abstract
Astroblastoma is extremely rare brain tumor which mostly arise in cerebral hemisphere of children and young adult. Limited data exists on its clinical feature and molecular analysis. We recently experienced two female patients with astroblastoma in the cerebrum.
Case 1 is three-year-old girl. She developed left hemiparesis. CT and MRI revealed large supratentorial mass with cystic component and calcification. Gross total removal was achieved. She is well without recurrence on MRI one year after surgery. Case 2 is 42-year-old lady. She developed partial seizure. CT and MRI revealed a mass with ring-enhancement in the left temporal lobe. Gross total removal was achieved under awake craniotomy. She is well without recurrence on MRI six months after surgery. Pathologic examination of both patients showed pseudorosette formation of tumor cells around vasculature. Molecular analysis revealed rearrangement of MN-1 in case 1 but not in case 2. Case 2 showed BRAF V600E mutation and loss of CDKN2A/2B. Both patients received no adjuvant therapy.
Prognosis of astroblastoma varies and standard of treatment is not established. Gross total resection is associated with increased survival, but the role of adjuvant chemotherapy and radiation therapy are controversial. Advances in molecular analysis will lead to establish molecular classification and risk-adapted treatment strategy.
CS-09 PROPOSAL OF A NEW CLASSIFICATION FOR DETERMINING THERAPEUTIC STRATEGY ACCORDING TO THE PROGRESSION OF OLFACTORY NEUROBLASTOMA
Authors: Motegi Hiroaki , Yamaguchi Shigeru , Ishi Yukitomo , Endo Shogo , Kobayashi Hiroyuki , Terasaka Shunsuke , Suzuki Masanobu , Nakamaru Yuji , Homma Akihiro , Houkin Kiyohiro 
Published: December 2019

Abstract

INTRODUCTION
Olfactory neuroblastoma (ONB) is a rare type of malignancy that infiltrates and propagates from the nasal cavity to the anterior skull base and into the cranium. Various treatment strategies have been used at different institutions and time of treatment. Although the staging system proposed by Kadish is commonly adopted has not proven useful for predicting prognosis or choosing among treatment strategies. Factors to be considered have increased accordingly, for example, whether to perform ESS alone or in combination with craniotomy, whether to try preserving the olfactory sense and whether to use neoadjuvant chemotherapy. In this study, we reviewed ONB cases treated at our institution to propose a new classification system to help determine treatment strategies.

METHODS
Thirty-four patients treated at Hokkaido University were included. Stages of craniocaudal progression were defined as Nasally/Paranasally localized (NP), Frontal Base progression (FB), and Brain invasion (BI). Stages of lateral progression were defined as Midline (M) or Lateral extension (L), and Unilateral (U) or Bilateral (B).

RESULTS
Between 2008 and 2016, patients at the BI stage were proactively treated with neoadjuvant chemotherapy and achieved long-term survival (mean overall survival, 64.2 months). However, no standard way of choosing among treatment options was established. M-stage patients underwent concurrent craniotomy. From 2017 onwards, 5 patients were treated according to the new classification system. All were FB-M cases, including 4 cases of B disease, in which ESS alone followed by radiotherapy was used. One patient in the FB-M-U category underwent unilateral resection and the olfactory sense was preserved. In general, the treatment with ESS alone appeared to be preferred for M disease, and surgery after neoadjuvant chemotherapy was advisable for BI cases.

CONCLUSION
The result suggests that the new classification system is helpful to decide the treatment strategy according to the progression of ONB.
COT-02 TREATMENT EXPERIENCE OF PAZOPANIB FOR A CASE OF VON-HIPPEL LINDAU DISEASE
Authors: Hayashi Nobuhide , Koyama Yoshiki , Tsuji Eisaku , Okada Hideo , Kuwata Toshikazu 
Published: December 2019

Abstract

INTRODUCTION
von Hippel-Lindau (VHL) disease is an autosomal dominant genetic disorder associated with neoplastic lesions in multiple organs. Here, we report our experience with a patient with VHL disease presenting with complications of renal tumors, wherein pancreatic cystic lesions and renal neoplastic lesions were reduced in size with the administration of pazopanib at our department.

PATIENT
The patient was a 26-year-old man who presented with hiccups and was diagnosed with medullary hemangioblastoma with a cyst that was resected. The other central nervous system lesions were located in the right optic nerve sheath, cervical and thoracic spinal cord, with multiple cystic type tumors in the pancreas, renal tumors, and epididymal tumors. Although the family history was unclear, the clinical diagnostic criteria for VHL disease were met, and mutations were found in the VHL gene analysis. Six months after the initiation of pazopanib therapy at a dose of 800 mg/day, there was no remarkable change in the hemangioblastoma on imaging; however, the pancreatic and renal lesions had shrunk. In addition, new lesions did not appear. Adverse events included diarrhea, graying of hair.

DISCUSSION
Pazopanib is a multi-tyrosine kinase inhibitor that inhibits angiogenesis and inhibits tumor growth. In VHL disease, pancreatic and renal tumors influence the survival prognosis, and for hemangioblastoma, the lesions increase in number and size and the corresponding surgery affects the functional prognosis. Although there was a poor tumor-reduction effect on the hemangioblastoma, there was a supposed inhibitory effect on the appearance of new lesions and the enlargement of the existing lesions.

CONCLUSION
Pazopanib administration resulted in the shrinkage or regression of pancreatic and renal lesions. In addition, it inhibited the increase in number and size of hemangioblastomas. Further, prolonging the surgical treatment interval may help maintain the patient’s quality of life.
COT-07 HEREDITARY BRAIN TUMOR DISEASE MEDICINE IN THE CANCER GENOME MEDICAL AGE-IMPORTANCE OF MULTIDISCIPLINARY COLLABORATION AND GENETIC COUNSELING
Authors: Takayanagi Shunsaku , Tanaka Shota , Shin Masahiro , Nakatomi Hirofumi , Oseto Kumiko , Sato Atsushi , Saito Nobuhito 
Published: December 2019

Abstract

INTRODUCTION
Recently, the cancer genome medical system has been rapidly promoted in Japan, such as cancer related gene panel test as NCC oncopanel and Fonduation One being covered by insurance. At the same time, there is a great deal of attention now on hereditary tumor diseases caused by abnormalities in cancer-related genes and their medical treatment. Von Hippel-Lindau (VHL) disease and tuberous sclerosis (TSC) are one such hereditary tumor disease, and develop tumors in multiple organs such as kidney tumors and lung tumors as well as brain tumors. The University of Tokyo Hospital has been developing an in-hospital medical care system for VHL disease and TSC. [VHL disease] If VHL disease is diagnosed at an early stage and strives for early detection and treatment of tumor, it is said that the prognosis is not largely different from normal people. Therefore, our hospital opened a specialized outpatient for VHL disease from 2012, and has carried out genetic diagnosis of VHL disease and medical treatment based on it. [TSC] Recently, the use of Afinitor for SEGA was covered by insurance in Japan, and a system in which multiple departments involved in our hospital cooperated was established, and regular meetings were held to determine treatment policies.

DISCUSSION
It was the development of genetic counseling system and multidisciplinary cooperation system that was important in any disease. Genetic counseling helps patients with illness and their families to genetically understand and adapt to illness. In the case of hereditary diseases, the practice of genetic counseling was very useful. In addition, since it is a disease involving multiple organs, close cooperation with other in-hospital clinics has been extremely useful for early detection and treatment of patient’s lesions.
COT-15 LITERATURE REVIEW ON THE DECISION MAKING OF THE BRAIN TUMOR PATIENT
Authors: Kishi Tatsuya , Sakurada Naoya , Horikawa Mayumi , Ohkubo Haruaki , Ishii Kazumi , Miyakita Yasuji , Ohno Makoto , Takahashi Masamichi , Narita Yoshitaka 
Published: December 2019

Abstract

BACKGROUND
Patients with primary brain tumors find it difficult to make decisions during the advanced disease stage and experience decreased consciousness. It is important for patients to receive supported decision-making early. Medical staff should know what to do and when to do it,but there are no clear guidelines. Therefore,we reviewed the literature for supported decision-making for primary brain tumor patients,particularly to provide information for understanding trends reported in previous research.

METHOD
On January 1,2019,we conducted a search using keywords,such as “brain tumor”and “decision-making,” via PubMed and “Igakuchuo-zashi” in Japan. We extracted literature about treatment decision support and end-of-life care for patients with primary brain tumors. Furthermore,we studied and chose the documents for information provision.

RESULT
Upon observing 7 studies,we found: 1) about 50% of the patients want more prognostic information; 2) patients with brain tumor tend to be anxious,but they want more information to develop a good understanding of the disease and to lower their anxiety; 3) about half of the brain tumor patients in end-of-life care are unable to make decisions sooner owing to impaired consciousness,and hence are unable to share treatment preferences with their doctors; 4) when medical professionals provide information,such as adding video tools about end-of-life care to oral explanations,it facilitates supported decision-making; and 5) when the caregiver intends to notify patients,the family feels conflicted.

DISCUSSION
The results suggested that if the timing of the end-of-life conversation is late,it becomes difficult for the patient to make decisions and the burden of decision-making falls on the family. It is necessary to examine effective supported decision-making tools for patients by assessing and comprehending information needs and anxiety levels of primary brain tumor patients.
COT-23 INITIAL EXPERIENCE OF TREATMENT FOR GLIOBLASTOMA BY NOVO-TTF
Authors: Omura Takaki , Miyakita Yasuji , Ohno Makoto , Takahashi Masamichi , Satomi Natsuko , Fujimoto Kenji , Omura Tomoko , Narita Yoshitaka 
Published: December 2019

Abstract

PURPOSE
In 2018,Optune (TTF) became available covered by public insurance for patients with glioblastoma,based on the effectiveness of the US EF-14 study (TTF + TMZ (temozolomide) vs. TMZ). There are problems such as use of at least 18 hours a day,behavior restrictions,expensive medical expenses,etc. From the initial use experience of optune in our hospital,we examined the problems of effectiveness and treatment of TTF.

METHOD
We examined patient approval rate of TTF use and treatment problems in patients with glioblastoma who started treatment in our department from 2017.11 when TTF became covered by insurance.

RESULT
There were 41 patients with primary glioblastoma during the study period (male: female = 25: 16,median age 60 years (22–77)). There are 10 patients with KPS &lt;70 that are not TTF indications (male: female = 7: 3,median age 45.5 (29–64),consent rate 38.5%). For 26 people (63% of the total) excluding 5 patients who participated in clinical trial,we explained that TTF indications,clinical trial results,adverse events,wearing rate should be 75% or more,and that full shaving is required. 10 people wanted to undergo TTF treatment. The consent rate of women is lower than that of men (46.7% vs. 27.3%),there was a tendency to take TTF treatment in young patients. Average wear rates of 5 patients who continued for more than 6 months were 55%,76%,84%,85%,91%,respectively. Use of TTF for 6 months or longer with residual tumor was CR 1 case,PR 1 case,SD 2 cases. Our department policy is to continue for 12 months. One person ends in 12 months,the other continues to use TTF. So far,no adverse events caused by TTF have been observed,though,there were complaints about the weight of the equipment,feeling of binding,restrictions on bathing,etc.,and one person stopped using it in the fifth month.

DISCUSSION
The consent rate for TTF use was about 40% as expected,and 80% of patients with an average wearing rate of 75% or more. TTF can generally continue once the patient is convinced and begins to use,but seems to be severely restricted in behavior in patients with paralysis. There is no profound data on TTF in Japan,and it is necessary to collect data at multiple facilities and clarify the effectiveness and safety in the future.