bluuebunny/crossref_metadata_2025_split huggingface dataset:
RARE-33. GIANT CELL TUMOR OF THE SKULL BASE WITH A HISTORY OF A SUCCESSFUL RESPONSE TO DENOSUMAB AND LATER DEVELOPING A SECOND TUMOR
Authors: Avagyan Manushak , Tamamyan Gevorg , Danielyan Samvel , Harutyunyan Martin , Karapetyan Nune , Karamyan Nerses , Vardevanyan Hovhannes , Mkhitaryan Armen , Bardakhchyan Samvel 
Published: December 2020

Abstract

BACKGROUND
Giant cell tumor of bone(GCTB) is a rare neoplasm with unpredictable behavior, possible malignant transformations, and/or lung metastases. Surgery is usually the treatment of choice. In unresectable or metastatic cases, treatment with denosumab is a new treatment option. CASE PRESENTATION: A 14-years-old female presented with cachexia, dysphagia, diplopia, discoordination, strabismus, and multiple cranial nerve palsies in 10.2015. MRI revealed intra-extracranial mass arising from C2 vertebrae, compressing the medulla oblongata and the left cerebellar hemisphere, invading to the sphenoid bone and nasopharynges. Biopsy showed a GCTB. Surgical resection was done, which was incomplete because of tumor location (cranial nerve and vertebral artery involvement). Then local radiation therapy was performed 50.4Gy. During RT patient’s condition declined and MRI showed disease progression. Treatment with denosumab 120mg q4w was initiated in 03.2016, which yielded successful results. Disease was under control for three years until 03.2019. Then she returned with clinical symptoms of diplopia and severe headache. MRI showed local tumor progression. Repeated biopsy revealed undifferentiated pleomorphic sarcoma, which could be either a malignant transformation of GCTB or a new tumor. The patient later underwent two cycles of chemotherapy with Ifosfamide/Doxorubicin. MRI after 2nd cycle showed marked tumor progression. The patient didn’t receive any further treatment because of cachexia and died due to disease progression in 12.2019.

CONCLUSION
To our knowledge, this is the youngest patient ever reported with a skull base tumor with such a clinical development, successful and long-time remission with denosumab and with such a chemotherapy-resistant malignant transformation or second cancer.
RARE-41. SECOND MALIGNANCIES FOLLOWING TREATMENT FOR PRIMARY CENTRAL NERVOUS SYSTEM TUMORS IN PEDIATRIC PATIENTS: A SINGLE-INSTITUTIONAL RETROSPECTIVE REVIEW
Authors: Pytel Nicholas , Dedekam Erik , Salamat M Shahriar , Puccetti Diane 
Published: December 2020

Abstract
Second malignant neoplasms following treatment for primary central nervous system (CNS) tumors in children are rare occurrences but may often have dire consequences, particularly, if thought to be induced by prior therapies. The authors retrospectively reviewed pediatric patients with primary CNS malignancies from the University of Wisconsin over the last 25 years (1994 – 2019) with any secondary malignant neoplasm and determined seven patients met criteria. Treatment modalities were reviewed with all patients receiving surgery, chemotherapy, and radiotherapy for treatment of their first malignancy. The second neoplasms found included 4 high-grade gliomas, 1 meningioma, 1 thyroid carcinoma, and 1 myelodysplastic syndrome. The median latency time between diagnoses was 9 years (range 4 -17 years). The outcomes varied according to histopathology of the second neoplasm with the high-grade glioma patients all deceased from progressive disease. The high-grade gliomas were thought to have been induced by prior radiation in most cases. The remaining patients are still alive, at the time of this writing, and in follow up after treatment for their second neoplasm. Thus, long-term follow up is essential for children treated for a primary CNS tumor given the variety of second neoplasms that could arise with differential consequences. In addition to our single institutional outcomes, we will also present an updated review of the literature of pediatric patients with primary CNS tumors and second malignancies.
RARE-43. FAVORABLE OUTCOME OF A YOUNG GIRL WITH RECURRENT METASTATIC PINEOBLASTOMA ASSOCIATED WITH A DICER1 MUTATION
Authors: Rossi Adam , Verona Gregory , Ritter Ann , Richard Hope , Sisler India , Wang Zhihong 
Published: December 2020

Abstract
Pineoblastomas have been thought to portend a poor prognosis, especially in younger children or those with metastases. Long term survivors after relapse, especially for those with metastatic disease are rare. We report a young girl with a DICER1 mutation who survived recurrent metastatic pineoblastoma. She was initially diagnosed at the age of 3 with a localized pineoblastoma, underwent gross total surgical resection, and received high dose chemotherapy with autologous stem cell transplant per COG ACNS0334 without radiation therapy. 16 months after completion of treatment, she relapsed at primary site with widespread spinal metastasis. She then received cranial spinal radiation of 3600Gy with proton beam, with boost to primary to 5580Gy, followed by chemotherapy with Temozolomide, Irinotecan and Avastin per COG ACNS0821. She is now 3 years and 3 months from completion of treatment, is doing well clinically with stable imaging findings. No particular alteration was identified from the tumor molecular testing of her initial pineoblastoma. Of note, she was diagnosed with pleuropulmonary blastoma soon after her initial diagnosis of pineoblastoma, and was found to have a DICER1 mutation (c.2062C&gt;T; pR688*) thought to be a nonsense mutation. While radiation therapy following recurrence is known to improve the outcome, more recent studies suggest that tumors lacking the molecular features of high grade glioma also has a positive impact on prognosis. In addition, we speculate that DICER1 mutations might increase sensitivity of cancer cells to some chemotherapy through modulating gene expression and /or interfering with DNA repair mechanisms, therefore, affecting treatment outcome.
RARE-45. SARCOMAS INVOLVING THE CENTRAL NERVOUS SYSTEM AT INITIAL PRESENTATION IN CHILDREN AND YOUNG ADULTS: A CASE SERIES
Authors: Liu Yen-Lin , Chen Shu-Mei , Lee Hsin-Lun , Huang Jia-Hui , Chen Shu-Huey , Kao Yu-Chien , Chang Hsi , Tsai Min-Lan , Tseng Sung-Hui , Hsieh Kevin L C , Chang Chia-Yau , Wang Jinn-Li , Miser James S , Wong Tai-Tong 
Published: December 2020

Abstract
Sarcomas of bone, soft tissue, or neural origin may occasionally invade the central nervous system (CNS), causing diagnostic and therapeutic challenges. We aim to investigate the clinical features of sarcomas involving the CNS at initial presentation. During 2015/01–2019/12, nine consecutive patients (4 Males and 5 Females) younger than 30 years of age treated at a University Healthcare System in Northern Taiwan were included. The median age was 8.7 years (range, 2–24 years); diagnoses were Ewing Sarcoma with EWSR1 rearrangements (n=4), CIC-NUTM1 Sarcoma (n=1), Osteosarcoma (n=2), Malignant Peripheral Nerve Sheath Tumor (MPNST; n=1), and extramedullary myeloid sarcoma (n=1). The tumors originated from the skull (n=1), dura (n=1), vertebra (n=4), spinal canal (n=1), or extra-CNS sites (n=2). Four patients had metastases (1 Ewing sarcoma, 2 osteosarcoma, and 1 extramedullary myeloid sarcoma). The main symptom at diagnosis was facial/eye pain (n=2), back pain (n=3), arm weakness (n=1), or gait disturbance (n=3). Upfront neurosurgical decompression (n=7) or urgent radiotherapy (n=1) was performed in most patients. At a median follow-up duration of 20.1 months, the overall survival rate was 70%. All patients with Ewing sarcoma (n=4) and CIC-NUTM1 sarcoma (n=1) achieved Complete Response after surgery, interval-compressed chemotherapy, radiotherapy, and adjuvant chemotherapy. Patients with stage IV osteosarcoma (n=2) had Partial Response; the patients with MPNST and extraskeletal myeloid sarcoma died of Progressive Disease at 18 and 3 months after diagnosis, respectively. We conclude that timely decompression, early diagnosis, and histology-driven multimodality treatment are effective strategies in managing sarcomas involving the CNS.
RARE-47. DIFFUSE LEPTOMENINGEAL DISSEMINATED GLIONEURONAL TUMOR: CASE-SERIES
Authors: Sanders Felipe Hada , Matushita Hamilton , Azambuja Alessandra , Frasseto Fernando , Rosemberg Sergio , Odone Vicente , Teixeira Manoel Jacobsen 
Published: December 2020

Abstract
Diffuse leptomeningeal disseminated glioneuronal tumor (DL-GNT) is a rare brain tumor that presents as a plaque-like subarachnoid tumor, commonly involving the basal cisterns and interhemispheric fissure of children but lacking intraparenchymal tumor. Here we report two cases focusing on clinicopathologic features. In all patients, radiography revealed characteristic leptomeningeal thickening and enhancement with minor superficial parenchymal lesions. The broadcast of the knowledge about this type of disease is important to increase awareness on this subject.
RARE-58. CONGENITAL METASTATIC CHORDOMA OF THE CLIVUS
Authors: Grajowska Wieslawa , Stepaniuk Maria , Trubicka Joanna , Wójcicka-Kowalczyk Katarzyna , Krych Piotr , Dembowska-Baginska Bozenna , Perek-Polnik Marta 
Published: December 2020

Abstract
Chordomas are rare midline axial skeletal neoplasms that typically present in adults. They are infrequent in childhood with typical localization in the spheno-occipital skull base. They are derived from remnants of the embryonic notochord. We present the case of 4 months old girl, who was born with „blueberry muffin” syndrome and was first negatively diagnosed for neuroblastoma and leukemia (two negative skin biopsies were performed) was admitted with axial laxity. In imaging testes there was a tumor of the scull base, metastases in the lungs and kidneys (that were not seen at previous assessments) and a small lesion in the heart. The third biopsy of skin lesion was performed and pathological examination revealed a neoplasm composed of cords, clusters, and chains of multivacuolated cells embedded within a myxoid matrix and separated by fibrous septa. No atypical and dedifferentiated features were present. Mitotic activity was not observed. Neoplastic cells showed the typical cytoplasmic immunostaining for EMA, S100 and cytokeratin AE1/AE3, strong nuclear brachyury expression, and retention of nuclear INI-1 expression. The diagnosis of chordoma was established. Neoplastic tissue and blood samples were obtained for molecular analysis using next generation sequencing, including germline mutations assessment (are ongoing). Chemotherapy as for soft tissue sarcomas was undertaken. Currently a patient is on treatment with improvement of neurological status.
RONC-08. SURVIVAL IMPACT OF POSTOPERATIVE RADIOTHERAPY TIMING IN PEDIATRIC AND YOUNG ADULT EPENDYMOMA
Authors: Shah Sunny , Mallory Chase , Gates Kevin , Rubens Muni , Maher Ossama , Niazi Toba , Khatib Ziad , Appel Haley , Kotecha Rupesh , Mehta Minesh , Hall Matthew 
Published: December 2020

Abstract

INTRODUCTION
Postoperative radiotherapy is commonly given for WHO Grade 2–3 intracranial ependymoma. Clinicians generally aim to begin radiotherapy ≤5 weeks following surgery, but the optimal timing remains uncertain.

METHODS
The National Cancer Database was queried for patients (age ≤39 years) with localized WHO Grade 2–3 intracranial ependymoma treated with surgery and postoperative radiotherapy. Multivariable logistic regression was used to identify factors associated with delayed postoperative radiotherapy, defined as starting &gt;8 weeks after surgery. Overall survival (OS) curves were plotted based on radiotherapy timing (≤5 weeks, 5–8 weeks, and &gt;8 weeks after surgery) and compared by log-rank test. Multivariate analysis (MVA) was used to identify factors associated with OS.

RESULTS
In the final analytic set of 1,043 patients, age ≥21 years (OR 2.07, 95% CI 1.56–2.74) and WHO Grade 2 tumors (OR 1.41, 95% CI 1.08–1.85) were significantly associated with delayed time to adjuvant radiotherapy. No difference in 3-year OS was observed in patients who initiated radiotherapy ≤5 weeks, 5–8 weeks, and &gt;8 weeks after surgery (89.8% vs. 89.1% vs. 88.4%; p= 0.796). On MVA, anaplastic histology (HR 2.414, 95% CI 1.784–3.268, p&lt;0.001) and subtotal resection (HR 2.398, 95% CI 1.519–3.788, p&lt;0.001) were significantly associated with reduced OS. Timing of radiotherapy, total radiotherapy dose, age, insurance status, and other factors were not significant.

CONCLUSION
Delayed postoperative radiotherapy was not associated with inferior survival in patients with intracranial ependymoma, suggesting delayed radiotherapy initiation may be considered in patients requiring longer postoperative recovery or referral to an appropriate radiotherapy center.
RONC-26. A CASE OF RADIATION NECROSIS OF THE CEREBELLUM 16 YEARS AFTER CHEMORADIOTHERAPY FOR MEDULLOBLASTOMA
Authors: Miyata Mayuko , Nonaka Masahiro , Asai Akio 
Published: December 2020

Abstract

BACKGROUND
If new lesions are observed during follow-up of the malignant tumor after treatment, it is difficult to distinguish whether the tumor is a recurrent lesion, secondary cancer, or radiation necrosis of the brain. We have encountered a patient with symptomatic radiation necrosis of the cerebellum 16 years after treatment of medulloblastoma. CASE PRESENTATION: A 24-year-old man who had received a tumor resection and chemoradiotherapy for cerebellar medulloblastoma at the age of 8 presented with dizziness. For the past 16 years, there was no recurrence of the tumor. He subsequently underwent MRI scan, and T1-Gd image showed enhanced lesion in the right cerebellar peduncle. Cerebrospinal fluid cytology analysis was negative for tumor. We suspected tumor reccurence or secondary cancer, and performed lesion biopsy. The result of the pathological examination was radiation necrosis of the cerebellum. DISCUSSION: The interval of radiation necrosis of the brain and radiotherapy can vary from months to more than 10 years. So, whenever a new lesion is identified, radiation brain necrosis must be envisioned. According to guidelines in Japan, there is no absolute examination for discriminating tumor recurrence from radiation brain necrosis and diagnosis by biopsy may be required.

CONCLUSION
We experienced a case of symptomatic radiation necrosis of the cerebellum 16 years after treatment. In patients showing new lesion after long periods of time, the possibility of radiation necrosis to be considered.
SURG-03. IMMERSIVE VIRTUAL REALITY APPLICATIONS IN NEUROSURGICAL ONCOLOGY
Authors: Carai Andrea , Mastronuzzi Angela , Colafati Giovanna Stefania , Voicu Paul , Onorini Nicola , Carboni Alessia , Del Baldo Giada , Herur-Raman Aalap Jamadagni , De Benedictis Alessandro , Marras Carlo Efisio 
Published: December 2020

Abstract
Tridimensional (3D) rendering of volumetric neuroimaging is increasingly been used to assist surgical management of brain tumors. New technologies allowing immersive virtual reality (VR) visualization of obtained models offer the opportunity to appreciate neuroanatomical details and spatial relationship between the tumor and normal neuroanatomical structures to a level never seen before. We present our preliminary experience with the Surgical Theatre, a commercially available 3D VR system, in 60 consecutive neurosurgical oncology cases. 3D models were developed from volumetric CT scans and MR standard and advanced sequences. The system allows the loading of 6 different layers at the same time, with the possibility to modulate opacity and threshold in real time. Use of the 3D VR was used during preoperative planning allowing a better definition of surgical strategy. A tailored craniotomy and brain dissection can be simulated in advanced and precisely performed in the OR, connecting the system to intraoperative neuronavigation. Smaller blood vessels are generally not included in the 3D rendering, however, real-time intraoperative threshold modulation of the 3D model assisted in their identification improving surgical confidence and safety during the procedure. VR was also used offline, both before and after surgery, in the setting of case discussion within the neurosurgical team and during MDT discussion. Finally, 3D VR was used during informed consent, improving communication with families and young patients. 3D VR allows to tailor surgical strategies to the single patient, contributing to procedural safety and efficacy and to the global improvement of neurosurgical oncology care.
SURG-24. NOVEL MALLEABLE FORCIPES FOR ENDOSCOPIC ASSISTED TECHNIQUE IN PEDIATRIC BRAIN TUMORS
Authors: Nakahara Yukiko , Ito Hiroshi , Yoshioka Fumitaka , Inoue Kohei , Ogata Atsushi , Masuoka Jun , Abe Tatsuya 
Published: December 2020

Abstract
Recent advances in optical devices and surgical instruments have been applied to neurosurgery. Even with modifications, one of the most serious risks is injury of neuronal and vascular structure caused by operation of surgical instruments in a narrow surgical field. Fixed instruments are not practical for pediatric brain tumor surgeries because the length of the curved or angled tip portion is limited because of the narrow entrance. We developed a novel malleable forceps to resolve the difficulties related to microsurgical procedures. The malleable forceps has two shafts with a sharp cup at the tip. The entire forceps was made of stainless steel, with a silver and nickel alloy inserted between 10 and 40 mm from the tip. In the alloy part, the surgeon can flex the forceps freely using a special cylinder. The special cylinder is useful to prevent from slipping of the cups of tip. The maximum angle that can be bent is 70 degrees vertically. We also developed a monoshaft malleable forceps. We used these flexible forcipes in the case of various pediatric brain tumors including craniopharyngioma. We performed tumor resection by anterior interhemispheric trans-lamina terminalis approach. After procedure of tumor resection using microscope, endoscope inserted around the pituitary stalk. The piece of calcified tumor could be easily removed without any complications. These forcipes can be deformed to an appropriate angle and can be applied to various cases, especially pediatric brain tumors.
SWK-02. WEAVING COMFORT AND SUPPORT FOR CHILDREN WITH BRAIN TUMORS AND THEIR FAMILIES IN AN OUTPATIENT CLINIC
Authors: Hawks Ria , Bloom Jane , Packard Maurine , Lugo Sonia , Ulysses Nadine , Zacharoulis Stergios 
Published: December 2020

Abstract
Parents of children diagnosed with brain tumors report high levels of stress at diagnosis and feelings of “being lost” on transition to outpatient follow-up care (Jackson AC, et al, 2007). Ssori is a Japanese form of free-style weaving that encourages people facing life-limiting challenges to discover inner strengths. We report our experience with Saori weaving with brain tumor patients and their families in a pediatric oncology outpatient clinic at a major university medical center. During 2019, we offered weaving sessions twice a week. We had a total of 151 encounters with hematology/oncology patients (age 5–18 years), siblings, or parents. Among these patients there were 20 with primary brain tumor diagnoses. Weaving was offered in the art therapy area of the clinic. After creating a fabric, the weavers had the opportunity to have their work sewn into functional objects, such as pillows, bags, purses, or healing pouches filled with beans that can be heated or cooled for comfort. Brain tumor patients readily engaged in weaving, despite various degrees of neurologic disability including hemiparesis or low vision. In the words of an 8 y/o weaver. “This is so cool. Daddy, can we always come when the weavers are here, so I can weave?” And from a mother: “This is great. She’s focused and busy!” Case studies, including a presentation of Legacy work, will be reported. In conclusion, Saori weaving can be an impactful intervention for childhood brain tumor patients and their families in an outpatient clinic setting.
SWK-03. CAREGIVER EXPERIENCES FOR PEDIATRIC BRAIN TUMOR PATIENTS AND THEIR FAMILIES AT A DEDICATED MEDICAL SPECIALTY CAMP
Authors: Brownfield Ryan , Galantowicz Barb , Russell Claire , Chabot Jennifer , Coven Scott 
Published: December 2020

Abstract

BACKGROUND
Medical specialty camps have provided children with unique psychosocial experiences; however, dedicated pediatric brain tumor camps are rare in the United States, except in limited locations. This study aimed to glean caregiver perceptions from a dedicated family brain tumor camp, and to learn about the family experience with navigating a neuro-oncology diagnosis. DESIGN: Flying Horse Farms is a non-profit organization located in Mt. Gilead, Ohio and a member of the SeriousFun Children’s Network, a global community of camps and programs serving children with serious illnesses and their families, at no cost. The institutional review board at Ohio University approved this project at Flying Horse Farms in September 2017. Consent from caregivers was obtained prior to participation in the study, which provided the opportunity to complete three separate phases: a pre-camp survey, attend a semi-structured interview during the weekend, and complete a post-camp survey.

RESULTS
11 families were present for the weekend, and 10 families consented to participate in all three phases. For 6 families, this was their first experience at Flying Horse Farms. For 9 of the 10 families, the camp met their expectations. Additionally, 9 out of 10 families reported they would be interested in attending a diagnosis specific camp again in the future.

CONCLUSIONS
This work demonstrates the feasibility of conducting research at a medical specialty camp without restricting the camp experience. Better understanding of the attendee’s attitudes toward camp may enhance the experience and the neuro-oncology journey in the future.
SWK-07. A MULTINATIONAL SURVEY OF PAEDIATRIC NEURO-ONCOLOGY SERVICES: A EUROPEAN RESEARCH NETWORK (ERN) PAEDCAN PROJECT
Authors: Cooper Katherine , Pizer Barry , Lane Steven , Rutkowski Stefan 
Published: December 2020

Abstract

BACKGROUND
Brain tumours are clinically and biologically highly diverse and account for 25% of paediatric neoplasms. They carry the highest mortality and morbidity of tumour groups. Their management presents significant challenges with performing modern diagnostic assessments, applying multimodal treatment and establishing interdisciplinary cooperation. Outcomes across Europe differ significantly with varying 5year survival reports of 42–79%. This SIOP-Europe PaedCan survey assessed the structures and facilities for individual states and highlight areas for cooperation and support. DESIGN: An online questionnaire was sent to SIOP-Europe Brain Tumour Group members. This had 55 questions assessing pathology, staging, surgery, radiotherapy and paediatric oncology infrastructure. For analysis of the data we divided countries into lower and higher economic status according to GDP (World Bank 2019) with a cut off of $30,100.

RESULTS
There were 388 respondents from 44 countries in 181 different institutions. In the lower GDP group we noted decreased access to biological characterisation of tumours and interdisciplinary tumour boards. In this group of nations, patients were less likely to have treatment by a paediatric specialist neurosurgeon, paediatric neuro-oncologist, neuroradiologist, and paediatric radiation oncologist. There was also less availability to perform early MRI (ventilated) and less access to proton beam therapy. This study supports the aim of the ERN to produce a roadmap document with specific standards and publish guidelines for all relevant diagnostic and therapeutic components of care. The ERN also aims to identify a network of institutions to provide patient advice and training to equalise treatment and outcomes for all children across Europe.