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360. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO).

Author

Jordan, Michael B, Jordan, Michael B, Allen, Carl E, Greenberg, Jay, Henry, Michael, Hermiston, Michelle L, Kumar, Ashish, Hines, Melissa, Eckstein, Olive, Ladisch, Stephan, Nichols, Kim E, Rodriguez-Galindo, Carlos, Wistinghausen, Birte, McClain, Kenneth L, Jordan, Michael B, Jordan, Michael B, Allen, Carl E, Greenberg, Jay, Henry, Michael, Hermiston, Michelle L, Kumar, Ashish, Hines, Melissa, Eckstein, Olive, Ladisch, Stephan, Nichols, Kim E, Rodriguez-Galindo, Carlos, Wistinghausen, Birte, and McClain, Kenneth L

Abstract

Hemophagocytic lymphohistiocytosis (HLH) is a syndrome of pathologic immune activation, often associated with genetic defects of lymphocyte cytotoxicity. Though a distinctive constellation of features has been described for HLH, diagnosis remains challenging as patients have diverse presentations associated with a variety of triggers. We propose two concepts to clarify how HLH is diagnosed and treated: within the broader syndrome of HLH, "HLH disease" should be distinguished from "HLH disease mimics" and HLH subtypes should be categorized by specific etiologic associations, not the ambiguous dichotomy of "primary" and "secondary." We provide expert-based advice regarding the diagnosis and initiation of treatment for patients with HLH, rooted in improved understanding of its pathophysiology.

Published
2019

365. Proceedings of the International Cancer Imaging Society (ICIS) 16th Annual Teaching Course

Author

Koh, Dow-Mu, Kaste, Sue Creviston, Vinnicombe, Sarah J., Morana, Giovanni, Rossi, Andrea, Herold, Christian J., McLoud, Theresa C., Frey, Kirk A., Gebauer, Bernhard, Roebuck, Derek, Fütterer, Jurgen J., Towbin, Alexander J., Huisman, Thierry A. G., Smets, Anne M. J. B., Lee, Jeong Min, Chandarana, Hersh, Mayerhoefer, Marius E., Raderer, Markus, Haug, Alexander, Eiber, Matthias, Rockall, Andrea, Sohaib, Aslam, Warbey, Victoria S, Vargas, Hebert Alberto, Heiken, Jay P., Francis, Isaac R., Al-Hawary, Mahmoud M., Kaza, Ravi K., D’Onofrio, Mirko, Thoeny, Harriet C., King, Ann D., Piccardo, Arnoldo, Garrè, Maria Luisa, Reed, Nick, Rodriguez-Galindo, Carlos, Wasnik, Ashish P., Diederich, Stefan, Oyen, Wim J. G., Chaw, Cheng Lee, van As, Nicholas, Vieira, Igor, De Keyzer, Frederik, Dresen, Elleke, Han, Sileny, Vergote, Ignace, Moerman, Philippe, Amant, Frederic, Koole, Michel, Vandecaveye, Vincent, Dresen, R., De Vuysere, S., De Keyzer, F., Van Cutsem, E., D’Hoore, A., Wolthuis, A., Vandecaveye, V., Pricolo, P., Alessi, S., Summers, P., Tagliabue, E., Petralia, G., Pfannenberg, C., Gückel, B., Schüle, S. C., Müller, A. C., Kaufmann, S., Schwenzer, N., Reimold, M., la Fougere, C., Nikolaou, K., Martus, P., Cook, G. J., Azad, G. K., Taylor, B. P., Siddique, M., John, J., Mansi, J., Harries, M., Goh, V., Seth, S., Burgul, R., Seth, A., Waugh, S., Gowdh, N. Muhammad, Purdie, C., Evans, A., Crowe, E., Thompson, A., Vinnicombe, S., Arfeen, F., Campion, T., Goldstraw, E., D’Onofrio, M., Ciaravino, V., Crosara, S., De Robertis, R., Mucelli, R. Pozzi, Uhrig, M., Simons, D., Schlemmer, H., Downey, Kate, Murdoch, S., Al-adhami, A. S., Viswanathan, S., Smith, S., Jennings, P., Bowers, D., Soomal, R., Mutala, T. M., Odhiambo, A. O., Harish, N., Hall, M., Sproule, M., Sheridan, S., Thein, K. Y., Tan, C. H., Thian, Y. L., Ho, C. M., De Luca, S., Carrera, C., Blanchet, V., Alarcón, L., Eyheremnedy, E., Choudhury, B. K., Bujarbarua, K., Barman, G., Lovat, E., Ferner, R., Warbey, V. S., Potti, L., Kaye, B., Beattie, A., Dutton, K., Seth, A. A., Constantinidis, F., Dobson, H., Bradley, R., Bozas, G., Avery, G., Stephens, A., Maraveyas, A., Bhuva, S., Johnson, C. A., Subesinghe, M., Taylor, N., Quint, L. E., Reddy, R. M., Kalemkerian, G. P., Zapico, G. González, Jauregui, E. Gainza, Francisco, R. Álvarez, Alonso, S. Ibáñez, Bahillo, I. Tavera, Álvarez, L. Múgica, Francies, O., Wheeler, R., Childs, L., Adams, A., Sahdev, A., De Luca, S. E., Vañek, M. E. Casalini, Pascuzzi, M. D., Gillanders, T., Ramos, P. M., Eyheremendy, E. P., Stove, C., Digby, M., Nazar, M., Wirtz, M., Troncoso, F., Saguier, F., Quint, D. J., Dang, L., Carlson, M., Leber, S., Silverstein, F., Rueben, R., Nazir, B., Teo, T. H., Khoo, J. B., Sharma, K., Gupta, N., Mathew, B., Jeyakumar, T., Harkins, K., Joshua, S., Christodoulou, D., Gourtsoyianni, S., Jacques, A., Griffin, N., Lee, J., Goodfellow, J. A., Yong, A., Jenkins, S., Joseph, G., Partington, K., Zanfardini, A., Cavanagh, K., and Lau, E.

Subjects
lcsh:Medical physics. Medical radiology. Nuclear medicine, Radiological and Ultrasound Technology, Oncology, Radiology Nuclear Medicine and imaging, lcsh:R895-920, lcsh:Neoplasms. Tumors. Oncology. Including cancer and carcinogens, Meeting Abstracts, lcsh:RC254-282
Abstract

Table of contents O1 Tumour heterogeneity: what does it mean? Dow-Mu Koh O2 Skeletal sequelae in adult survivors of childhood cancer Sue Creviston Kaste O3 Locoregional effects of breast cancer treatment Sarah J Vinnicombe O4 Imaging of cancer therapy-induced CNS toxicity Giovanni Morana, Andrea Rossi O5 Screening for lung cancer Christian J. Herold O6Risk stratification of lung nodules Theresa C. McLoud O7 PET imaging of pulmonary nodules Kirk A Frey O8 Transarterial tumour therapy Bernhard Gebauer O9 Interventional radiology in paediatric oncology Derek Roebuck O10 Image guided prostate interventions Jurgen J. Fütterer O11 Imaging cancer predisposition syndromes Alexander J. Towbin O12Chest and chest wall masses Thierry AG Huisman O13 Abdominal masses: good or bad? Anne MJB Smets O14 Hepatobiliary MR contrast: enhanced liver MRI for HCC diagnosis and management Giovanni Morana O15 Role of US elastography and multimodality fusion for managing patients with chronic liver disease and HCC Jeong Min Lee O16 Opportunities and challenges in imaging metastatic disease Hersh Chandarana O17 Diagnosis, treatment monitoring, and follow-up of lymphoma Marius E. Mayerhoefer, Markus Raderer, Alexander Haug O18 Managing high-risk and advanced prostate cancer Matthias Eiber O19 Immunotherapy: imaging challenges Bernhard Gebauer O20 RECIST and RECIST 1.1 Andrea Rockall O21 Challenges of RECIST in oncology imaging basics for the trainee and novice Aslam Sohaib O22 Lymphoma: PET for interim and end of treatment response assessment: a users’ guide to the Deauville Score Victoria S Warbey O23 Available resources Hebert Alberto Vargas O24 ICIS e-portal and the online learning community Dow-Mu Koh O25 Benign lesions that mimic pancreatic cancer Jay P Heiken O26 Staging and reporting pancreatic malignancies Isaac R Francis, Mahmoud, M Al-Hawary, Ravi K Kaza O27 Intraductal papillary mucinous neoplasm Giovanni Morana O28 Cystic pancreatic tumours Mirko D’Onofrio O29 Diffusion-weighted imaging of head and neck tumours Harriet C. Thoeny O30 Radiation injury in the head and neck Ann D King O31 PET/MR of paediatric brain tumours Giovanni Morana, Arnoldo Piccardo, Maria Luisa Garrè, Andrea Rossi O32 Structured reporting and beyond Hebert Alberto Vargas O33 Massachusetts General Hospital experience with structured reporting Theresa C. McLoud O34 The oncologist’s perspective: what the oncologist needs to know Nick Reed O35 Towards the cure of all children with cancer: global initiatives in pediatric oncology Carlos Rodriguez-Galindo O36 Multiparametric imaging of renal cancers Hersh Chandarana O37 Linking imaging features of renal disease and their impact on management strategies Hebert Alberto Vargas O38 Adrenals, retroperitoneum and peritoneum Isaac R Francis, Ashish P Wasnik O39 Lung and pleura Stefan Diederich O40 Advances in MRI Jurgen J. Fütterer O41 Advances in molecular imaging Wim J.G. Oyen O42 Incorporating advanced imaging, impact on treatment selection and patient outcome Cheng Lee Chaw, Nicholas van As S1 Combining ADC-histogram features improves performance of MR diffusion-weighted imaging for Lymph node characterisation in cervical cancer Igor Vieira, Frederik De Keyzer, Elleke Dresen, Sileny Han, Ignace Vergote, Philippe Moerman, Frederic Amant, Michel Koole, Vincent Vandecaveye S2 Whole-body diffusion-weighted MRI for surgical planning in patients with colorectal cancer and peritoneal metastases R Dresen, S De Vuysere, F De Keyzer, E Van Cutsem, A D’Hoore, A Wolthuis, V Vandecaveye S3 Role of apparent diffusion coefficient (ADC) diffusion-weighted MRI for predicting extra capsular extension of prostate cancer. P. Pricolo (paola.pricolo@ieo.it), S. Alessi, P. Summers, E. Tagliabue, G. Petralia S4 Generating evidence for clinical benefit of PET/CT – are management studies sufficient as surrogate for patient outcome? C. Pfannenberg, B. Gückel, SC Schüle, AC Müller, S. Kaufmann, N. Schwenzer, M. Reimold,C. la Fougere, K. Nikolaou, P. Martus S5 Heterogeneity of treatment response in skeletal metastases from breast cancer with 18F-fluoride and 18F-FDG PET GJ Cook, GK Azad, BP Taylor, M Siddique, J John, J Mansi, M Harries, V Goh S6 Accuracy of suspicious breast imaging—can we tell the patient? S Seth, R Burgul, A Seth S7 Measurement method of tumour volume changes during neoadjuvant chemotherapy affects ability to predict pathological response S Waugh, N Muhammad Gowdh, C Purdie, A Evans, E Crowe, A Thompson, S Vinnicombe S8 Diagnostic yield of CT IVU in haematuria screening F. Arfeen, T. Campion, E. Goldstraw S9 Percutaneous radiofrequency ablation of unresectable locally advanced pancreatic cancer: preliminary results D’Onofrio M, Ciaravino V, Crosara S, De Robertis R, Pozzi Mucelli R S10 Iodine maps from dual energy CT improve detection of metastases in staging examinations of melanoma patients M. Uhrig, D. Simons, H. Schlemmer S11Can contrast enhanced CT predict pelvic nodal status in malignant melanoma of the lower limb? Kate Downey S12 Current practice in the investigation for suspected Paraneoplastic Neurological Syndromes (PNS) and positive malignancy yield. S Murdoch, AS Al-adhami, S Viswanathan P1 Technical success and efficacy of Pulmonary Radiofrequency ablation: an analysis of 207 ablations S Smith, P Jennings, D Bowers, R Soomal P2 Lesion control and patient outcome: prospective analysis of radiofrequency abaltion in pulmonary colorectal cancer metastatic disease S Smith, P Jennings, D Bowers, R Soomal P3 Hepatocellular carcinoma in a post-TB patient: case of tropical infections and oncologic imaging challenges TM Mutala, AO Odhiambo, N Harish P4 Role of apparent diffusion coefficient (ADC) diffusion-weighted MRI for predicting extracapsular extension of prostate cancer P. Pricolo, S. Alessi, P. Summers, E. Tagliabue, G. Petralia P5 What a difference a decade makes; comparison of lung biopsies in Glasgow 2005 and 2015 M. Hall, M. Sproule, S. Sheridan P6 Solid pseudopapillary tumour of pancreas: imaging features of a rare neoplasm KY Thein, CH Tan, YL Thian, CM Ho P7 MDCT - pathological correlation in colon adenocarcinoma staging: preliminary experience S De Luca, C Carrera, V Blanchet, L Alarcón, E Eyheremnedy P8 Image guided biopsy of thoracic masses and reduction of pneumothorax risk: 25 years experience B K Choudhury, K Bujarbarua, G Barman P9 Tumour heterogeneity analysis of 18F-FDG-PET for characterisation of malignant peripheral nerve sheath tumours in neurofibromatosis-1 GJ Cook, E Lovat, M Siddique, V Goh, R Ferner, VS Warbey P10 Impact of introduction of vacuum assisted excision (VAE) on screen detected high risk breast lesions L Potti, B Kaye, A Beattie, K Dutton P11 Can we reduce prevalent recall rate in breast screening? AA Seth, F Constantinidis, H Dobson P12 How to reduce prevalent recall rate? Identifying mammographic lesions with low Positive Predictive Value (PPV) AA Seth (archana.seth@nhs.net), F Constantinidis, H Dobson P13 Behaviour of untreated pulmonary thrombus in oncology patients diagnosed with incidental pulmonary embolism on CT R. Bradley, G. Bozas, G. Avery, A. Stephens, A. Maraveyas P14 A one-stop lymphoma biopsy service – is it possible? S Bhuva, CA Johnson, M Subesinghe, N Taylor P15 Changes in the new TNM classification for lung cancer (8th edition, effective January 2017) LE Quint, RM Reddy, GP Kalemkerian P16 Cancer immunotherapy: a review of adequate imaging assessment G González Zapico, E Gainza Jauregui, R Álvarez Francisco, S Ibáñez Alonso, I Tavera Bahillo, L Múgica Álvarez P17 Succinate dehydrogenase mutations and their associated tumours O Francies, R Wheeler, L Childs, A Adams, A Sahdev P18 Initial experience in the usefulness of dual energy technique in the abdomen SE De Luca, ME Casalini Vañek, MD Pascuzzi, T Gillanders, PM Ramos, EP Eyheremendy P19 Recognising the serious complication of Richter’s transformation in CLL patients C Stove, M Digby P20 Body diffusion-weighted MRI in oncologic practice: truths, tricks and tips M. Nazar, M. Wirtz, MD. Pascuzzi, F. Troncoso, F. Saguier, EP. Eyheremendy P21 Methotrexate-induced leukoencephalopathy in paediatric ALL Patients D.J. Quint, L. Dang, M. Carlson, S. Leber, F. Silverstein P22 Pitfalls in oncology CT reporting. A pictorial review R Rueben, S Viswanathan P23 Imaging of perineural extension in head and neck tumours B Nazir, TH Teo, JB Khoo P24 MRI findings of molecular subtypes of breast cancer: a pictorial primer K Sharma, N Gupta, B Mathew, T Jeyakumar, K Harkins P25 When cancer can’t wait! A pictorial review of oncological emergencies K Sharma, B Mathew, N Gupta, T Jeyakumar, S Joshua P26 MRI of pancreatic neuroendocrine tumours: an approach to interpretation D Christodoulou, S Gourtsoyianni, A Jacques, N Griffin, V Goh P27 Gynaecological cancers in pregnancy: a review of imaging CA Johnson, J Lee P28 Suspected paraneoplastic neurological syndromes - review of published recommendations to date, with proposed guideline/flowchart JA Goodfellow, AS Al-adhami, S Viswanathan P29 Multi-parametric MRI of the pelvis for suspected local recurrence of prostate cancer after radical prostatectomy R Bradley P30 Utilisation of PI-RADS version 2 in multi-parametric MRI of the prostate; 12-months experience R Bradley P31 Radiological assessment of the post-chemotherapy liver A Yong, S Jenkins, G Joseph P32 Skeletal staging with MRI in breast cancer – what the radiologist needs to know S Bhuva, K Partington P33 Perineural spread of lympoma: an educational review of an unusual distribution of disease CA Johnson, S Bhuva, M Subesinghe, N Taylor P34 Visually isoattenuating pancreatic adenocarcinoma. Diagnostic imaging tools. C Carrera, A Zanfardini, S De Luca, L Alarcón, V Blanchet, EP Eyheremendy P35 Imaging of larynx cancer: when is CT, MRI or FDG PET/CT the best test? K Cavanagh, E Lau

Published
2016

366. Neonatal alveolar rhabdomyosarcoma with skin and brain metastases

Author

Rodriguez-Galindo, Carlos, Hill, D. Ashley, Onyekwere, Onynye, Pin, Noel, Rao, Bhaskar N., Hoffer, Fredric A., Kun, Larry E., Pappo, Alberto S., and Santana, Victor M.

Subjects
Rhabdomyosarcoma -- Case studies, Infants (Newborn) -- Diseases, Health
Published
2001

367. Interdisciplinary care of pediatric oncology patients in Central America and the Caribbean.

Author

Graetz, Dylan E., Chen, Yichen, Devidas, Meenakshi, Antillon‐Klussmann, Federico, Fu, Ligia, Quintero, Karina, Fuentes‐Alabi, Soad L., Gassant, Pascale Y., Kaye, Erica C., Baker, Justin N., Rodriguez Galindo, Carlos, and Mack, Jennifer W.

Subjects
PEDIATRIC therapy, PEDIATRIC oncology, CHILD patients, CANCER treatment, JOB satisfaction, ONCOLOGISTS, PEDIATRIC nursing
Abstract

Background: Interdisciplinary teamwork supports high‐quality cancer care and effective utilization of limited resources. This study purposed to examine the value, structure, process, and effectiveness of interdisciplinary care (IDC) among pediatric oncology providers in low‐income and middle‐income countries in Central America and the Caribbean. Methods: A cross‐sectional survey was disseminated to pediatric oncology providers at 5 centers participating in the Pediatric Hematology‐Oncology Association of Central America. The survey included previously validated items and novel questions assessing the value (importance), structure (multidisciplinary meeting attendance), process (team climate), and effectiveness (job satisfaction, quality of care and communication) of IDC. Results: The survey was completed by 174 providers, including 22 oncologists, 9 pathologists, 9 radiologists, 5 radiation oncologists, 12 surgeons, 35 subspecialists, 60 nurses, 20 psychosocial providers, and 2 other staff. Participants agreed that IDC benefits team members (95%) and patients (96%). IDC structure and processes varied across the region. Multidisciplinary meeting attendance differed by center (P =.005) and discipline (P <.0001). Participants who frequently attended multidisciplinary meetings reported a more positive team climate (P =.0003). Team climate was positively associated with job satisfaction (P <.001). In multivariable analyses, team climate was predictive of an improved perception of communication between professionals (P <.0001), with families (P <.0001), and with patients (P =.0005), as well as with quality of the care environment (P =.006) and overall care quality (P <.0001). Conclusions: Nearly all surveyed participants valued IDC, and the structure and processes supporting IDC varied by center. Associations between a collaborative professional climate, job satisfaction, and the perception of quality care encourage continued investigation and prioritization of IDC in these settings. Structures and processes supporting interdisciplinary care for pediatric oncology patients in Central America and the Caribbean vary. Associations between a collaborative professional climate, job satisfaction, and perception of quality care support the continued investigation and prioritization of interdisciplinary care in these settings. [ABSTRACT FROM AUTHOR]

Published
2021
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368. Hemangiopericytoma in children and infants

Author

Rodriguez-Galindo, Carlos, Ramsey, Kirk, Jenkins, Jesse J., Poquette, Catherine A., Kaste, Sue C., Merchant, Thomas E., Rao, Bhaskar N., Pratt, Charles B., and Pappo, Alberto S.

Subjects
Hemangioma -- Care and treatment, Tumors in children -- Care and treatment, Health
Published
2000

369. Clinical and mutational spectrum of highly differentiated, PAX:FOXO1 fusion-negative rhabdomyosarcoma – a report from the Children’s Oncology Group

Author

Teot, Lisa A., Schneider, Michaela, Thorner, Aaron R., Tian, Jing, Chi, Yueh-Yun, Ducar, Matthew, Lin, Ling, Wlodarski, Marcin, Grier, Holcombe E, Fletcher, Christopher DM, van Hummelen, Paul, Skapek, Stephen X, Hawkins, Douglas S, Wagers, Amy J, Rodriguez-Galindo, Carlos, and Hettmer, Simone

Subjects
Adult, Male, Adolescent, Oncogene Proteins, Fusion, DNA Mutational Analysis, Article, Young Adult, Rhabdomyosarcoma, Humans, Paired Box Transcription Factors, Hedgehog Proteins, Child, Muscle Cells, Muscles, TOR Serine-Threonine Kinases, Infant, Cell Differentiation, Survival Rate, Head and Neck Neoplasms, Child, Preschool, Mutation, ras Proteins, Female, Urogenital Neoplasms, Follow-Up Studies, Signal Transduction
Abstract

BACKGROUND: Pediatric PAX:FOXO1 fusion-negative rhabdomyosarcoma represents a diverse spectrum of tumors with marked differences in histology, myogenic differentiation and clinical behavior. METHODS: This study sought to evaluate the clinical and mutational spectrum of 24 pediatric PAX:FOXO1 fusion-negative human rhabdomyosarcomas with high levels of myogenic differentiation. Tumors were sequenced using OncoPanel v.2 (OPv2), a panel consisting of the coding regions of 504 genes, previously linked to human cancer. RESULTS: Most (19 of 24) tumors arose at head/ neck or genitourinary sites, and overall survival was 100% with a median followup time of 4.6 years (range: 1.4 to 8.6 years). RAS pathway gene mutations were the most common mutations in PAX:FOXO1 fusion-negative, highly differentiated rhabdomyosarcomas. In addition, Hedgehog (Hh) and mechanistic Target of Rapamycin (mTOR) gene mutations with evidence for functional relevance (high-impact) were identified in subsets of tumors. The presence of Hh and mTOR pathway gene mutations was mutually exclusive, and associated with high-impact RAS pathway gene mutations in 3 of 4 Hh-mutated and 1 of 6 mTOR-mutated tumors. CONCLUSIONS: Interestingly, Hh and mTOR gene mutations were previously associated with rhabdomyomas, which are also known to preferentially arise at head/neck and genitourinary sites. Findings from this study further support that PAX:FOXO1 fusion-negative, highly differentiated rhabdomyosarcoma and rhabdomyoma may represent a continuous spectrum of tumors.

Published
2018

370. The global burden of childhood and adolescent cancer in 2017: an analysis of the Global Burden of Disease Study 2017

Author

Force, Lisa M., primary, Abdollahpour, Ibrahim, additional, Advani, Shailesh M, additional, Agius, Dominic, additional, Ahmadian, Elham, additional, Alahdab, Fares, additional, Alam, Tahiya, additional, Alebel, Animut, additional, Alipour, Vahid, additional, Allen, Christine A., additional, Almasi-Hashiani, Amir, additional, Alvarez, Elysia M, additional, Amini, Saeed, additional, Amoako, Yaw Ampem, additional, Anber, Nahla Hamed, additional, Arabloo, Jalal, additional, Artaman, Al, additional, Atique, Suleman, additional, Awasthi, Ashish, additional, Bagherzadeh, Mojtaba, additional, Basaleem, Huda, additional, Bekru, Eyasu Tamru, additional, Bijani, Ali, additional, Bogale, Kassawmar Angaw, additional, Car, Mate, additional, Carvalho, Félix, additional, Castro, Clara, additional, Catalá-López, Ferrán, additional, Chu, Dinh-Toi, additional, Costa, Vera M, additional, Darwish, Amira Hamed, additional, Demeke, Feleke Mekonnen, additional, Demis, Asmamaw Bizuneh, additional, Demoz, Gebre Teklemariam, additional, Dharmaratne, Samath Dhamminda, additional, Do, Huyen Phuc, additional, Doan, Linh Phuong, additional, Dubey, Manisha, additional, Eftekhari, Aziz, additional, El-Khatib, Ziad, additional, Emamian, Mohammad Hassan, additional, Farhangi, Mahdieh Abbasalizad, additional, Fernandes, Eduarda, additional, Fischer, Florian, additional, Fouladi Fard, Reza, additional, Friedrich, Paola M, additional, Fukumoto, Takeshi, additional, Gedefaw, Getnet Azeze, additional, Ghashghaee, Ahmad, additional, Gholamian, Asadollah, additional, Haj-Mirzaian, Arvin, additional, Haj-Mirzaian, Arya, additional, Hamidi, Samer, additional, Harvey, James D., additional, Hassen, Hamid Yimam, additional, Hay, Simon I., additional, Hoang, Chi Linh, additional, Hole, Michael K., additional, Horita, Nobuyuki, additional, Hosseini, Seyyed Nasrollah, additional, Hosseinzadeh, Mehdi, additional, Hsairi, Mohamed, additional, Hudson, Melissa Maria, additional, Innos, Kaire, additional, Jalilian, Farzad, additional, James, Spencer L., additional, Kasaeian, Amir, additional, Kassa, Tesfaye Dessale, additional, Kassebaum, Nicholas J, additional, Keiyoro, Peter Njenga, additional, Khader, Yousef Saleh, additional, Khubchandani, Jagdish, additional, Kianipour, Neda, additional, Kirby, Jeannette, additional, Kisa, Adnan, additional, Kisa, Sezer, additional, Kocarnik, Jonathan M, additional, Lauriola, Paolo, additional, Lopez, Alan D, additional, Mägi, Margit, additional, Malik, Manzoor Ahmad, additional, Manafi, Ali, additional, Manafi, Navid, additional, Mansournia, Mohammad Ali, additional, Massenburg, Benjamin Ballard, additional, Mehta, Varshil, additional, Meles, Hagazi Gebre, additional, Meretoja, Tuomo J, additional, Mestrovic, Tomislav, additional, Mir, Seyed Mostafa, additional, Mirzaei-Alavijeh, Mehdi, additional, Mohammad, Dara K., additional, Mohammad Darwesh, Aso, additional, Mohammad Gholi Mezerji, Naser, additional, Mohammadibakhsh, Roghayeh, additional, Mohammadoo-Khorasani, Milad, additional, Mokdad, Ali H, additional, Moodley, Yoshan, additional, Moosazadeh, Mahmood, additional, Moossavi, Maryam, additional, Moradpour, Farhad, additional, Morrison, Shane Douglas, additional, Muchie, Kindie Fentahun, additional, Naghavi, Mohsen, additional, Nazari, Javad, additional, Ngunjiri, Josephine W., additional, Nguyen, Cuong Tat, additional, Nguyen, Long Hoang, additional, Nguyen, Son Hoang, additional, Nguyen, Trang Huyen, additional, Nixon, Molly R, additional, Olagunju, Andrew T, additional, Olagunju, Tinuke O, additional, Ong, Sok King, additional, Paapsi, Keiu, additional, Pennini, Alyssa, additional, Pereira, David M., additional, Pillay, Julian David, additional, Qorbani, Mostafa, additional, Rabiee, Mohammad, additional, Rabiee, Navid, additional, Raoofi, Samira, additional, Rawaf, David Laith, additional, Rawaf, Salman, additional, Reiner, Robert C, additional, Rezaei, Nima, additional, Rezapour, Aziz, additional, Roba, Kedir Teji, additional, Robison, Les L., additional, Rodriguez-Galindo, Carlos, additional, Roshandel, Gholamreza, additional, Safiri, Saeid, additional, Salahshoor, Mohamadreza, additional, Salehi Zahabi, Saleh, additional, Samy, Abdallah M., additional, Santric Milicevic, Milena M, additional, Satpathy, Maheswar, additional, Sawyer, Susan M, additional, Seyedmousavi, Seyedmojtaba, additional, Shabaninejad, Hosein, additional, Shaikh, Masood Ali, additional, Shamshirian, Amir, additional, Shamsizadeh, Morteza, additional, Shirkoohi, Reza, additional, Siabani, Soraya, additional, Singh, Jasvinder A., additional, SiRamlee, Khairil, additional, Tabarés-Seisdedos, Rafael, additional, Temsah, Mohamad-Hani, additional, Tran, Bach Xuan, additional, Ullah, Irfan, additional, Vahedian-Azimi, Amir, additional, Vollset, Stein Emil, additional, Vos, Theo, additional, Waheed, Yasir, additional, Weldesamuel, Girmay Teklay, additional, Workie, Hailemariam Mekonnen, additional, Xu, Rixing, additional, Yaseri, Mehdi, additional, Yonemoto, Naohiro, additional, Yu, Chuanhua, additional, Zadnik, Vesna, additional, Zahirian Moghadam, Telma, additional, Zaidi, Zoubida, additional, Zangeneh, Alireza, additional, Zewale, Taye Abuhay, additional, Ziapour, Arash, additional, Zodpey, Sanjay, additional, Murray, Christopher J L, additional, Fitzmaurice, Christina, additional, and Bhakta, Nickhill, additional

Published
2019
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372. α‐Fetoprotein as a predictor of outcome for children with germ cell tumors: A report from the Malignant Germ Cell International Consortium

Author

O’Neill, Allison F., primary, Xia, Caihong, additional, Krailo, Mark D., additional, Shaikh, Furqan, additional, Pashankar, Farzana D., additional, Billmire, Deborah F., additional, Olson, Thomas A., additional, Amatruda, Jim F., additional, Villaluna, Doojduen, additional, Huang, Li, additional, Malogolowkin, Marcio, additional, Rodriguez‐Galindo, Carlos, additional, and Frazier, A. Lindsay, additional

Published
2019
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373. Challenges in the diagnosis of hemophagocytic lymphohistiocytosis: Recommendations from the North American Consortium for Histiocytosis (NACHO)

Author

Jordan, Michael B., primary, Allen, Carl E., additional, Greenberg, Jay, additional, Henry, Michael, additional, Hermiston, Michelle L., additional, Kumar, Ashish, additional, Hines, Melissa, additional, Eckstein, Olive, additional, Ladisch, Stephan, additional, Nichols, Kim E., additional, Rodriguez‐Galindo, Carlos, additional, Wistinghausen, Birte, additional, and McClain, Kenneth L., additional

Published
2019
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375. Alfa-feto protein (AFP) as a predictor of outcome for children with germ cell tumors: A report from the malignant germ cell international consortium.

Author

O'Neill, Allison Frances, primary, Xia, Caihong, additional, Krailo, Mark D., additional, Shaikh, Furqan, additional, Pashankar, Farzana D., additional, Billmire, Deborah F, additional, Olson, Thomas Arthur, additional, Amatruda, James F., additional, Villaluna, Doojduen, additional, Malogolowkin, Marcio H., additional, Rodriguez-Galindo, Carlos, additional, and Frazier, A. Lindsay, additional

Published
2019
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377. Outcomes of adolescent males with extracranial malignant germ cell tumors compared with children and young adults: A report from the Malignant Germ Cell Tumors International Consortium (MaGIC) group.

Author

Shaikh, Furqan, primary, Stark, Daniel P., additional, Dang, Ha, additional, Xia, Caihong, additional, Krailo, Mark D., additional, Stenning, Sally Patricia, additional, Pashankar, Farzana D., additional, Rodriguez-Galindo, Carlos, additional, Olson, Thomas Arthur, additional, Hale, Juliet, additional, Depani, Sarita, additional, Stoneham, Sara, additional, Nicholson, James, additional, Murray, Matthew, additional, Amatruda, James F., additional, Billmire, Deborah F, additional, Fonseca, Adriana, additional, and Frazier, A. Lindsay, additional

Published
2019
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378. Minimal adjuvant chemotherapy for children with hepatoblastoma resected at diagnosis (AHEP0731): a Children's Oncology Group, multicentre, phase 3 trial

Author

Katzenstein, Howard M, primary, Langham, Max R, additional, Malogolowkin, Marcio H, additional, Krailo, Mark D, additional, Towbin, Alexander J, additional, McCarville, Mary Beth, additional, Finegold, Milton J, additional, Ranganathan, Sarangarajan, additional, Dunn, Stephen, additional, McGahren, Eugene D, additional, Tiao, Gregory M, additional, O'Neill, Allison F, additional, Qayed, Muna, additional, Furman, Wayne L, additional, Xia, Caihong, additional, Rodriguez-Galindo, Carlos, additional, and Meyers, Rebecka L, additional

Published
2019
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379. Clinical Cancer Advances 2019: Annual Report on Progress Against Cancer From the American Society of Clinical Oncology

Author

Pal, Sumanta K., primary, Miller, Michael J., additional, Agarwal, Neeraj, additional, Chang, Susan Marina, additional, Chavez-MacGregor, Mariana, additional, Cohen, Ezra, additional, Cole, Suzanne, additional, Dale, William, additional, Magid Diefenbach, Catherine S., additional, Disis, Mary L., additional, Dreicer, Robert, additional, Graham, David L., additional, Henry, N. Lynn, additional, Jones, Joshua, additional, Keedy, Vicki, additional, Klepin, Heidi D., additional, Markham, Merry Jennifer, additional, Mittendorf, Elizabeth A., additional, Rodriguez-Galindo, Carlos, additional, Sabel, Michael S., additional, Schilsky, Richard L., additional, Sznol, Mario, additional, Tap, William D., additional, Westin, Shannon Neville, additional, and Johnson, Bruce E., additional

Published
2019
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380. Detection of Relapse by Tumor Markers Versus Imaging in Children and Adolescents With Nongerminomatous Malignant Germ Cell Tumors: A Report From the Children’s Oncology Group

Author

Fonseca, Adriana, primary, Xia, Caihong, additional, Lorenzo, Armando J., additional, Krailo, Mark, additional, Olson, Thomas A., additional, Pashankar, Farzana, additional, Malogolowkin, Marcio H., additional, Amatruda, James F., additional, Billmire, Deborah F., additional, Rodriguez-Galindo, Carlos, additional, Frazier, A. Lindsay, additional, and Shaikh, Furqan, additional

Published
2019
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381. Spatial trends in congenital malformations and stream water chemistry in Southern Brazil

Author

Ibañez, Humberto C., primary, Melanda, Viviane S., additional, Gerber, Viviane K.Q., additional, Licht, Otavio A.B., additional, Ibañez, Marilea V.C., additional, Aguiar Júnior, Terêncio R., additional, Mello, Rosiane G., additional, Komechen, Heloisa, additional, Andrade, Diancarlos P., additional, Picharski, Gledson L., additional, Figueiredo, Damasio P.G., additional, Pianovski, Mara A.D., additional, Figueiredo, Mirna M.O., additional, Custódio, Gislaine, additional, Parise, Ivy Z.S., additional, Castilho, Laura M., additional, Paraizo, Mariana M., additional, Edinger, Chloe, additional, Fiori, Carmem M.C.M., additional, Pedrini, Hélio, additional, Kiesel Filho, Nilton, additional, Fabro, Ana Luiza M.R., additional, Fachin, Rayssa D., additional, Ogradowski, Karin R.P., additional, Parise, Guilherme A., additional, Saldiva, Paulo H.N., additional, Legal, Edith F., additional, Rosati, Roberto, additional, Rodriguez-Galindo, Carlos, additional, Ribeiro, Raul C., additional, Zambetti, Gerard P., additional, Lalli, Enzo, additional, and Figueiredo, Bonald C., additional

Published
2019
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383. α-Fetoprotein as a predictor of outcome for children with germ cell tumors: A report from the Malignant Germ Cell International Consortium.

Author

O'Neill, Allison F., Xia, Caihong, Krailo, Mark D., Shaikh, Furqan, Pashankar, Farzana D., Billmire, Deborah F., Olson, Thomas A., Amatruda, Jim F., Villaluna, Doojduen, Huang, Li, Malogolowkin, Marcio, Rodriguez‐Galindo, Carlos, Frazier, A. Lindsay, and Rodriguez-Galindo, Carlos

Subjects
TERATOCARCINOMA, GERM cells, TUMOR markers, CHILDREN, CONSORTIA, GERM cell tumors
Abstract

Background: There are several studies describing the correlation between unsatisfactory tumor marker decline and a poor prognosis for adult patients treated for germ cell tumors. In pediatric patients, the data are limited. Therefore, this study retrospectively analyzed data from Children's Oncology Group (COG) protocol AGCT0132 to determine whether a relationship exists between α-fetoprotein (AFP) decline and outcome.Methods: One hundred thirty-one patients with germ cell tumors who were enrolled in COG protocol AGCT0132 were eligible for this analysis of AFP decline. The serum AFP half-life was calculated from levels collected postoperatively as a baseline and after the start of chemotherapy. AFP decline was defined as automatically satisfactory (AFP normalized within the first 2 AFP measures after the start of chemotherapy), calculated satisfactory (AFP half-life ≤7 days after the start of chemotherapy), and unsatisfactory.Results: The 3-year cumulative incidence of relapse was 11% (95% confidence interval [CI], 6.0%-18%) for patients with a satisfactory decline and 38% (95% CI, 13%-64%) for patients with an unsatisfactory decline (P = .006). In stratified analyses, this effect was limited to patients who were 11 years of age or older and had standard risk 2 (SR2) disease (P = .004 and P = .007, respectively). Three-year overall survival (OS) for patients with a satisfactory decline versus an unsatisfactory decline was not statistically significant.Conclusions: This study is the first to show an association between AFP decline and the cumulative incidence of relapse in pediatric patients treated for germ cell tumors. Recognition of patients at high risk for relapse may allow for early intensification of therapy, which could affect future clinical trial design. [ABSTRACT FROM AUTHOR]

Published
2019
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385. Improving Immunohistochemistry Capability for Pediatric Cancer Care in the Central American and Caribbean Region: A Report From the AHOPCA Pathology Working Group

Author

Santiago, Teresa, primary, Hayes, Caleb, additional, Polanco, Ana Concepción, additional, Miranda, Lisa, additional, Aybar, Argelia, additional, Gomero, Belkis, additional, Orellana, Elizabeth, additional, Anglade, Fabienne, additional, Toledo González, Mázlova Luxely, additional, Ruiz, Eduviges, additional, Espino-Durán, Moisés, additional, Rodriguez-Galindo, Carlos, additional, and Metzger, Monika L., additional

Published
2018
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386. Brain metastases in children with melanoma

Author

Rodriguez-Galindo, Carlos, Pappo, Alberto S., Kaste, Sue C., Rao, Baskar N., Cain, Alvida, Jenkins, Jesse J., Kun, Larry E., and Pratt, Charles B.

Subjects
Melanoma -- Metastasis, Brain cancer -- Prognosis, Health
Published
1997

388. CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.

Author

McClain, Kenneth L, McClain, Kenneth L, Picarsic, Jennifer, Chakraborty, Rikhia, Zinn, Daniel, Lin, Howard, Abhyankar, Harshal, Scull, Brooks, Shih, Albert, Lim, Karen Phaik Har, Eckstein, Olive, Lubega, Joseph, Peters, Tricia L, Olea, Walter, Burke, Thomas, Ahmed, Nabil, Hicks, M John, Tran, Brandon, Jones, Jeremy, Dauser, Robert, Jeng, Michael, Baiocchi, Robert, Schiff, Deborah, Goldman, Stanton, Heym, Kenneth M, Wilson, Harry, Carcamo, Benjamin, Kumar, Ashish, Rodriguez-Galindo, Carlos, Whipple, Nicholas S, Campbell, Patrick, Murdoch, Geoffrey, Kofler, Julia, Heales, Simon, Malone, Marian, Woltjer, Randy, Quinn, Joseph F, Orchard, Paul, Kruer, Michael C, Jaffe, Ronald, Manz, Markus G, Lira, Sergio A, Parsons, D Williams, Merad, Miriam, Man, Tsz-Kwong, Allen, Carl E, McClain, Kenneth L, McClain, Kenneth L, Picarsic, Jennifer, Chakraborty, Rikhia, Zinn, Daniel, Lin, Howard, Abhyankar, Harshal, Scull, Brooks, Shih, Albert, Lim, Karen Phaik Har, Eckstein, Olive, Lubega, Joseph, Peters, Tricia L, Olea, Walter, Burke, Thomas, Ahmed, Nabil, Hicks, M John, Tran, Brandon, Jones, Jeremy, Dauser, Robert, Jeng, Michael, Baiocchi, Robert, Schiff, Deborah, Goldman, Stanton, Heym, Kenneth M, Wilson, Harry, Carcamo, Benjamin, Kumar, Ashish, Rodriguez-Galindo, Carlos, Whipple, Nicholas S, Campbell, Patrick, Murdoch, Geoffrey, Kofler, Julia, Heales, Simon, Malone, Marian, Woltjer, Randy, Quinn, Joseph F, Orchard, Paul, Kruer, Michael C, Jaffe, Ronald, Manz, Markus G, Lira, Sergio A, Parsons, D Williams, Merad, Miriam, Man, Tsz-Kwong, and Allen, Carl E

Abstract

BackgroundCentral nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH.MethodsCerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Additionally, the presence of BRAFV600E was tested in peripheral mononuclear blood cells (PBMCs) as well as brain biopsies from LCH-ND patients, and the response to BRAF-V600E inhibitor was evaluated in 4 patients with progressive disease.ResultsOsteopontin was the only consistently elevated CSF protein in patients with CNS-LCH compared with patients with other brain pathologies. BRAFV600E DNA was detected in CSF of only 2/20 (10%) cases, both with LCH-ND and active lesions outside the CNS. However, BRAFV600E+ PBMCs were detected with significantly higher frequency at all stages of therapy in LCH patients who developed LCH-ND. Brain biopsies of patients with LCH-ND demonstrated diffuse perivascular infiltration by BRAFV600E+ cells with monocyte phenotype (CD14+ CD33+ CD163+ P2RY12- ) and associated osteopontin expression. Three of 4 patients with LCH-ND treated with BRAF-V600E inhibitor experienced significant clinical and radiologic improvement.ConclusionIn LCH-ND patients, BRAFV600E+ cells in PBMCs and infiltrating myeloid/monocytic cells in the brain is consistent with LCH-ND as an active demyelinating process arising from a mutated hematopoietic precursor from which LCH lesion CD207+ cells are also derived. Therapy directed against myeloid precursors with activated MAPK signaling may be effective for LCH-ND. Cancer 2018;124:2607-20. © 2018 American Cancer Society.

Published
2018

389. CNS Langerhans cell histiocytosis: Common hematopoietic origin for LCH-associated neurodegeneration and mass lesions.

Author

McClain, Kenneth, McClain, Kenneth, Picarsic, Jennifer, Chakraborty, Rikhia, Zinn, Daniel, Lin, Howard, Abhyankar, Harshal, Scull, Brooks, Shih, Albert, Lim, Karen, Eckstein, Olive, Lubega, Joseph, Peters, Tricia, Olea, Walter, Burke, Thomas, Ahmed, Nabil, Hicks, M, Tran, Brandon, Jones, Jeremy, Dauser, Robert, Jeng, Michael, Baiocchi, Robert, Goldman, Stanton, Heym, Kenneth, Wilson, Harry, Carcamo, Benjamin, Kumar, Ashish, Rodriguez-Galindo, Carlos, Whipple, Nicholas, Campbell, Patrick, Murdoch, Geoffrey, Kofler, Julia, Heales, Simon, Malone, Marian, Woltjer, Randy, Quinn, Joseph, Orchard, Paul, Kruer, Michael, Jaffe, Ronald, Manz, Markus, Lira, Sergio, Parsons, D, Merad, Miriam, Man, Tsz-Kwong, Allen, Carl, Schiff, Deborah, McClain, Kenneth, McClain, Kenneth, Picarsic, Jennifer, Chakraborty, Rikhia, Zinn, Daniel, Lin, Howard, Abhyankar, Harshal, Scull, Brooks, Shih, Albert, Lim, Karen, Eckstein, Olive, Lubega, Joseph, Peters, Tricia, Olea, Walter, Burke, Thomas, Ahmed, Nabil, Hicks, M, Tran, Brandon, Jones, Jeremy, Dauser, Robert, Jeng, Michael, Baiocchi, Robert, Goldman, Stanton, Heym, Kenneth, Wilson, Harry, Carcamo, Benjamin, Kumar, Ashish, Rodriguez-Galindo, Carlos, Whipple, Nicholas, Campbell, Patrick, Murdoch, Geoffrey, Kofler, Julia, Heales, Simon, Malone, Marian, Woltjer, Randy, Quinn, Joseph, Orchard, Paul, Kruer, Michael, Jaffe, Ronald, Manz, Markus, Lira, Sergio, Parsons, D, Merad, Miriam, Man, Tsz-Kwong, Allen, Carl, and Schiff, Deborah

Abstract

BACKGROUND: Central nervous system Langerhans cell histiocytosis (CNS-LCH) brain involvement may include mass lesions and/or a neurodegenerative disease (LCH-ND) of unknown etiology. The goal of this study was to define the mechanisms of pathogenesis that drive CNS-LCH. METHODS: Cerebrospinal fluid (CSF) biomarkers including CSF proteins and extracellular BRAFV600E DNA were analyzed in CSF from patients with CNS-LCH lesions compared with patients with brain tumors and other neurodegenerative conditions. Additionally, the presence of BRAFV600E was tested in peripheral mononuclear blood cells (PBMCs) as well as brain biopsies from LCH-ND patients, and the response to BRAF-V600E inhibitor was evaluated in 4 patients with progressive disease. RESULTS: Osteopontin was the only consistently elevated CSF protein in patients with CNS-LCH compared with patients with other brain pathologies. BRAFV600E DNA was detected in CSF of only 2/20 (10%) cases, both with LCH-ND and active lesions outside the CNS. However, BRAFV600E+ PBMCs were detected with significantly higher frequency at all stages of therapy in LCH patients who developed LCH-ND. Brain biopsies of patients with LCH-ND demonstrated diffuse perivascular infiltration by BRAFV600E+ cells with monocyte phenotype (CD14+ CD33+ CD163+ P2RY12- ) and associated osteopontin expression. Three of 4 patients with LCH-ND treated with BRAF-V600E inhibitor experienced significant clinical and radiologic improvement. CONCLUSION: In LCH-ND patients, BRAFV600E+ cells in PBMCs and infiltrating myeloid/monocytic cells in the brain is consistent with LCH-ND as an active demyelinating process arising from a mutated hematopoietic precursor from which LCH lesion CD207+ cells are also derived. Therapy directed against myeloid precursors with activated MAPK signaling may be effective for LCH-ND. Cancer 2018;124:2607-20. © 2018 American Cancer Society.

Published
2018

390. Gonadal dysgenesis is associated with worse outcomes in patients with ovarian nondysgerminomatous tumors: A report of the Children's Oncology Group AGCT 0132 study.

Author

Dicken, Bryan J, Dicken, Bryan J, Billmire, Deborah F, Krailo, Mark, Xia, Caihong, Shaikh, Furqan, Cullen, John W, Olson, Thomas A, Pashankar, Farzana, Malogolowkin, Marcio H, Amatruda, James F, Rescorla, Frederick J, Egler, Rachel A, Ross, Jonathan H, Rodriguez-Galindo, Carlos, Frazier, A Lindsay, Dicken, Bryan J, Dicken, Bryan J, Billmire, Deborah F, Krailo, Mark, Xia, Caihong, Shaikh, Furqan, Cullen, John W, Olson, Thomas A, Pashankar, Farzana, Malogolowkin, Marcio H, Amatruda, James F, Rescorla, Frederick J, Egler, Rachel A, Ross, Jonathan H, Rodriguez-Galindo, Carlos, and Frazier, A Lindsay

Abstract

In this report, we characterize the timing and behavior of malignant ovarian germ cell tumors (GCTs) in pediatric patients with dysgenetic gonads compared to those with normal gonadal development. Patients from the Children's Oncology Group AGCT0132 with malignant ovarian GCTs were included. Within this population, we sought to identify patients with gonadoblastoma, streak ovaries, or other evidence of gonadal dysgenesis (GD). Patients with malignant GCTs containing one or more of the following histologies-yolk sac tumor, embryonal carcinoma, or choriocarcinoma-were included. Patients were compared with respect to event-free survival (EFS) and overall survival (OS). Nine patients with GD, including seven with gonadoblastoma (mean age, 9.3 years), were compared to 100 non-GD patients (mean age, 12.1 years). The estimated 3-year EFS for patients with GD was 66.7% (95% CI 28.2-87.8%) and for non-GD patients was 88.8% (95% CI 80.2-93.8%). The estimated 3-year OS for patients with GD was 87.5% (95% CI 38.7-98.1%) and for non-GD patients was 97.6% (95% CI of 90.6-99.4%). Patients presenting with nongerminomatous malignant ovarian GCTs in the context of GD have a higher rate of events and death than counterparts with normal gonads. These findings emphasize the importance of noting a contralateral streak ovary or gonadoblastoma at histology for any ovarian GCT and support the recommendation for early bilateral gonadectomy in patients known to have GD with Y chromosome material. In contrast to those with pure dysgerminoma, these patients may represent a high-risk group that requires a more aggressive chemotherapy regimen.

Published
2018

392. Upfront window vincristine/irinotecan treatment of high-risk hepatoblastoma: A report from the Children's Oncology Group AHEP0731 study committee

Author

Katzenstein, Howard M, Furman, Wayne L, Malogolowkin, Marcio H, Krailo, Mark D, McCarville, M Beth, Towbin, Alexander J, Tiao, Greg M, Finegold, Milton J, Ranganathan, Sarangarajan, Dunn, Stephen P, Langham, Max R, McGahren, Eugene D, Rodriguez-Galindo, Carlos, and Meyers, Rebecka L

Subjects
Hepatoblastoma, Male, Adolescent, Pediatric Cancer, Oncology and Carcinogenesis, Irinotecan, Rare Diseases, high-risk, Antineoplastic Combined Chemotherapy Protocols, Humans, Hepatectomy, Oncology & Carcinogenesis, Child, Preschool, Cancer, Pediatric, Liver Neoplasms, Infant, Evaluation of treatments and therapeutic interventions, Liver Transplantation, metastatic, Survival Rate, Vincristine, 6.1 Pharmaceuticals, Public Health and Health Services, Camptothecin, Female, alpha-Fetoproteins
Abstract

BackgroundThe identification of new therapies for high-risk (HR) hepatoblastoma is challenging. Children's Oncology Group study AHEP0731 included a HR stratum to explore the efficacy of novel agents. Herein, the authors report the response rate to the combination of vincristine (V) and irinotecan (I) and the outcome of patients with high-risk hepatoblastoma.MethodsPatients with newly diagnosed metastatic hepatoblastoma or those with a serum α-fetoprotein (AFP) level 1 log10 ) decline in their AFP level. Responders were to receive 2 additional cycles of VI intermixed with 6 cycles of the combination of cisplatin, doxorubicin, 5-fluorouracil, and vincristine (C5VD). Nonresponders were to receive 6 cycles of C5VD alone.ResultsA total of 32 patients with a median age at diagnosis of 26 months (range, 11-159 months) were enrolled between September 2009 and February 2012. Fourteen of 30 evaluable patients were responders (RECIST and AFP in 6 patients, RECIST only in 3 patients, and AFP only in 5 patients). The median AFP decline after 2 cycles of VI for the entire group was 345,565 ng/mL (85% of the initial AFP). The 3-year event-free and overall survival rates were 49% (95% confidence interval, 30%-65%) and 62% (95% confidence interval, 42%-77%), respectively.ConclusionsThe VI combination appears to have substantial activity against HR hepatoblastoma. The ultimate impact of this regimen in improving the outcomes of children with HR hepatoblastoma remains to be determined. Cancer 2017;123:2360-2367. © 2017 American Cancer Society.

Published
2017

395. Clinical Outcomes and Molecular Responses in Children with Langerhans Cell Histiocytosis Treated with MAPK Pathway Inhibitors

Author

Eckstein, Olive S., primary, Zinn, Daniel, additional, Fein Levy, Carolyn, additional, Henry, Michael M., additional, Campbell, Patrick, additional, Jordan, Michael B., additional, Kumar, Ashish R, additional, Rodriguez-Galindo, Carlos, additional, Hijiya, Nobuko, additional, Abla, Oussama, additional, McClain, Kenneth L., additional, and Allen, Carl E., additional

Published
2018
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398. Is carboplatin-based chemotherapy as effective as cisplatin-based chemotherapy in the treatment of advanced-stage dysgerminoma in children, adolescents and young adults?

Author

Shah, Rachana, primary, Xia, Caihong, additional, Krailo, Mark, additional, Amatruda, James F., additional, Arul, Suren G., additional, Billmire, Deborah F., additional, Brady, William E., additional, Covens, Allan, additional, Gershenson, David M., additional, Hale, Juliet P., additional, Hurteau, Jean, additional, Murray, Matthew J., additional, Nicholson, James C., additional, Olson, Thomas A., additional, Pashankar, Farzana, additional, Rodriguez-Galindo, Carlos, additional, Shaikh, Furqan, additional, Stark, Daniel, additional, Frazier, A. Lindsay, additional, and Stoneham, Sara, additional

Published
2018
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399. Long-term remission of refractory Rosai-Dorfman disease after salvage therapy with clofarabine in an adult patient

Author

Hinterleitner, Clemens, primary, Steurer, Martina, additional, Dörfel, Daniela, additional, Heitmann, Jonas, additional, Kreisselmeier, Klaus-Peter, additional, Müller, Karin, additional, Kopp, Hans-Georg, additional, Wirths, Stefan, additional, Haap, Michael, additional, de Fend, Leticia Quintanilla-Martinez, additional, Horger, Marius, additional, Rodriguez-Galindo, Carlos, additional, Kanz, Lothar, additional, and Müller, Martin R., additional

Published
2018
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400. Comparison of carboplatin versus cisplatin in the treatment of paediatric extracranial malignant germ cell tumours: A report of the Malignant Germ Cell International Consortium

Author

Frazier, A. Lindsay, primary, Stoneham, Sara, additional, Rodriguez-Galindo, Carlos, additional, Dang, Ha, additional, Xia, Caihong, additional, Olson, Thomas A., additional, Murray, Matthew J., additional, Amatruda, James F., additional, Shaikh, Furqan, additional, Pashankar, Farzana, additional, Billmire, Deborah, additional, Krailo, Mark, additional, Stark, Dan, additional, Brougham, Mark F.H., additional, Nicholson, James C., additional, and Hale, Juliet P., additional

Published
2018
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