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20161213 Binnenwerk Kathelijne.Indd UvA-DARE (Digital Academic Repository) Optimising therapy with 131I-MIBG for neuroblastoma patients and for neuroblastoma patients with intraspinal extension Kraal, K.C.J.M. Publication date 2017 Document Version Final published version License Other Link to publication Citation for published version (APA): Kraal, K. C. J. M. (2017). Optimising therapy with 131I-MIBG for neuroblastoma patients and for neuroblastoma patients with intraspinal extension. General rights It is not permitted to download or to forward/distribute the text or part of it without the consent of the author(s) and/or copyright holder(s), other than for strictly personal, individual use, unless the work is under an open content license (like Creative Commons). Disclaimer/Complaints regulations If you believe that digital publication of certain material infringes any of your rights or (privacy) interests, please let the Library know, stating your reasons. In case of a legitimate complaint, the Library will make the material inaccessible and/or remove it from the website. Please Ask the Library: https://uba.uva.nl/en/contact, or a letter to: Library of the University of Amsterdam, Secretariat, Singel 425, 1012 WP Amsterdam, The Netherlands. You will be contacted as soon as possible. UvA-DARE is a service provided by the library of the University of Amsterdam (https://dare.uva.nl) Download date:23 Sep 2021 Optimising therapy with 131I-MIBG Optimising therapy with Optimising therapy and for neuroblastoma patients with intraspinal extension. patients with intraspinal and for neuroblastoma for neuroblastoma patients and for neuroblastoma patients with intraspinal extension. 131 I-MIBG for neuroblastoma patients I-MIBG for neuroblastoma Kathelijne Kraal Kathelijne Kraal OPTIMISING THERAPY WITH 131I-MIBG FOR NEUROBLASTOMA PATIENTS AND FOR NEUROBLASTOMA PATIENTS WITH INTRASPINAL EXTENSION. The research described in this thesis was financially supported by KiKa, Tom Voute Fonds (also known as SKK) and Stichting zeldzame ziekten fonds (ZZF). Printing of this thesis was financially supported by Academic medical Center (University of Amsterdam) and this is gratefully acknowledged. ISBN: 978-94-6233-520-2 Cover design: Merel en Lotte Koenecke. Layout: Annelies Wisse. Printing: Gildeprint. OPTIMISING THERAPY WITH 131I-MIBG FOR NEUROBLASTOMA PATIENTS AND FOR NEUROBLASTOMA PATIENTS WITH INTRASPINAL EXTENSION ACADEMISCH PROEFSCHRIFT ter verkrijging van de graad van doctor aan de Universiteit van Amsterdam op gezag van de Rector Magnificus prof. dr. ir. K.I.J. Maex ten overstaan van een door het College voor Promoties ingestelde commissie, in het openbaar te verdedigen in de Aula der Universiteit op vrijdag 10 februari 2017, te 13 uur door door Kathelijne Catherina Jeanette Maria Kraal geboren te Zaandam Promotie commissie Promotores Prof. dr. H.N. Caron Universiteit van Amsterdam Copromotor Dr. G.A.M. Tytgat Universiteit van Amsterdam Dr. M.M. van Noesel Universiteit van Utrecht Overige leden Dr. P. Brock Great Ormond Street Hospital Prof. dr. J Booij Universiteit van Amsterdam Prof. dr. J.B. van Goudoever Universiteit van Amsterdam Prof. dr. P.M. Hoogerbrugge Prinses Máxima Centrum, Radboud Universiteit Nijmegen Prof dr. T. Simon Universität zu Köln Prof. dr. C.E van der Schoot Universiteit van Amsterdam Faculteit der Geneeskunde Aan mijn dochters, Merel en Lotte Koenecke en mijn ouders. Table of Contents Chapter 1 9 General introduction and scope of the thesis. Part A 25 Optimising therapy with 131I-MIBG for neuroblastoma patients. Chapter 2 27 131I-metaiodobenzylguanidine (MIBG), hyperbaric oxygen and Vitamin C in relapsed/ refractory high risk neuroblastoma patients. Chapter 3 43 Upfront treatment of high-risk neuroblastoma with 131I-MIBG therapy and Topotecan. Chapter 4 61 Feasibility, toxicity and response of upfront 131I-MIBG therapy followed by GPOH NB 2004 protocol in newly diagnosed stage 4 neuroblastoma patients. Chapter 5 79 131I-meta-iodobenzylguanidine therapy for patients with newly diagnosed high risk neuroblastoma, a Cochrane review. Chapter 6 127 Autologous stem cell transplantation harvesting and hematological reconstitution in high-risk neuroblastoma patients treated with 131Iodine- metaiodobenzylguanidine. Part B 149 Neuroblastoma patients with intraspinal extension Chapter 7 151 Neuroblastoma With Intraspinal Extension: Health Problems in Long-Term Survivors. Chapter 8 179 Treatment and outcome of neuroblastoma with intraspinal extension: A systematic review. Chapter 9 223 General discussion and implications for clinical practice and future research. Appendix 242 English summary 246 Nederlandse samenvatting (Dutch summary) 252 List of co-authors and their contribution to the manuscript 256 List of publications 258 PhD portfolio 261 Acknowledgements 268 Curriculum Vitae 269 Abbreviations Chapter 1 General introduction and scope of the thesis Partly adapted from: Nieuwe behandelingen voor pediatrische (HR) NBL patiënten. Ned Tijdschr Oncol 2013;10:11-9. KCJM Kraal, BLF van Eck-Smit, MM van Noesel, HN Caron en GAM Tytgat Neuroblastoma Epidemiology and pathogenesis Neuroblastoma (NBL) is the most common extra cranial solid tumor of childhood, derived from the sympathetic nervous system (1). NBL accounts for 8-10% of all childhood cancers, but is responsible for more than 15% of childhood cancer deaths (2). The median age at diagnosis is 18 months (3). In the Netherlands approximately 20-25 children per year are diagnosed with neuroblastoma. The neuroblastoma can arise anywhere along the sympathetic side chain (cervical, thoracic, abdominal and pelvic cavity), but mainly from the adrenal gland. Clinical presentation and classification The International Neuroblastoma Staging System (INSS), is a post-surgical staging system, and classifies NBL into five stages, 1 to 4 and 4S, a special stage (4). Children with stage 4 NBL present with metastatic disease at diagnosis, mainly involving the bone marrow (BM) and lymph nodes (LN). The defining charac- teristics of high-risk (HR) NBL include an age of more than one year, with metastatic disease, unfavourable Shimada histology or amplification of MYCN (NMA) (5-7). More recently, the International Neuroblastoma Risk Group Staging System was developed, since the INSS classification is a post-surgical staging system (Table I) (8). The combination of stage and other prognostic factors results in the INRG classification system, defining 3 risk groups: low risk (LR), medium risk (MR) and high risk (HR). At diagnosis 50% of NBL patients have metastatic disease, classifying them as HR. Diagnosis Catecholamines (CME) are produced mainly by the chromaffin cells of the adrenal medulla and the postganglionic fibers of the sympathetic nervous system. They can be divided into dopaminergic, adrena- Stage Description L1 Localized tumor not involving vital structures as defined by the list of image-defined risk factors and confined to one body compartment L2 Locoregional tumor with presence of one or more image- defined risk factors M Distant metastatic disease (except stage MS) MS Metastatic disease in children younger than 18 months with metastases confined to skin, liver, and/or bone marrow Table I: International Neuroblastoma Risk Group Staging System 10 Chapter 1 Response Primary tumor Metastatic sites CR (1) No tumor No tumor; CME normal. VGPR (2) Decreased by 90-99% No tumor; CME normal; residual 99Tc bone changes allowed. PR (3) Decreased by > 50% All measurable sites decreased by > 50%. Bones and BM: number of positive bone sites decreased by > 50%; no more than 1 positive BM site allowed.* MR (4) No new lesions; > 50% reduction of any measurable lesion (primary or metastases) with < 50% reduction in any other; < 25% increase in any existing lesion. NR (5) No new lesions; < 50% reduction but < 25% increase in any existing lesion. PD (6) Any new lesion; increase of any measurable lesion by > 25%; previous negative BM positive for tumor. * One positive BM aspirate or trephine biopsy allowed for PR if this represents a decrease from the number of positive sites at diagnosis. Figure 1. International Neuroblastoma Response Criteria (INRC) (4) linergic and noradrenalinergic. CME are metabolised and eventually degraded into “metanephrines” and their acidic metabolites (homovanillic acid (HVA), vanylmandylic acid (VMA) and 3-Methoxytyramine (3-MT)). They can be elevated and measured in the urine of NBL patients. Other investigations performed at diagnosis are MRI/ CT or ultrasound of the primary tumor, 123I-metaiodobenzylguanidine scan (123I-MIBG) and BM investigation for NBL infiltration (using BM aspirate or trephine biopsy and cytology staining). Response The response has been scored according to the revised International Neuroblastoma Response Criteria (INRC), taking into account the volume of the primary tumor (using MRI/ CT or ultrasound), metastatic sites using urinary CME, 123I-MIBG scans and BM examination (4). Response categories are complete response (CR), very good partial response (VGPR), partial response (PR), mixed response (MR), no response (NR) and progressive disease (PD). Therapy and prognosis Internationally the treatment for HR NBL consists of different treatment modalities: • 131I-metaiodobenzylguanidine (131I-MIBG), General introduction and scope of this thesis 11 • Induction chemotherapy, • Myeloablative chemotherapy (MAT) with autologous stem cell transplantation (ASCT)), • Surgery, • Radiotherapy, • Maintenance therapy (anti-disialoganglioside (GD2) therapy, granulocyte-macrophage colony stimulating-factor
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