The degree of glucose metabolism, and thus uptake of FDG, is higher in tumours such as NBL. California Privacy Statement, Article  Although achieving higher cure rates, combined-modality treatment is often rigorous, and has its associated early and late complications. Chronic Inflammatory Bowel Disease 2. The same post-operative staging system is utilised in European SIOP studies, albeit after courses of pre-operative chemotherapy rather than with upfront surgery as in COG studies. Yes. []. The INRGSS has 4 simpler stages of disease summarised in Table 2 [13,14]. Google Scholar. NBs and WTs are the two most frequent tumors in children together accounting for nearly 15% of all pediatric cancers (14,15).They occur in early childhood and typically present as large abdominal masses closely associated with the kidneys. McHugh K. Renal and adrenal tumours in children. General imaging differential considerations include: 1. neuroblastoma: see neuroblastoma vs. Wilms tumor 2. cystic partially differentiated nephroblastoma and pediatric cystic nephroma: appear identical to very cystic Wilms tumor 1 3. clear cell sarcoma: generally indistinguishable on the bases of imaging, but may show early skeletal metastasis, a site which is unusual for Wilms tumor 4. renal rhabdoid tumor: generally, it can not be distinguished from Wilms on imaging, but the former has an established associa… The authors declare that they have no competing interests. Taupathy. Studies comparing other functional imaging techniques are currently being undertaken. N Neuroblastoma commonly crosses the midline and usually calcifies. Shamberger RC, Ritchey ML, Haase GM, Bergemann TL, Loechelt-Yoshioka T, Breslow NE. The natural histories and typical clinical courses of these common tumours are very different. Understanding the principles in management of Wilms tumour: Can imaging assist in patient selection? doi:10.1200/JCO.2012.45.9339. Nucl Med. PubMed  Mesoblastic nephroma occurs in the neonate whereas Wilms tumor is very rare in the newborn. Radiotherapy directly to the mass is also routinely administered in high-risk tumours post-chemotherapy [8]. A unilateral Wilms tumour is treated with nephrectomy. Renal vein thrombus may be more difficult to evaluate or exclude with US, and CT or MRI tend to be easier to interpret in this setting. Methods. Manage cookies/Do not sell my data we use in the preference centre. Most commonly, NBLs are located within the adrenal gland, but can be found in sympathetic ganglia of the retroperitoneum, posterior mediastinum, neck or pelvis [1]. Low to intermediate risk tumours tend to have a reasonably good prognosis (90% survival approximately) with high-risk tumours being much less favourable (40-50% survival) [5]. J Clin Oncol. MIBG in its most simple form provides 2D planar information (scintigraphy). 2011;253(5):1004-10. The clinical approach should involve multidisciplinary discussion following thorough risk-assessment. Low risk patients with local tumour masses should have them surgically resected. Results from the NWTS Group has found overall 10-year survival rates for favourable histology of 96-89% for stages I-III disease (82-49% for unfavourable histology), 81% for stage IV disease (18% for unfavourable histology) and 78% for stage V disease [23]. To determine prognostic significance of hospital surgical volume and Children's Oncology Group (COG) membership on neuroblastoma (NBL) and Wilms tumor (WT) survival. Here we review the literature related to cancer energy metabolism in neuroblastoma (NB) and Wilms tumor (WT). doi:10.1002/pbc.23222. Wilms' tumor provides a spectacular example (Fig 1). There are now two distinct histopathological types based on prognosis – favourable (over 90%) and unfavourable (6-10%). Lancet. Neuroblastoma (NBL) is the most common extra-cranial tumour in childhood. Presentation is usually with a large, painless abdominal mass and very little in the way of constitutional symptoms [20]. All authors read and approved the final manuscript. Hemi-nephrectomy, wedge resections and nephron-sparing surgery require accurate pre-operative imaging. Beckwith JB, Palmer NF. J Nucl Med. American University of Beirut 2. Wilms tumor and neuroblastoma 1. Progressive Supranuclear Palsy (PSP) (Syn : Steele-Richardson-Olszewski Syndrome) Adult onset neurodegenerative disorder. Pre-operative chemotherapy is vital as each kidney is ultimately staged separately. Young boy with a left sided Wilms tumor. They can demonstrate extension across the midline and into adjacent body cavities. Google Scholar. Brisse HJ, McCarville MB, Granata C, Krug KB, Wootton-Gorges SL, Kanegawa K. International Neuroblastoma Risk Group Project. NBLs have a variable prognosis; tumour stage, patient age, tumour oncogenes and DNA content are all known to be implicated. •Tumors may occur anywhere along the sympathetic chain within the neck, thorax, retroperitoneum, or pelvis, or in the adrenal gland. This type of tumours are characterised by the proliferation of immature cells that are usually only seen in the developing embryo. 2014;61(1):3–6. Neuroblastoma and Wilms' tumor are the most common noncentral nervous system solid tumors in children. Neuroblastoma and Wilms tumour are both relatively common abdominal childhood cancers. Distinguishing between the two is important, and a number of features are helpful. Privacy Intermediate risk patients have chemotherapy followed by surgery [17]. Neuroblastoma, Ganglioneuroblastoma, and Ganglioneuroma: Radiologic - Pathologic Correlation. Imaging neuroblastoma: what the radiologist needs to know. and 4 2001;11:2071–81. © 2020 BioMed Central Ltd unless otherwise stated. Cite this article. However, the role of chest CT is not controversial in patients who post-operatively are found to have unfavourable histology or stage III disease, as accurate staging at diagnosis appears to improve overall survival in these patients [28]. Incidence is slightly higher in Caucasians. These are the CT Brain images of a young male patient, who had h/o fever & Status Epilepticus  (? Wilm’s Tumor • AKA: Nephroblastoma • the most common intra-abdominal cancer in children. doi:10.2967/jnumed.110.085100. PubMed  2016;34(1):13-23. The Creative Commons Public Domain Dedication waiver ( applies to the data made available in this article, unless otherwise stated. Paediatr Child Health. In: Radiology Review Manual 7th edition, Lippincott Williams & Wilkins, (2011), p.895-1012. Studies comparing MIBG to FDG-PET have shown that the former can be sensitive and specific in higher stage disease with FDG-PET being useful in stage 1 and 2 disease or in MIBG non-avid tumours [9]. There are slightly higher local relapse rates reported in SIOP studies, but these radiotherapy naïve patients do appear to have high salvage rates [29] The American approach is initial surgery and then chemotherapy after post-surgical staging. In children, the paraspinal lines are less frequently observed than in adults due to less mediastinal fat and no aortic ectasia. The anaplastic and sarcomatous variants are the unfavourable histologies associated with a poorer outcome [21]. Now wrist pain. For surgical planning and risk stratification, more detailed cross-sectional imaging is necessary. 2009;50(8):1237–43. The COG stratify risk as low, intermediate or high based on prognostic factors and the INSS staging system. 2009;27(2):298–303. Nephroblastomatosis, which consists of immature metanephric tissue (nephrogenic rests), is considered a precursor to Wilms tumour [Figure 8]. doi:10.1102/1470-7330.2011.9008. Article  The right and left paraspinal lines are where the lung or pleura interacts with the mediastinal soft tissues. Ultrasound abdomen of a 4 year old boy with a left-sided Wilms tumour, presenting here unusually as a solid uniform mass. doi:10.1007/s003300100931. Wood L, Lowis S. An update on neuroblastoma. Department of Radiology, Great Ormond Street Hospital for Children, Great Ormond Street, London, WC1N 3JH, UK, Maureen Dumba, Noorulhuda Jawad & Kieran McHugh, You can also search for this author in CAS  MD drafted the abstract, introduction, neuroblastoma and conclusion sections, assisted with editing the manuscript including all revisions, edited the images and prepared the references. Diffusion-weighted MRI for differentiating Wilms tumor from neuroblastoma. doi: One of the key defining features is the presence of calcification [Figure 6] seen in 80-90% of CT studies [2]. Nephroblastoma, also more commonly known as a Wilms tumour, is the commonest renal tumour in childhood and more typically presents as abdominal pathology with few constitutional symptoms, although rarely haematuria can be a presenting feature. Jan 27, 2017 - Both neuroblastoma and Wilms tumor occur in early childhood and typically present as large abdominal masses closely related to the kidneys. The management of synchronous bilateral Wilms tumor: a report from the National Wilms Tumor Study Group. It can present as an abdominal mass, but is usually metastatic at diagnosis so the symptomatology can be varied. doi: Diagn Interv Radiol . Neuroblastoma is a neuroendocrine tumor that usually develops in the adrenal glands, but can develop in any neuroectodermal tissue. initially was evaluated by ultrasound and the mass seen arising from the left kidney. Chest x-ray of 3 year old girl showing thoracic NBL. Wilms tumor (WT; nephroblastoma) is the most common malignant renal tumor in children, accounting for about 85% of pediatric renal tumors.1 2 The incidence of WT is about 1 per 10 000 children in Europe and North America. Lowe HL, Isuani BH, Heller RM, Stein SM, Johnson JE, Navarro OM, et al. The INRGSS has not been designed to replace the INSS and centres will likely use both in the management of NBL patients [14]. In six cases where this phenomenon was observed, the histologic findings of each tumor were identical: undifferentiated neuroblastoma. Results of the SIOP 2001 study. High-risk patients have a more intensive chemotherapy course followed by resection then myeloablative chemotherapy [16]. This differentiation is critical as the treatments for these two tumors are different. Recently, MRI diffusion studies have possibly made it easier to differentiate Wilms tumor from neuroblastoma, the other common abdominal malignancy in children. 2010;75:321–8. Assessment of risk pre-surgery was recently deemed to need better defining, with now more reliance placed on pre-operative imaging [8,9]. Wilm's Tumor. 177 Lu-DOTATATE molecular radiotherapy for childhood neuroblastoma. We also searched the reference lists of articles identified by this strategy. 2007;369:2106–20. Gains J, Bomanji JB, Fersht N, Sullivan T, D’Souza D, Sullivan KP. • peak incidence is 2 to 3 years of age 3. Nephrogenic rests, nephroblastomatosis, and associated lesions of the kidney. Article  The full extent of the mass can be difficult to define on a pre-operative MRI scan and thus make surgical planning more challenging. Wilms Tumor Updated by Eva N. Christensen. doi: Semi Cancer Biol. Urol Oncol. Differentiating Normal from Abnormal Inferior Thoracic Paravertebral Soft Tissues on Chest Radiograph in Children. McHugh K, Roebuck DJ. Both are treated with surgical excision and the final distinction is made with pathology. Renal preservation surgery becomes paramount. MR findings in Wilms tumour are low signal intensity on T1W, with variable/high signal intensity on T2W [26]. Dahnert W. Urogenital tract. doi:10.1148/radiol.11101352. 2009;27:289–97. J Postgrad Med Edu Res. These features are particularly important when the mass is known to be encasing major vessels. After hydronephrosis and multicystic dysplastic kidney, it is the most common cause of a renal mass in a child [19]. The International Neuroblastoma Risk Group (INRG) was thus established in 2004 to create a more comprehensive staging system. Over 90% are MIBG-sensitive, but for those primary tumours that are not MIBG avid, 99mTc-diphosphonate bone scintigraphy is currently recommended to look for bony disease [1]. This paper reviews the clinical aspects and imaging characteristics of both tumours, summarising the key differences to aid the paediatrician and radiologist in their diagnosis and management. Cancer. An emerging PET/CT compound is Ga-68 DOTATATE, which utilizes somatostatin receptor expression in NBLs, in particular sub-type 2 [10,11]. Ann Surg. Features that are suggestive of thoracic disease include abnormalities of the normal silhouettes typically seen on chest x-rays. Hamilton TE, Ritchey ML, Haase GM, Argani P, Peterson SM, Anderson JR, et al. NJ drafted the nephroblastoma section and assisted with editing the manuscript including the revisions and references. CT should ideally now, in our opinion, be reserved for pre-operative surgical planning, particularly if there is a surgical preference for CT, when contrast-enhanced images can delineate the vasculature to best effect [1]. Thickening and irregularity of these lines, in particular the right paraspinal line which is not normally seen in healthy children, can indicate the presence of increased mediastinal soft tissue and this warrants further investigation [7]. Neuroblastoma (NBL) is the most common solid extra-cranial tumour in childhood [1]. Maureen Dumba. Dumba, M., Jawad, N. & McHugh, K. Neuroblastoma and nephroblastoma: a radiological review. On a CT study post-chemotherapy, the solid portions of the mass are easier to define than on MRI and the extent of calcification, which increases after treatment and which can be important for the surgeon to appreciate before surgery, is more easily characterised. To enable consistent reporting, image defined risk factors (IDRFs) have been identified by the INRG – these describe the relationship between the tumour and adjacent vascular, major airway or nervous system structures, which ideally should not be injured at surgery [6,14]. Coronal T2 MR of a 2 year old boy showing left-sided NBL mass with bone marrow involvement (blue arrow). US is useful for the assessment of caval patency and IVC tumour thrombus and is the preferred modality for this in our experience. Fusing these 3D images with CT can enable tissue differentiation. cancer imaging 15, 5 (2015). Davidoff AM. Wilms tumor manifests as a solid intrarenal mass with a pseudocapsule and distortion of the renal parenchyma and collecting system. 2001;21:217–28. If a removed kidney containing a Wilms tumour is found to have nephroblastomatosis, there is a 20% chance of developing a Wilms tumour in the contralateral kidney [22]. Ora I, Eggert A. The biology of thoracic NBL tends to be less aggressive than with abdominal disease and as such the prognosis tends to be more favourable. Nephroblastoma, also more commonly known as a Wilms tumour, is the commonest renal tumour in childhood and more typically presents as abdominal pathology in an otherwise asymptomatic patient. Axial CT of a 3 year old boy with right-sided Wilms tumour, again demonstrating the ‘claw sign’ (blue arrow). 2007;7:41–51. It is often confused clinically and histologically with Wilms' tumor, rhabdomyosarcoma, lymphoma, and especially, Ewing's sarcoma. This variability means survival rates also differ. Wilms tumor. Of the solid tumors of childhood, neuroblastoma--the prototypic small-, round-, blue-cell neoplasm--occurs in the youngest patients and has shown the least predictable biologic behavior and response to therapy. ribs indicating a posterior mediastinal mass. It can, however, be acquired in a 3D format with the resultant Single Positron Emission Tomography (SPET or SPECT) images providing more detailed information. Mittal BR, Agrawal K, Shukla J, Bhattacharya A, Singh B, Sood A. Ga-68 DOTATATE PET/CT in neuroendocrine tumours: Initial Experience. PubMed Google Scholar. Mosby; 1994. Foci of nephroblastomatosis may be small cystic lesions, hyperintense on T2W but sclerotic nephrogenic rests may appear fibrotic, being relatively hypointense on T2W sequences. Tumours with MYCN amplification, whether localised or metastatic, are all categorised as high risk tumours in both North American Children's Oncology Group (COG) and European (SIOPEN) neuroblastoma studies. … This is an Open Access article distributed under the terms of the Creative Commons Attribution License (, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly credited. The National Wilms Tumour Study Group have comprehensively staged Wilms tumours and this is reviewed as it impacts significantly on management. Complete White Out’ on the CXR has a limited number of causes. Pediatric oncology surveillance imaging: Two recommendations. Coronal T2 MR of a 4 year old girl with bilateral Wilms tumours, more cystic on the left. The goals of modern pediatric oncology reflect both of these facts. Simon T, Häberle B, Hero B, von Schweinitz D, Berthold F. Role of surgery in the treatment of patients with stage 4 neuroblastoma age 18 months or older at diagnosis. J Clin Oncol. Neuroblastoma vs Wilms tumor | Radiology Reference Article | 2011;11(1A):S44–7. There are a wide number of conditions that have been associated with NBL; neurofibromatosis type 1, Beckwith-Weidemann syndrome, Hirschsprung’s disease and DiGeorge syndrome are all described in the literature [6]. ... 'A' ABSENT BOW TIE SIGN Seen in MRI Knee, in Bucket handle tear of meniscus. Oncogenetics and chromosomal abnormalities are increasingly recognised as important prognostic indicators and their impact on initial management is considered. 2000;175(2):477–83. 2012;59(1):247–67. 2002;22:911–34. The incidence is much lower (3–4 per 10 000 children) in Asian countries.3 US National Wilms Tumor Study revealed that the median onset age is 38 months, … Some behave aggressively while others, typically in infancy, may spontaneously regress. Aslan M, Aslan A, Arıöz Habibi H, Kalyoncu Uçar A, Özmen E, Bakan S, et al. Simple theme. A PubMed search was done with the terms “Wilms tumour”, “Wilms tumor”, “nephroblastoma”, “low income”, “resource limited” and “Africa”. Clin Radiol. Accurate staging and discovery of the full extent of metastatic disease would therefore aid survival. Axial T2 MR of a 4 year old boy with right-sided Wilms tumour and left-sided nephroblastomatosis (blue arrow). statement and Survival rates for neuroblastoma. Wilms tumour classically follows the “rule of 10’s”: up to 10% may have unfavourable histology, 10% are bilateral, 10% have vascular invasion, 10% have calcifications on CT and 10% have pulmonary metastases at presentation [19]. This may occur following chemotherapy to try and shrink the mass, ensuring full resection [8]. Kembhavi SA, Qureshi S, Vora T, Chinnaswamy G, Laskar S, Ramadwar M, et al. Cancer Imaging. The tumour extent is easily visualised on non-contrast T1W and T2W sequences, but small bilateral tumours and foci of nephroblastomatosis are often best seen after gadolinium administration. In 2002, approximately 15% of childhood cancer deaths were due to NBL [6]. This is a 5 year old girl, presenting to her primary physician with abdominal mass. Biology • somatic mutations restricted to tumor tissue (Majority of Cases) • germline mutations Sporadic: Almost 97% of Wilms' … Neuroblastoma vs Wilms tumor Dr Ammar Haouimi ◉ and Assoc Prof Frank Gaillard ◉ ◈ et al. Approximately 1% are familial, displaying an autosomal dominant pattern of inheritance with incomplete penetrance [1,4]. The organ of Zuckerkandl is a mass of neural crest tissue adjacent to the mid to distal abdominal aorta and it is another recognized site of disease. Presenting features are diverse and very much dependent on the anatomical location of the tumour. The peptide binds to somatostatin receptors and can, therefore, be used for diagnosis and follow-up [10]. Typically, NBLs occur in early childhood with up to 90% diagnosed by 6 years of age [4]. Vaginal Cysts   a) Gartner Duct Cysts   b) Bartholin Gland Cysts  2. In children with bilateral disease, the therapeutic approach and philosophy are very different. Hyperechoic areas may represent areas of fat, calcification or haemorrhage. Maris JM, Hogarty MD, Bagatell R, Cohn SL. Pediatric Renal Masses: Wilms Tumor and Beyond. Monclair T, Brodeur GM, Ambros PF, Brisse HJ, Cecchetto G, Holmes K. The International Neuroblastoma Risk Group (INRG) Staging System: An INRG Task Force Report. 1978;41(5):1937–48. 2013;68:646–53. Is there a sex predilection?,,,, doi:10.1200/JCO.2008.16.6876. All the images posted in this site are from author's collection, unless otherwise specified.. Most arise from the adrenal gland and displace the kidney inferomedially. Some notable key differences are summarised in Table 5. The important finding which strongly points to the kidney as the site of origin of the mass is the so called claw sign; the renal parenchyma seems to embrace the mass, with sharp acute angles. From the Archives of the AFIP. Radiology of neuroblastoma in paediatrics. Xu Y, Wang J, Peng Y, Zeng J. CT characteristics of primary retroperitoneal neoplasms in children. For patients with unilateral Wilms tumour with lung lesions only visible on chest CT, not seen on plain chest radiographs (but deemed metastases and treated as such), their overall survival and event-free survival was no different to those patients whose lung lesions were not deemed metastatic [28]. This negative feature occasionally includes those children who otherwise have favourable tumour presentation features, for example 4S/MS disease and young age [1]. This is thought to be secondary to a reduction in mitosis [1]. Axial T2 MR of 2 year old girl showing NBL with rib invasion (blue arrow), anterior aortic displacement and encasement (red arrow) and bilateral pleural effusions. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Google Scholar. Adv Pediatr. Pediatr Blood Cancer. Google Scholar. The heterogeneity of the tumour, and its biological characteristics, mean the prognosis is highly variable at different ages. DOTATATE is not necessarily limited to use as a diagnostic agent. Thoracic NBL may present with airway compromise, scoliosis or as an incidental finding on chest x-ray. Up to 10% are discovered incidentally after trauma, 25% have microscopic haematuria and 25% manifest with hypertension secondary to renin production [20]. The other is the excessive production of vasoactive intestinal peptide (VIP) resulting in watery diarrhoea and failure to thrive [1]. volume 15, Article number: 5 (2015) 2011;52:1041–7. Terms and Conditions, Introduction. No calcification in the right sided abdominal mass. Am J Roentgenol. Paraneoplastic syndromes may be associated with non-metastatic disease. 2001;234(1):116–21. doi:10.1200/JCO.2008.16.6785. We invited one expert on state of the art Wilms tumour treatment from each discipline (surgery, pathology, paediatric oncology) to join the writing group. In one North American study, contrast-enhanced CT was more sensitive to disease in these vessels and they report US is not always necessary if a staging CT has already confirmed the presence of thrombus [25]. Wilms tumour is an undifferentiated mesodermal tumour, containing a variable amount of embryonic renal elements (blastema, epithelium and stroma) [20]. Post-treatment surveillance for bilateral disease should be with MRI and not CT to reduce the radiation burden in these children the vast majority of whom have a good long-term prognosis [27], but some institutions may image post-operatively with CT at the surgeon’s request.
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