These are the CT Brain images of a young male patient, who had h/o fever & Status Epilepticus  (? • peak incidence is 2 to 3 years of age 3. The authors declare that they have no competing interests. Diffusion-weighted MRI for differentiating Wilms tumor from neuroblastoma. NBL with DNA that has a hyperdiploid structure appears to be less aggressive. Hyperechoic areas may represent areas of fat, calcification or haemorrhage. doi:http://dx.doi.org/10.1148/radiographics.20.6.g00nv051585. If performed, a feature that may be seen is a soft tissue mass displacing loops of bowel. MRI should now be the cornerstone imaging modality for all primary NBL tumours [Figure 2], whether in the neck, chest, abdomen or pelvis. 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. It may also appear less commonly as a solid spherical mass [19]. Distinguishing between the two is important, and a number of features are helpful. Aslan M, Aslan A, Arıöz Habibi H, Kalyoncu Uçar A, Özmen E, Bakan S, et al. This is a 5 year old girl, presenting to her primary physician with abdominal mass. http://www.ejcancer.com/article/S0959-8049(06)00448-5/abstract. Understanding the principles in management of Wilms tumour: Can imaging assist in patient selection? J Nucl Med. J Clin Oncol. doi:10.1002/pbc.23222. 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. It is often confused clinically and histologically with Wilms' tumor, rhabdomyosarcoma, lymphoma, and especially, Ewing's sarcoma. The other is the excessive production of vasoactive intestinal peptide (VIP) resulting in watery diarrhoea and failure to thrive [1]. Maris JM, Hogarty MD, Bagatell R, Cohn SL. It is a little more prevalent in people of African descent. This is thought to be secondary to a reduction in mitosis [1]. Two of the most common are neuroblastoma and Wilms Tumor. NJ drafted the nephroblastoma section and assisted with editing the manuscript including the revisions and references. Dumba, M., Jawad, N. & McHugh, K. Neuroblastoma and nephroblastoma: a radiological review. Occasionally, however, a neuroblastoma appears to be intrarenal. The COG stratify risk as low, intermediate or high based on prognostic factors and the INSS staging system. They can demonstrate extension across the midline and into adjacent body cavities. Nephroblastoma is more commonly known as a Wilms tumour after Dr Max Wilms, the German surgeon who first described it in 1899. Plain abdominal radiographs are non-specific for Wilms tumours. Radiology. The right and left paraspinal lines are where the lung or pleura interacts with the mediastinal soft tissues. Now wrist pain. Blickman, GJ & Blickman, H: Pediatric radiology 3rd edition. Radiotherapy directly to the mass is also routinely administered in high-risk tumours post-chemotherapy [8]. Pediatr Blood Cancer. for 1 hour),became unresponsive and... “Cortical laminar necrosis” was defined as hyperintense cortical lesions on T1-weighted imaging found during the subacute or chronic phase... CT Bowel Fat Halo Sign Seen in  1. Cytoreductive therapy 3. Paraspinal tumor may invade the spinal canal via extension through adjacent neural foramina is s/o NB." Radiographics. Typically, NBLs occur in early childhood with up to 90% diagnosed by 6 years of age [4]. The tumor typically spreads by direct extension and displaces adjacent structures but does not typically encase or elevate the aorta; such encasement or elevation is a distinguishing characteristic of neuroblastoma. The surgical approach in bilateral disease aims to spare any normal renal parenchyma where possible. After hydronephrosis and multicystic dysplastic kidney, it is the most common cause of a renal mass in a child [19]. doi: http://dx.doi.org/10.1016/j.ejca.2011.05.025. Google Scholar. doi: http://dx.doi.org/10.1148/radiographics.22.4.g02jl15911. This showed promising early results as a feasible agent in a select patient cohort [11]. Taupathy. Conventional radiograph of abdomen shows a large right upper mass displacing bowel loops into the pelvis and across the midline (blue arrows). Origin: Embryonic nephrogenic (mesodermal) cells Mechanism: Deletion of WT-1 gene on chromosome 11 Pathology: a. Macroscopic (Gross): large, solitary tan to grey color mass; pale on cut section with areas of hemorrhage; 10% are bilateral or multicentric; grossly difficult to distinguish from neuroblastoma From the Archives of the AFIP. Solid intrarenal neoplasms in children are usually Wilms tumors. 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. th 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. Saved from radiopaedia.org. 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. Radiotherapy is indicated for local stage III, so down-staged patients avoid this and the long-term post-radiotherapy sequelae. A study by Gains et al combined the use of Ga-68 DOTATATE for assessment and followed this with Lu-177-DOTATATE for targeted molecular therapy [11]. ∼500 cases/yr of WT are diagnosed in the U.S. What is the median age at Dx? Eur Radiol. 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]. Like CT, MRI can also readily demonstrate the ‘claw sign’ of normal renal tissue around the tumour [Figure 10]. Lonergan GJ, Schwab CM, Suarez ES, Carlson CL. Article  In: Radiology Review Manual 7th edition, Lippincott Williams & Wilkins, (2011), p.895-1012. The vast majority of cases are sporadic. In more localised adrenal or other L1 tumours, pre-operative MRI is preferred over CT as no significant vascular encasement is typically present. 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]. Ultrasound abdomen of a 4 year old boy with a left-sided Wilms tumour, presenting here unusually as a solid uniform mass. NBLs have a variable prognosis; tumour stage, patient age, tumour oncogenes and DNA content are all known to be implicated. Cookies policy. The International Neuroblastoma Staging System (INSS) has been used since 1986 to stage NBL [Table 1], however, there was significant worldwide variability in the application of this system, which to some extent is dependent on local protocol and experience [12]. 9 year old male child for follow-up scan of an incidentally detected right temporal lobe cyst. Jan 27, 2017 - Both neuroblastoma and Wilms tumor occur in early childhood and typically present as large abdominal masses closely related to the kidneys. Wilms Tumor Updated by Eva N. Christensen. Paraspinal tumor may invade the spinal canal via extension through adjacent neural foramina is s/o NB. 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]. The result is less stage III disease, thus some patients are down-staged. Article  Ultrasound (US) is often the first line investigation in paediatrics, particularly for those presenting with an abdominal mass. Neuroblastoma is a neuroendocrine tumor that usually develops in the adrenal glands, but can develop in any neuroectodermal tissue. Urol Oncol. DOTATATE is not necessarily limited to use as a diagnostic agent. Wilm's Tumor. statement and doi:10.1148/radiol.11101352. In six cases where this phenomenon was observed, the histologic findings of each tumor were identical: undifferentiated neuroblastoma. 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. Google Scholar. Axial T2 MR of 3 year old boy showing intraspinal extent of NBL with tumour seen in both neural foramina on this single image (blue arrows). Neuroblastoma and Wilms' tumor are the most common noncentral nervous system solid tumors in children. and 4 Wilms' tumor provides a spectacular example (Fig 1). Springer Nature. 2000;175(2):477–83. Lonergan GJ, Martinez-Leon MI, Agrons GA, Montemarano H, Suarez ES. PubMed Google Scholar. Mesoblastic nephroma and Wilms tumor look very similar on imaging as both present as large solid renal masses. Perivaginal Cysts... Sagittal T1 Weighted MRI Brain Question 3 : Name the labelled structure ? Sharp SE, Shulkin BL, Gelfand MJ, Salisbury S, Furman WL. MRI can easily assess the extent of disease, being superior to CT in assessing metastatic marrow disease, chest wall invasion and spinal canal involvement [Figures 3, 4 & 5]. 4. [http://www.cancer.org/cancer/neuroblastoma/detailedguide/neuroblastoma-survival-rates]. Coronal and sagittal reformatted CT demonstrate a large, heterogeneous mass arising from the right kidney (white arrows) . 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]. Plain films are non-specific for NBL and are largely unhelpful in the diagnostic pathway. Since it is a suprarenal tumor, it displaces the kidney while Wilms’ tumor destroys and … The differences in management strategies are outlined. In patients who have relapsed, routine PET-CT may be of benefit as their prognosis is more guarded and the best chance of cure is at the first relapse. Some surgeons may quite reasonably prefer later pre-operative CT prior to surgery, and this would generally be a local preference [Figure 12]. FDG combined with CT, PET-CT, allows for more accurate localization of disease. They share similar cell characteristics on gross histological evaluation to other relatively common paediatric tumours such as Ewing’s sarcoma, primitive neuroectodermal tumours (P… It can, however, be acquired in a 3D format with the resultant Single Positron Emission Tomography (SPET or SPECT) images providing more detailed information. 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 This differentiation is critical as the treatments for these two tumors are different. doi:10.1002/pbc.24757. Wilms tumor manifests as a solid intrarenal mass with a pseudocapsule and distortion of the renal parenchyma and collecting system. In children, the paraspinal lines are less frequently observed than in adults due to less mediastinal fat and no aortic ectasia. The INRGSS has not been designed to replace the INSS and centres will likely use both in the management of NBL patients [14]. Wood L, Lowis S. An update on neuroblastoma. The International Neuroblastoma Risk Group (INRG) was thus established in 2004 to create a more comprehensive staging system. Coronal T2 MR of a 2 year old boy showing left-sided NBL mass with bone marrow involvement (blue arrow). Neuroblastoma. Oncogenetics and chromosomal abnormalities are increasingly recognised as important prognostic indicators and their impact on initial management is considered. Wilms tumor have a mutation in the germline or in tumor tissue. 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. Cancer Imaging. Some notable key differences are summarised in Table 5. rd The MYCN oncogene is responsible for providing the code used by proteins in tissue development. Approximately 1% are familial, displaying an autosomal dominant pattern of inheritance with incomplete penetrance [1,4]. The INRGSS has 4 simpler stages of disease summarised in Table 2 [13,14]. The peptide binds to somatostatin receptors and can, therefore, be used for diagnosis and follow-up [10]. 2012;59(1):247–67. 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