Viewer. ), Figure 16a. 83, No. (c) CT scan of the lung bases shows multiple bilateral small cysts.Download as PowerPointOpen in Image Wilms tumor outnumbers renal cell carcinoma in childhood by a ratio of 30:1 (,8). At CT performed before intravenous administration of contrast material, the mass may be isoattenuating or hyperattenuating and small calcifications may be present (,30,,31). (a) Nonenhanced CT scan shows multiple fat-attenuation foci within the kidneys (arrowheads). Photograph of the cut surface of a gross specimen shows a whorled, myomatous appearance with prominent medial extension (arrow) and an ill-defined margin. Figure 1a. Figure 19b. Wilms’ tumour and the nephroblastoma complex. 1, No. Rhabdoid tumor in an 8-month-old boy with hematuria. From this location, it extends in a polypoid fashion into the collecting system. Rhabdoid tumor of the kidney (RTK), a rare aggressive cancer occurring in infancy and early childhood, was recognized as a distinct tumor type in 1978, although initially it was classified as a possible rhabdomyosarcomatoid variant of Wilms' tumor. 5, 3 July 2010 | Pediatric Radiology, Vol. Wilms tumor. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). The differential diagnosis includes transitional cell carcinoma, which is exceedingly rare and poorly documented in children, and rhabdoid tumor, which typically occurs in children aged 3 years or younger. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). Teratoid Wilms' Tumor: Report of a Unilateral Case. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). Renal lymphoma in a 16-year-old girl with no renal symptoms. Rhabdoid tumor. (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). Mature osteoid elements tend to be more prominent in older patients. The prognosis is extremely poor, with advanced disease at the time of diagnosis. (Courtesy of Tim Booth, MD, Children's Medical Center, Dallas, Tex. Although the appearance may closely resemble that of Wilms tumor, several features can suggest the diagnosis: subcapsular fluid collections, tumor lobules separated by dark areas of necrosis or hemorrhage, and linear calcifications outlining tumor lobules. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). 64, No. Nephroblastomatosis occurs most often in neonates and is characterized by multiple bilateral subcapsular masses, often associated with Wilms tumors. At histologic analysis, the tumor is monomorphic, with fingerlike, infiltrating projections of spindle-shaped mesenchymal cells and embryonal metaplasia of entrapped renal tissue (,3,,10). Primitive neuroectodermal tumor, ependymoma, and cerebellar and brainstem astrocytoma have all been documented (,15). 38, No. Rhabdoid tumor may manifest as hematuria, but due to its aggressive nature, symptoms may be referable to metastatic disease. The mean age at presentation is 41 years, with a 4:1 female predominance. At gross analysis, the tumor has a rubbery appearance, similar to a uterine leiomyoma. Figure 18. 2, Ultrasound in Medicine & Biology, Vol. Viewer. (b) Contrast-enhanced CT scan shows large, homogeneous Wilms tumors (W) superimposed on a background of multiple peripheral nephrogenic rests (arrowheads). ), Figure 21a. 2, Journal of the Korean Society of Radiology, Vol. Figure 2a. (c) Contrast-enhanced CT scan shows a heterogeneous mass (curved arrows) infiltrating the right kidney and causing hydronephrosis. Extensive adenopathy is seen surrounding the retroperitoneal vessels (straight arrows). Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. (b) Contrast-enhanced CT scan shows heterogeneous enhancement of soft tissue within the lesions. 39, No. 1, The Egyptian Journal of Radiology and Nuclear Medicine, Vol. ), Figure 7b. Gadolinium-enhanced coronal fat-suppressed T1-weighted MR image shows a large, well-defined mass in the right kidney (arrows) that enhances less than adjacent renal parenchyma and contains multiple hypointense hemorrhagic foci. )Download as PowerPointOpen in Image A child with bilateral Wilms' tumors is reported. †Tuberous sclerosis, neurofibromatosis, von Hippel–Lindau syndrome. At MR imaging, the nodules demonstrate low-signal-intensity foci on both T1-weighted and T2-weighted images. Extensive adenopathy is seen surrounding the retroperitoneal vessels (straight arrows). However, to date, Wilms tumor has not been reported in any of these patients. 9, Néphrologie & Thérapeutique, Vol. There is a slight male predominance (,10). (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). 50, No. Viewer, Figure 21c. US demonstrates highly echogenic nonshadowing foci, which correlate with the fatty elements. Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. )Download as PowerPointOpen in Image The pathogenesis of this complex neoplasm is discussed, and argument is presented that primitive renal blastema may be capable of more diverse differentiation than has previously been realized. 3, 16 January 2008 | Der Urologe, Vol. (d) Anterior and posterior images from a bone scan show multiple foci of increased uptake (arrows) and lack of renal excretion, findings consistent with extensive metastases. Adult teratoid Wilms' tumor with prominent neuroepithelial differentiation. The tumor is characterized by its aggressive behavior and is associated with a higher rate of relapse and mortality than Wilms tumor. (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. Imaging demonstrates a large, centrally located, heterogeneous soft-tissue mass involving the renal hilum with indistinct margins (,Fig 15). It is currently believed that nephrogenic rests give rise to approximately 30%–40% of Wilms tumors (,4), and they are found in up to 99% of bilateral Wilms tumors (,5). AT/RT most commonly occurs intracranially. 07, 12 September 2017 | RadioGraphics, Vol. Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. Some investigators believe that the ossification is the result of the osteogenic potential of urothelial cells (,24). Perinephric involvement can arise from retroperitoneal disease (most commonly) or transcapsular spread of parenchymal involvement. MR imaging also permits assessment of caval patency and multifocal disease. Perilobar rests lie in the peripheral cortex or columns of Bertin. Nephroblastomatosis consists of diffuse or multifocal involvement of the kidneys with nephrogenic rests. Extrarenal testicular Wilms’ tumor in a 3-year-old child. Mesoblastic nephroma. 1, Korean Journal of Radiology, Vol. (Reprinted, with permission, from reference 7. (b) Contrast-enhanced CT scan shows large, homogeneous Wilms tumors (W) superimposed on a background of multiple peripheral nephrogenic rests (arrowheads).Download as PowerPointOpen in Image (Courtesy of Maria M. Rodriguez, MD, Miami, Fla; reprinted, with permission, from reference 7. The tumor responds poorly to chemotherapy or radiation therapy, with a mean survival of 15 weeks from diagnosis. The cells contain extensive clear cytoplasm with displaced nuclei. (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). At histologic analysis, Wilms tumor is composed of variable amounts of blastema, stroma, and epithelium (,,,Fig 1). Primary extrarenal Wilms' tumor in children. (b) Photomicrograph (original magnification, ×50; hematoxylin-eosin stain) shows tubular (arrows) and acinar (A) structures adjacent to normal kidney on the right. 4, Seminars in Roentgenology, Vol. 70, No. At CT, macroscopic nephrogenic rests appear as low-attenuation peripheral nodules with poor enhancement relative to that of adjacent normal renal parenchyma (,,,Fig 5). Rarely, the lesion may recur locally if incompletely resected or metastasize to the lungs, brain, or bones. Viewer, Figure 7a. The right‐sided tumor showed the more familiar triphasic pattern of nephroblastoma. 1, 10 June 2015 | Current Radiology Reports, Vol. Median age at Dx is 3–4 yrs (95% <10 yrs) for WT. (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (a) Photograph of a gross specimen shows a lesion that extends into the renal pelvis. 1,2 Subsequent studies confirmed its distinctive nature and the designation rhabomyosarcomatoid pattern was shortened to rhabdoid. Journal of Evolution of Medical and Dental sciences. Viewer. (d) Anterior and posterior images from a bone scan show multiple foci of increased uptake (arrows) and lack of renal excretion, findings consistent with extensive metastases.Download as PowerPointOpen in Image CT demonstrates the heterogeneous mass and nodal metastases (,,,Fig 2), as well as areas of calcification and fat. 6, No. Recurrence is seen both within the tumor bed, as well as distally within the lungs or liver 1-2. When the cystic spaces are small, the tumor may appear solid. Gross painless hematuria, flank pain, and a palpable mass are the most common presenting symptoms. It can be hypoechoic or hyperechoic or even cystic with a mural nodule (,32). (b) Photomicrograph (original magnification, ×50; hematoxylin-eosin stain) shows tubular (arrows) and acinar (A) structures adjacent to normal kidney on the right. However, rare cases of isolated renal lymphoma have been reported, and this topic remains controversial (,36). 3, 12 September 2016 | RadioGraphics, Vol. Wilms tumor. Approximately 80% occur in patients less than 2 years of age and 60% in patients less than 1 year of age, with the majority (25%) diagnosed between 6 and 12 months of age (,3). If the address matches an existing account you will receive an email with instructions to reset your password. However, since massive replacement of the renal parenchyma by angiomyolipomas and cysts in patients with tuberous sclerosis may result in end-stage renal disease, preservation of functional renal tissue is an important consideration when partial nephrectomy is performed. Viewer, Figure 20b. Rhabdoid tumor. Treatment for nephrogenic rests is controversial. Figure 3. Experience of the Kiel pediatric tumor registry. 1, Seminars in Roentgenology, Vol. ), Figure 11a. 3, No. It is usually identified within the first 3 months of life, with 90% of cases discovered within the 1st year of life. 23, No. The most common radiologic pattern is multiple parenchymal masses or nodules that occasionally distort the renal contour and displace the collecting system. Familial synchronous bilateral teratoid Wilms tumor with elevated alpha-fetoprotein level. ), Figure 1b. In children with bilateral Wilms tumor, preoperative chemotherapy is especially important because each kidney is staged separately, and complete resolution of disease in one kidney may allow surgery on the contralateral kidney with eventual cure. Unilateral Wilms tumors are, usually, treated by a combination of nephrectomy and chemotherapy. It is seldom more than 2—3 cm in diameter. Wilms tumor is the most common pediatric solid renal tumor. Presurgical treatment with chemotherapy may be used to promote shrinkage of the tumor and improve outcome. Extensive adenopathy is seen surrounding the retroperitoneal vessels (straight arrows). Correlate the anatomic, radiologic, and histopathologic findings in pediatric renal masses. Viewer, Figure 21b. Black race, sickle cell trait, and hemoglobin SC disease further support the diagnosis. Eighty percent develop metastases, most commonly to the lungs and less often to the liver, abdomen, brain, lymph nodes, or skeleton. Use the link below to share a full-text version of this article with your friends and colleagues. Doppler evaluation shows the lesion to be hypovascular. TABLE 1. Renal medullary carcinoma in a 10-year-old boy with hematuria and sickle cell trait. Screening for Wilms tumor in patients with associated syndromes should begin at 6 months of age with initial computed tomography (CT) followed by serial ultrasonography (US) every 3 months up to 7 years of age. Viewer, Figure 8. Wilms tumor (WT) may show a diverse range of heterologous elements (HEs). Enter your email address below and we will send you your username, If the address matches an existing account you will receive an email with instructions to retrieve your username. Mesoblastic nephroma in a 5-week-old male infant with an in utero renal mass. Teratoid Wilms’ Tumor. Very little normal kidney (K) remains visible. Identified in 1980 (,24), ossifying renal tumor of infancy is a rare benign renal mass, with only 11 cases reported in the literature, to our knowledge (,25–,27). 45, No. 26, No. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. 7, Contemporary Diagnostic Radiology, Vol. Cystic nephroma and cystic partially differentiated nephroblastoma are uncommon, benign lesions that cannot be differentiated by means of their gross or radiographic appearance (,3) (,Fig 10). The heterologous elements were mainly composed of neuroepithelial tissue including ganglion cells, differentiating The lesion has a pseudocapsule, and its surface is divided by septa. Although renal cell carcinoma tends to be smaller than Wilms tumor, its gross morphology is similar, and the two can be indistinguishable preoperatively. 3, No. (Reprinted, with permission, from reference 7. Teratoid Wilms' tumor: case report of a rare variant that can mimic aggressive biology during chemotherapy. Symptoms are related to intratumoral bleeding from aneurysms that develop due to the abundant abnormal, elastin-poor vascularity of the tumor. It tends to be a large infiltrative mass with ill-defined margins and no capsule (,Fig 8). (a) CT scan shows a left renal mass with heterogeneous enhancement (thick arrow) and multiple hepatic metastases (thin arrows). Cases with predominant/prominent HEs have been reported as "teratoid" WT, albeit on the basis of poorly defined criteria. (a) Nonenhanced CT scan shows a calcification within the left kidney (arrow). At imaging, the renal outline is usually maintained; however, filling defects with partial obstruction of the collecting system are often seen. It is not related to Wilms tumor or rhabdomyosarcoma and was recently recognized as a distinct pathologic entity (,15). (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (a) Nonenhanced CT scan shows multiple fat-attenuation foci within the kidneys (arrowheads). (a) Longitudinal US scan shows a mass of mixed echotexture replacing the lower pole of the right kidney (arrows). 45, No. Intravenous administration of contrast material is mandatory to detect nodal or hepatic metastases, tumor extension into the renal vein or inferior vena cava, contralateral synchronous tumor, and associated nephrogenic rests. (Courtesy of Maria M. Rodriguez, MD, Miami, Fla; reprinted, with permission, from reference 7. (a) CT scan shows a right renal mass with heterogeneous enhancement and cystic foci (arrow). (c) CT scan of the lung bases shows multiple bilateral small cysts. Teratoid Wilms Tumor in a Child: A Case Report ABSTRACT Teratoid Wilms is a very rare histopathological variant of Wilms tumors that is characterized by the predominance of heterologous components, and is described by many to be a nonaggressive, nonmetastatic tumor with a favorable prognosis. Angiomyolipoma frequently contains fat and is associated with tuberous sclerosis. (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. Figure 17a. 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