), Figure 21b. Mesoblastic nephroma in a 5-week-old male infant with an in utero renal mass. Figure 2a. (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 2, Journal of the Korean Society of Radiology, Vol. (a) Plain radiograph shows displacement of bowel loops into the midabdomen by bilateral flank masses. Nephroblastomatosis consists of diffuse or multifocal involvement of the kidneys with nephrogenic rests. (Courtesy of Esperanza M. Tiamson, MD, Baltimore, Md; reprinted, with permission, from reference 7. Classical Wilms tumors are characterized by a triphasic histologic pattern that includes blastemic, stromal, and epithelial cell types. At gross analysis, the tumor has a rubbery appearance, similar to a uterine leiomyoma. At imaging, the renal outline is usually maintained; however, filling defects with partial obstruction of the collecting system are often seen. 6, Magnetic Resonance Imaging Clinics of North America, Vol. At histologic analysis, small cells with inconspicuous nucleoli and ill-defined cell membranes and a prominent capillary network commonly characterize this tumor (,Fig 12). 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. (b) Contrast-enhanced CT scan obtained at the same level shows a low-attenuation mass (arrows) surrounding the calcification. Metanephric adenoma. Neuroglial tissue in partially cystic Wilms' tumour. 41, No. Figure 9b. 05, No. (a) Plain radiograph shows displacement of bowel loops into the midabdomen by bilateral flank masses. Wilms tumor in a 4-year-old boy with an abdominal mass. 45, No. Cystic partially differentiated nephroblastoma in a 12-year-old girl with an abdominal mass. Clinicopathological Study of Nephroblastomas in Ibadan. 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. Clear cell sarcoma in a 13-month-old girl with an abdominal mass. Like the classical Wilms tumor, this variant may also occur as a renal mass or may be found in extra renal locations. ), Figure 7a. Viewer. 10.1002/1097-0142(19931115)72:10<2910::AID-CNCR2820721009>3.0.CO;2-4. (Courtesy of Frederic B. Askin, MD, St Louis, Mo; reprinted, with permission, from reference 7. 3, No. Working off-campus? The left renal tumor showed nephroblastoma with several tissues of apparent mesenchymal derivation and tubules with diverse epithelial differentiation. Photograph of a gross specimen shows diffuse overgrowth of peripheral mesoblastic tissue, which causes loss of corticomedullary differentiation and nephromegaly with preservation of reniform shape. Teratoid Wilms' tumor is considered by some as a variant of Wilms' tumor containing at least 50% heterologous differentiated tissue. Photograph of the cut surface of a gross specimen shows a whorled, myomatous appearance with prominent medial extension (arrow) and an ill-defined margin. The CT findings of perinephric disease are variable and include small curvilinear areas of high attenuation, soft-tissue attenuation nodules, thickening of the Gerota fascia, or a mass contiguous with retroperitoneal disease. Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. Mesoblastic nephroma generally exhibits benign behavior and is successfully treated with nephrectomy alone (,1). Ossifying renal tumor of infancy in a 2-month-old boy with an abdominal mass. (a, b) Longitudinal US scans of the right (a) and left (b) kidneys show multiple hypoechoic solid masses (arrowheads in a, arrows in b) and nephromegaly. Rarely, the lesion may recur locally if incompletely resected or metastasize to the lungs, brain, or bones. Viewer, Figure 1b. (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. (c) Contrast-enhanced CT scan shows a heterogeneous mass (curved arrows) infiltrating the right kidney and causing hydronephrosis. Journal of Pediatric Hematology / Oncology. 47, No. Renal lymphoma is characterized by multiple homogeneous masses, often with associated adenopathy. Angiography can demonstrate the characteristic dilated tortuous vessels with aneurysm formation, although some lesions are hypovascular. Histologic characteristics are not reliable for predicting the biologic behavior of the tumor. At histologic analysis, there is proliferation of spindle-shaped mesenchymal cells encasing nodules of embryonal epithelium. 3, © 2020 Radiological Society of North America, SCIENTIFIC EXHIBIT - Continuing Medical Education, https://doi.org/10.1148/radiographics.20.6.g00nv051585, Open in Image Figure 17c. Cases with predominant/prominent HEs have been reported as “ teratoid ” WT, albeit on the basis of poorly defined criteria. 15, No. 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. Angiomyolipoma frequently contains fat and is associated with tuberous sclerosis. (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). Occasionally chemotherapy can be administered prior to surgery to downstage the tumor 1. Therefore, it is currently recommended that patients be closely followed up for 1 year after surgical resection (,3,,12). Eighty percent develop metastases, most commonly to the lungs and less often to the liver, abdomen, brain, lymph nodes, or skeleton. 1,2 Subsequent studies confirmed its distinctive nature and the designation rhabomyosarcomatoid pattern was shortened to rhabdoid. (b) Photomicrograph (magnification reduced from ×330; hematoxylin-eosin stain) shows an acinar arrangement and clear cytoplasm. (Courtesy of Maria M. Rodriguez, MD, Miami, Fla; reprinted, with permission, from reference 7. Viewer, Figure 14. Yes.… (a) Photograph of a gross specimen of the clear cell type of renal cell carcinoma shows a golden color due to cytoplasmic lipids. Viewer. 13, No. SPICER, MB, BS. Viewer, Figure 21a. Wilms tumor in a 4-year-old boy with an abdominal mass. 1, 1 January 2008 | Veterinary Pathology, Vol. (Courtesy of Esperanza M. Tiamson, MD, Baltimore, Md; reprinted, with permission, from reference 7. Use the link below to share a full-text version of this article with your friends and colleagues. )Download as PowerPointOpen in Image Viewer, Figure 1a. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). Metastatic disease has not been documented, to our knowledge. (b) Photomicrograph (original magnification, ×50; hematoxylin-eosin stain) shows tubular (arrows) and acinar (A) structures adjacent to normal kidney on the right. However, they may occur in 40%–80% of patients with tuberous sclerosis (,19). MR imaging has been reported to be the most sensitive modality for determination of caval patency, but it requires sedation. At CT performed before intravenous administration of contrast material, the mass may be isoattenuating or hyperattenuating and small calcifications may be present (,30,,31). There is a slight male predominance (,10). Multilocular cystic renal tumor is suggested by a large mass with multiple cysts and little solid tissue. Figure 2b. ), Figure 6b. Teratoid Wilms' tumor is an unusual morphological entity characterized by a classic triphasic malignancy with predominantly heterologous tissue. 3, 1 July 2008 | RadioGraphics, Vol. We report a 9-month-old boy with bilateral Wilms' tumors who did not respond to multiagent chemotherapy and underwent right nephrectomy that showed a teratoid Wilms' tumor. )Download as PowerPointOpen in Image Nine of the 11 cases occurred in the left kidney, and in seven cases the upper pole calices were involved. Approximately 2% of pediatric renal malignancies are rhabdoid tumors 1. Figure 2b. In many children, the appropriate information for surgical planning may be obtained from meticulous US and contrast material–enhanced CT. Wilms tumor is occasionally largely cystic, in which case it may not be differentiated from cystic partially differentiated nephroblastoma, a subtype of multilocular cystic nephroma. The hypointense area in the medial spleen is due to partial volume artifact.Download as PowerPointOpen in Image Rhabdoid tumors occur exclusively in children, with 60% occurring before the age of 1 year of age, and 80% before the age of 2 years (average age of presentation = 11 months). A Teratoid Wilms’ Tumor with Raised Serum Alpha-Fetoprotein Level. 1, Seminars in Roentgenology, Vol. Local radiation therapy of the tumor bed is advocated in some cases, and complete abdominal irradiation can be used when there is gross tumor spillage at surgery or peritoneal tumor implantation. However, their incidence is nearly equal in the 2nd decade of life (,9). 38, No. 26, No. Journal of Family Medicine and Primary Care. Ossifying renal tumor of infancy in a 2-month-old boy with an abdominal mass. Rarely, angiomyolipoma may become locally aggressive and invade neighboring structures. 5, 27 October 2014 | Organogenesis, Vol. (c) CT scan shows multiple bilateral masses (arrows) with homogeneous enhancement.Download as PowerPointOpen in Image )Download as PowerPointOpen in Image (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). 10, 8 October 2009 | Pediatric Hematology and Oncology, Vol. At US, the mass has heterogeneous echogenicity, which represents hemorrhage, fat, necrosis, or calcification (,5). ), Nefroblastomatosis frente a tumor de Wilms: a propósito de un caso, Part II: Treatment of primary malignant non-Wilms' renal tumours in children, Part I: Primary malignant non-Wilms' renal tumours in children, CT imaging of mass-like renal lesions in children, Ultrasound Evaluation of Pediatric Abdominal Masses, Magnetic Resonance Imaging of the Urinary Tract in the Fetal and Pediatric Population, Displasia renal multiquística segmentaria: hallazgos radiológicos y diagnóstico diferencial, Tumores renales en niños menores de un año, Radiology–Pathology Conference: rhabdoid tumor of the kidney, Tumeurs du rein et des voies excrétrices chez l'enfant, Kindliche Nierentumoren — Relevanz der Bildgebung, Lactante de 9 meses con aumento unilateral del tamaño testicular, Mixed epithelial and stromal tumor of the kidney in a 12-year-old girl, Problems in Colon Cancer and a Child With Renal Lymphoma, Congenital Mesoblastic Nephroma Presenting with Neonatal Hypertension, Neoplasms of the Renal Medulla: Radiologic-Pathologic Correlation1, Benign renal mesenchymoma in the pediatric age group: a novel pathologic and karyotype entity, Renal tumors in infants less than 6 months of age, Imagerie des tumeurs rénales chez l’enfant, Casos en imagen 2.—Nefroma mesoblástico (tumor de bolande), Imaging of renal tumours in infancy and childhood, Imagerie des tumeurs néonatales : certitudes et incertitudes. Since the renal parenchyma does not contain lymphatic vessels, the existence of primary lymphoma in the kidney is unlikely. An abnormal WT1 gene is present in patients with WAGR syndrome (Wilms tumor, aniridia, genitourinary abnormalities, mental retardation) or Drash syndrome (male pseudohermaphroditism, progressive glomerulonephritis); an abnormal WT2 gene is present in patients with Beckwith-Wiedemann syndrome or hemihypertrophy. 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). (a, b) Longitudinal US scans of the right (a) and left (b) kidneys show multiple hypoechoic solid masses (arrowheads in a, arrows in b) and nephromegaly. (b) Photomicrograph (magnification reduced from ×330; hematoxylin-eosin stain) shows an acinar arrangement and clear cytoplasm. Severe retroperitoneal hemorrhage has been termed Wunderlich syndrome (,20). Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. (a) CT scan shows a right renal mass with heterogeneous enhancement and cystic foci (arrow). 83, No. 28, No. Figure 22b. The tumor quickly grows to fill the renal pelvis and invade vascular and lymphatic structures. (a) Abdominal radiograph obtained during intravenous urography shows lack of excretion from the right kidney. (a) Photograph of the cut surface of a gross specimen shows a focus of hemorrhage without necrosis. It is rare in neonates, with less than 0.16% of cases manifesting in this age group (,3). In patients with bilateral Wilms tumor, the current approach is tumor resection with sparing of normal parenchyma. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). Two of the authors (J.M.H., J.A.B.) Retroperitoneal disease occasionally leads to vascular and ureteral encasement. Renal lymphoma in a 16-year-old girl with no renal symptoms. Department of Radiology, Necmettin Erbakan University, Meram School of Medicine, Konya, Turkey Abstract. ), Figure 21a. (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). Fewer than 30 cases have been described. Viewer. (b) Contrast-enhanced CT scan shows heterogeneous enhancement of soft tissue within the lesions. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). There is heterogeneous enhancement at CT, and US shows heterogeneous echotexture. 23, No. The prognosis is very poor, with a pejorative and rapidly lethal evolution. (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 Viewer. Occurred in the spine, though previously reported, to our knowledge, all documented cases been... 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