Pediatric Renal Masses: Wilms Tumor and Beyond1
Lisa H. Lowe, MD,
Bernardo H. Isuani, MD,
Richard M. Heller, MD,
Sharon M. Stein, MD,
Joyce E. Johnson, MD,
Oscar M. Navarro, MD and
Marta Hernanz-Schulman, MD
1 From the Departments of Radiology and Radiological Sciences (L.H.L., B.H.I., R.M.H., S.M.S., M.H.S.) and Pathology (J.E.J.), Vanderbilt University Children's Hospital and Medical Center, D-1120 Medical Center North, 1211 22nd Ave S, Nashville, TN 37232; and the Department of Diagnostic Imaging, Hospital for Sick Children, Toronto, Ontario, Canada (O.M.N.). Presented as a scientific exhibit at the 1999 RSNA scientific assembly. Received March 1, 2000; revision requested March 28 and received May 15; accepted May 16. Address correspondence to M.H.S. (e-mail: marta.schulman@mcmail.vanderbilt.edu).
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Figure 1a. Wilms tumor. (a) Photograph of a gross specimen shows a lesion that extends into the renal pelvis. The lesion has a pseudocapsule, and its surface is divided by septa. (b) Photomicrograph (original magnification, x62.5; hematoxylin-eosin stain) shows the triphasic pattern of stromal (curved arrow), blastemal (straight arrow), and tubular (arrowhead) elements. (Courtesy of Maria M. Rodriguez, MD, Miami, Fla; reprinted, with permission, from reference 7.)
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Figure 1b. Wilms tumor. (a) Photograph of a gross specimen shows a lesion that extends into the renal pelvis. The lesion has a pseudocapsule, and its surface is divided by septa. (b) Photomicrograph (original magnification, x62.5; hematoxylin-eosin stain) shows the triphasic pattern of stromal (curved arrow), blastemal (straight arrow), and tubular (arrowhead) elements. (Courtesy of Maria M. Rodriguez, MD, Miami, Fla; reprinted, with permission, from reference 7.)
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Figure 2a. Wilms tumor in a 4-year-old boy with an abdominal mass. (a) CT scan shows a left renal mass with heterogeneous enhancement (thick arrow) and multiple hepatic metastases (thin arrows). (b) CT scan obtained at a higher level again shows multiple hepatic metastases in addition to tumor thrombus within the portal veins (arrows).
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Figure 2b. Wilms tumor in a 4-year-old boy with an abdominal mass. (a) CT scan shows a left renal mass with heterogeneous enhancement (thick arrow) and multiple hepatic metastases (thin arrows). (b) CT scan obtained at a higher level again shows multiple hepatic metastases in addition to tumor thrombus within the portal veins (arrows).
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Figure 3. Wilms tumor in a 3-year-old boy with an abdominal mass. 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. The hypointense area in the medial spleen is due to partial volume artifact.
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Figure 4. Hyperplastic perilobar nephroblastomatosis. 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. (Courtesy of Frederic B. Askin, MD, St Louis, Mo; reprinted, with permission, from reference 7.)
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Figure 5a. Nephroblastomatosis in a 16-month-old boy with an abdominal mass. (a) Plain radiograph shows displacement of bowel loops into the midabdomen by bilateral flank masses. (b) Contrast-enhanced CT scan shows large, homogeneous Wilms tumors (W) superimposed on a background of multiple peripheral nephrogenic rests (arrowheads).
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Figure 5b. Nephroblastomatosis in a 16-month-old boy with an abdominal mass. (a) Plain radiograph shows displacement of bowel loops into the midabdomen by bilateral flank masses. (b) Contrast-enhanced CT scan shows large, homogeneous Wilms tumors (W) superimposed on a background of multiple peripheral nephrogenic rests (arrowheads).
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Figure 6a. Renal cell carcinoma. (a) Photograph of a gross specimen of the clear cell type of renal cell carcinoma shows a golden color due to cytoplasmic lipids. (b) Photomicrograph (magnification reduced from x330; hematoxylin-eosin stain) shows an acinar arrangement and clear cytoplasm. (Reprinted, with permission, from reference 7.)
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Figure 6b. Renal cell carcinoma. (a) Photograph of a gross specimen of the clear cell type of renal cell carcinoma shows a golden color due to cytoplasmic lipids. (b) Photomicrograph (magnification reduced from x330; hematoxylin-eosin stain) shows an acinar arrangement and clear cytoplasm. (Reprinted, with permission, from reference 7.)
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Figure 7a. Renal cell carcinoma in a teenage boy. (a) Coronal T1-weighted MR image shows a large right renal mass (arrows) that is isointense to renal parenchyma except for a central area of high signal intensity. (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). (Courtesy of Michael Ambrosino, MD, New York University Medical Center.)
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Figure 7b. Renal cell carcinoma in a teenage boy. (a) Coronal T1-weighted MR image shows a large right renal mass (arrows) that is isointense to renal parenchyma except for a central area of high signal intensity. (b) Gadolinium-enhanced coronal T1-weighted MR image shows enhancement of the lesion (arrows). (Courtesy of Michael Ambrosino, MD, New York University Medical Center.)
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Figure 8. Mesoblastic nephroma. Photograph of the cut surface of a gross specimen shows a whorled, myomatous appearance with prominent medial extension (arrow) and an ill-defined margin. Very little normal kidney (K) remains visible. (Courtesy of Jane Chatten, MD, Philadelphia, Pa; reprinted, with permission, from reference 7.)
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Figure 9a. Mesoblastic nephroma in a 5-week-old male infant with an in utero renal mass. (a) Longitudinal US scan shows a mass of mixed echotexture replacing the lower pole of the right kidney (arrows). (b) CT scan shows moderate enhancement (arrow).
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Figure 9b. Mesoblastic nephroma in a 5-week-old male infant with an in utero renal mass. (a) Longitudinal US scan shows a mass of mixed echotexture replacing the lower pole of the right kidney (arrows). (b) CT scan shows moderate enhancement (arrow).
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Figure 10. Cystic partially differentiated nephroblastoma. Photograph of a gross specimen shows a cystic mass with well-defined, smooth margins between the lesion and normal kidney. (Courtesy of Esperanza M. Tiamson, MD, Baltimore, Md; reprinted, with permission, from reference 7.)
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Figure 11a. Cystic partially differentiated nephroblastoma in a 12-year-old girl with an abdominal mass. (a) CT scan shows a right renal mass with heterogeneous enhancement and cystic foci (arrow). (b) CT scan obtained at a lower level shows numerous septa and a rind of enhancing solid tissue.
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Figure 11b. Cystic partially differentiated nephroblastoma in a 12-year-old girl with an abdominal mass. (a) CT scan shows a right renal mass with heterogeneous enhancement and cystic foci (arrow). (b) CT scan obtained at a lower level shows numerous septa and a rind of enhancing solid tissue.
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Figure 12. Clear cell sarcoma. Photomicrograph (original magnification, x62.5; hematoxylin-eosin stain) shows characteristic histologic findings, with numerous cytoplasmic vesicles. The cells contain extensive clear cytoplasm with displaced nuclei. (Reprinted, with permission, from reference 7.)
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Figure 13a. Clear cell sarcoma in a 13-month-old girl with an abdominal mass. (a) CT scan shows extensive central necrosis within a mass arising from the right kidney. (b) CT scan obtained at a higher level shows heterogeneous enhancement (arrows) with mass effect and hydronephrosis of adjacent calices (arrowheads). (Courtesy of Tim Booth, MD, Children's Medical Center, Dallas, Tex.)
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Figure 13b. Clear cell sarcoma in a 13-month-old girl with an abdominal mass. (a) CT scan shows extensive central necrosis within a mass arising from the right kidney. (b) CT scan obtained at a higher level shows heterogeneous enhancement (arrows) with mass effect and hydronephrosis of adjacent calices (arrowheads). (Courtesy of Tim Booth, MD, Children's Medical Center, Dallas, Tex.)
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Figure 14. Rhabdoid tumor. Photograph of a gross specimen shows a round, lobulated mass with a nonspecific appearance. A small amount of normal kidney (K) is noted at the edge of the specimen. (Reprinted, with permission, from reference 7.)
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Figure 15. Rhabdoid tumor in an 8-month-old boy with hematuria. CT scan shows a left renal mass with heterogeneous enhancement (arrow). (Courtesy of Tim Booth, MD, Children's Medical Center, Dallas, Tex.)
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Figure 16a. Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. (a) Nonenhanced CT scan shows multiple fat-attenuation foci within the kidneys (arrowheads). (b) Contrast-enhanced CT scan shows heterogeneous enhancement of soft tissue within the lesions. (c) CT scan of the lung bases shows multiple bilateral small cysts.
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Figure 16b. Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. (a) Nonenhanced CT scan shows multiple fat-attenuation foci within the kidneys (arrowheads). (b) Contrast-enhanced CT scan shows heterogeneous enhancement of soft tissue within the lesions. (c) CT scan of the lung bases shows multiple bilateral small cysts.
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Figure 16c. Angiomyolipoma in a 17-year-old girl with tuberous sclerosis. (a) Nonenhanced CT scan shows multiple fat-attenuation foci within the kidneys (arrowheads). (b) Contrast-enhanced CT scan shows heterogeneous enhancement of soft tissue within the lesions. (c) CT scan of the lung bases shows multiple bilateral small cysts.
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Figure 17a. Renal medullary carcinoma in a 10-year-old boy with hematuria and sickle cell trait. (a) Abdominal radiograph obtained during intravenous urography shows lack of excretion from the right kidney. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). (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). (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.
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Figure 17b. Renal medullary carcinoma in a 10-year-old boy with hematuria and sickle cell trait. (a) Abdominal radiograph obtained during intravenous urography shows lack of excretion from the right kidney. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). (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). (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.
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Figure 17c. Renal medullary carcinoma in a 10-year-old boy with hematuria and sickle cell trait. (a) Abdominal radiograph obtained during intravenous urography shows lack of excretion from the right kidney. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). (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). (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.
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Figure 17d. Renal medullary carcinoma in a 10-year-old boy with hematuria and sickle cell trait. (a) Abdominal radiograph obtained during intravenous urography shows lack of excretion from the right kidney. Normal excretion from the left kidney is noted, as is contrast material in the small intestine from a prior CT scan. (b) Longitudinal US scan of the right kidney shows loss of corticomedullary differentiation and hydronephrotic calices (arrows). (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). (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.
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Figure 18. Ossifying renal tumor of infancy. Photomicrograph (original magnification, x62.5; hematoxylin-eosin stain) shows an osteoid core (O) with a peripheral tubule (T) and mesenchyme or fibrous connective tissue.
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Figure 19a. Ossifying renal tumor of infancy in a 2-month-old boy with an abdominal mass. (a) Nonenhanced CT scan shows a calcification within the left kidney (arrow). (b) Contrast-enhanced CT scan obtained at the same level shows a low-attenuation mass (arrows) surrounding the calcification.
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Figure 19b. Ossifying renal tumor of infancy in a 2-month-old boy with an abdominal mass. (a) Nonenhanced CT scan shows a calcification within the left kidney (arrow). (b) Contrast-enhanced CT scan obtained at the same level shows a low-attenuation mass (arrows) surrounding the calcification.
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Figure 20a. Metanephric adenoma. (a) Photograph of the cut surface of a gross specimen shows a focus of hemorrhage without necrosis. (b) Photomicrograph (original magnification, x50; hematoxylin-eosin stain) shows tubular (arrows) and acinar (A) structures adjacent to normal kidney on the right. Nuclei are homogeneous and bland without mitotic activity. (Courtesy of G. Taylor, Hospital for Sick Children, Toronto, Ontario, Canada; reprinted, with permission, from reference 31.)
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Figure 20b. Metanephric adenoma. (a) Photograph of the cut surface of a gross specimen shows a focus of hemorrhage without necrosis. (b) Photomicrograph (original magnification, x50; hematoxylin-eosin stain) shows tubular (arrows) and acinar (A) structures adjacent to normal kidney on the right. Nuclei are homogeneous and bland without mitotic activity. (Courtesy of G. Taylor, Hospital for Sick Children, Toronto, Ontario, Canada; reprinted, with permission, from reference 31.)
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Figure 21a. Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (Reprinted, with permission, from reference 31.)
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Figure 21b. Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (Reprinted, with permission, from reference 31.)
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Figure 21c. Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (Reprinted, with permission, from reference 31.)
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Figure 21d. Metanephric adenoma in a 9-year-old girl with three episodes of urinary tract infection. (a) Longitudinal US scan shows a well-defined, hypoechoic mass in the right kidney (arrows) with a central focus of echogenicity (arrowhead). (b) Longitudinal power Doppler US scan shows decreased vascularity of the mass (arrows) compared with that of normal adjacent kidney. (c) Nonenhanced CT scan shows a hyperattenuating mass (black arrows) with a central low-attenuation focus (white arrow). (d) Contrast-enhanced CT scan shows mild homogeneous enhancement within the mass (black arrows) except for the persistent low-attenuation focus (white arrow). (Reprinted, with permission, from reference 31.)
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Figure 22a. Renal lymphoma in a 16-year-old girl with no renal symptoms. (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. (c) CT scan shows multiple bilateral masses (arrows) with homogeneous enhancement.
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Figure 22b. Renal lymphoma in a 16-year-old girl with no renal symptoms. (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. (c) CT scan shows multiple bilateral masses (arrows) with homogeneous enhancement.
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Figure 22c. Renal lymphoma in a 16-year-old girl with no renal symptoms. (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. (c) CT scan shows multiple bilateral masses (arrows) with homogeneous enhancement.
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Copyright © 2000 by the Radiological Society of North America.
