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Wilms’ tumor, Stage I (Stage III at biopsy) with favorable histology

Patterson RAP - MedPix

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Wilms’ tumor, Stage I (Stage III at biopsy) with favorable histology

History: This 2.5-year-old girl presented to her pediatrician in Italy after her parents noted a prominence in the right abdomen after bathing her in March 2004. The child had consistently been at the fifth percentile for growth up to that point. An ultrasound in Italy revealed a 7 x 8cm abdominal mass, and she was immediately air-evac’d to WRAMC for further evaluation and treatment.

Findings: CT in the axial plane demonstrates a heterogeneous mass in the right kidney, with considerable mass effect. The liver and IVC are markedly compressed with no obvious invasion. Initial measurements of this mass were 15.4 cm in the craniocaudal dimension, 12.3 cm in the AP dimension, and 7.7 cm in the lateral dimension. The left kidney is normal with no evidence of masses or hydronephrosis. Coronal and sagittal reconstruction views demonstrate a well-circumscribed mass that has most likely infiltrated the right renal capsule. Cystic regions within the mass are most notable on the sagittal view. The lateral image shows medial displacement of functioning right renal parenchyma (i.e., claw sign) with contrast excretion, and there is normal excretion from the left kidney. The right adrenal gland and right renal vein are not well visualized. Followup CT after 5 weeks of preoperative chemotherapy shows considerable tumor shrinkage and more defined areas of margination. The tumor now measures 10.5 cm in the craniocaudal dimension, 8 cm in the AP dimension, and 5.2 cm in the lateral dimension. The right renal vein is not well visualized. Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus. Flow is seen throughout the lumen.

Ddx: Wilms’ tumor Clear cell sarcoma Mesoblastic nephroma Nephroblastomatosis Neuroblastoma Complex teratoma Cystic renal dysplasia

Dxhow: Surgical resection and pathologic examination

Exam: BP 137/90 Pulse 112 Resp 28 Wt 11kg Ht 80.6cm BMI 16.9 Temp 36.2&#730;C Gen: Crying but consolable; child is small for age but proportionate. Height is < 5th percentile for age, weight just at 5th percentile for age. HEENT: Normal. Oropharynx is clear. Neck supple with no lymphadenopathy. Lungs: CTAB CV: No murmurs, RRR Abd: Distended but nontender. +BS. Palpation c/w large firm mass filling right side of the abdomen and extending below the umbilicus. Lower edge of mass is palpable about the level of the anterior iliac spine. GU: Only superficial visual inspection of perineal region. Normal female genitalia with no labial edema or obvious rash. Ext: No C/C/E Neuro: CN 2-12 intact, reflexes symmetric. Normal gait. Skin: No lesions or rashes. SOFT TISSUE, RIGHT RENAL MASS, BIOPSIES: Tissue suboptimal for definitive evaluation. Small round blue cell neoplasm. Unable to evaluate the architecture of the neoplasm. Immunostains for vimentin, LCA, synaptophysin and chromogranin all appear negative while keratin appears weakly positive. The differential diagnosis includes Wilms tumor, clear cell sarcoma, or other small round blue cell neoplasm, although the negative immunostains would seem to exclude neuroblastoma, lymphoma and Ewing's sarcoma.

No MeSH data available.


Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus.
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MPX1279_synpic24376: Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus.


Wilms’ tumor, Stage I (Stage III at biopsy) with favorable histology

Patterson RAP - MedPix

Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus.
© Copyright Policy - open-access
Related In: Results  -  Collection

License
Show All Figures
getmorefigures.php?uid=MPX1279&req=5

MPX1279_synpic24376: Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus.

View Article: MedPix Image - MedPix Case

Affiliation: Uniformed Services University

ABSTRACT

Diagnosis: Wilms’ tumor, Stage I (Stage III at biopsy) with favorable histology

History: This 2.5-year-old girl presented to her pediatrician in Italy after her parents noted a prominence in the right abdomen after bathing her in March 2004. The child had consistently been at the fifth percentile for growth up to that point. An ultrasound in Italy revealed a 7 x 8cm abdominal mass, and she was immediately air-evac’d to WRAMC for further evaluation and treatment.

Findings: CT in the axial plane demonstrates a heterogeneous mass in the right kidney, with considerable mass effect. The liver and IVC are markedly compressed with no obvious invasion. Initial measurements of this mass were 15.4 cm in the craniocaudal dimension, 12.3 cm in the AP dimension, and 7.7 cm in the lateral dimension. The left kidney is normal with no evidence of masses or hydronephrosis. Coronal and sagittal reconstruction views demonstrate a well-circumscribed mass that has most likely infiltrated the right renal capsule. Cystic regions within the mass are most notable on the sagittal view. The lateral image shows medial displacement of functioning right renal parenchyma (i.e., claw sign) with contrast excretion, and there is normal excretion from the left kidney. The right adrenal gland and right renal vein are not well visualized. Followup CT after 5 weeks of preoperative chemotherapy shows considerable tumor shrinkage and more defined areas of margination. The tumor now measures 10.5 cm in the craniocaudal dimension, 8 cm in the AP dimension, and 5.2 cm in the lateral dimension. The right renal vein is not well visualized. Sedated abdominal U/S demonstrates no right renal vein thrombus, although there is considerable mass effect and tortuosity of the right renal vein. The IVC is clearly visualized with no evidence of intraluminal thrombus. Flow is seen throughout the lumen.

Ddx: Wilms’ tumor Clear cell sarcoma Mesoblastic nephroma Nephroblastomatosis Neuroblastoma Complex teratoma Cystic renal dysplasia

Dxhow: Surgical resection and pathologic examination

Exam: BP 137/90 Pulse 112 Resp 28 Wt 11kg Ht 80.6cm BMI 16.9 Temp 36.2&#730;C Gen: Crying but consolable; child is small for age but proportionate. Height is < 5th percentile for age, weight just at 5th percentile for age. HEENT: Normal. Oropharynx is clear. Neck supple with no lymphadenopathy. Lungs: CTAB CV: No murmurs, RRR Abd: Distended but nontender. +BS. Palpation c/w large firm mass filling right side of the abdomen and extending below the umbilicus. Lower edge of mass is palpable about the level of the anterior iliac spine. GU: Only superficial visual inspection of perineal region. Normal female genitalia with no labial edema or obvious rash. Ext: No C/C/E Neuro: CN 2-12 intact, reflexes symmetric. Normal gait. Skin: No lesions or rashes. SOFT TISSUE, RIGHT RENAL MASS, BIOPSIES: Tissue suboptimal for definitive evaluation. Small round blue cell neoplasm. Unable to evaluate the architecture of the neoplasm. Immunostains for vimentin, LCA, synaptophysin and chromogranin all appear negative while keratin appears weakly positive. The differential diagnosis includes Wilms tumor, clear cell sarcoma, or other small round blue cell neoplasm, although the negative immunostains would seem to exclude neuroblastoma, lymphoma and Ewing's sarcoma.

No MeSH data available.