• Nuclear Medicine and Molecular Imaging
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• 제42권sup1호
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• pp.137-139
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• 2008

Wilms Tumor is a great therapeutic success story within pediatric oncology. Therefore, accurate initial staging is needed to assess tumor spread and to assign patients appropriately to the different risk branches. However, it is recognized that FDG-PET can provide useful information about tumor and has better accuracy than CT and MRI for staging, but its role in Wilms tumor is unclear. According to clinical research data, FDG PET may be useful for the management of selected patients with Wilms tumors.

• Clinical and Experimental Pediatrics
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• 제48권1호
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• pp.112-115
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• 2005

저자들은 8세 남아에서 교통사고 후 우연히 발견된 혈흉과 복부 종물의 진단 과정에서 확진된 폐 및 골 전이를 동반한 후복강 내 신외 윌름 종양 1례를 경험하였기에 문헌 고찰과 함께 보고 하고자 한다.

• Clinical and Experimental Pediatrics
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• 제59권sup1호
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• pp.96-98
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• 2016

Wilms' tumor is the most common malignant renal tumor in childhood. The brain metastasis of a Wilms' tumor with anaplastic histopathology is rare. We present the case of an 8-year-old girl with Wilms' tumor, who presented with multiple brain metastases 5 years after her primary diagnosis. The brain masses were diagnosed after a generalized tonic-clonic seizure attack. The big solid mass in the cerebellum was resected, and whole-brain radiotherapy was performed, after which, she succumbed to her disease. In the case of clinical suspicion, cranial surveillance should be included in the routine clinical work-up for Wilms' tumor. Combined aggressive therapy (surgery+radiotherapy+chemotherapy) should be applied whenever possible, for both better survival and palliative aspects.

• Korean Journal of Radiology
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• 제22권7호
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• pp.1185-1193
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• 2021

Objective: Clear cell sarcoma of the kidney (CCSK) is the second-most common but extremely rare primary renal malignancy in children after Wilms' tumor. The aims of this study were to evaluate the imaging features that could distinguish between CCSK and Wilms' tumor and to assess the features with diagnostic value for identifying CCSK. Materials and Methods: We reviewed the initial contrast-enhanced abdominal-pelvic CT scans of children with CCSK and Wilms' tumor between 2010 to 2019. Fifty-eight children (32 males and 26 females; age, 0.3-10 years), 7 with CCSK, and 51 with Wilms' tumor, were included. The maximum tumor diameter, presence of engorged perinephric vessels, maximum density of the tumor (Tmax) of the enhancing solid portion, paraspinal muscle, contralateral renal vein density, and density ratios (Tmax/muscle and Tmax/vein) were analyzed on the renal parenchymal phase of contrast-enhanced CT. Fisher's exact tests and Mann-Whitney U tests were conducted to analyze the categorical and continuous variables, respectively. Logistic regression and receiver operating characteristic curve analyses were also performed. Results: The age, sex, and tumor diameter did not differ between the two groups. Engorged perinephric vessels were more common in patients in the CCSK group (71% [5/7] vs. 16% [8/51], p = 0.005). Tmax (median, 148.0 vs. 111.0 Hounsfield unit, p = 0.004), Tmax/muscle (median, 2.64 vs. 1.67, p = 0.002), and Tmax/vein (median, 0.94 vs. 0.59, p = 0.002) were higher in the CCSK compared to the Wilms' group. Multiple logistic regression revealed that engorged vessels (odds ratio 13.615; 95% confidence interval [CI], 1.770-104.730) and Tmax/muscle (odds ratio 5.881; 95% CI, 1.337-25.871) were significant predictors of CCSK. The cutoff values of Tmax/muscle (86% sensitivity, 77% specificity) and Tmax/vein (71% sensitivity, 86% specificity) for the diagnosis of CCSK were 1.97 and 0.76, respectively. Conclusion: Perinephric vessel engorgement and greater tumor enhancement (Tmax/muscle > 1.97 or Tmax/vein > 0.76) are helpful for differentiating between CCSK and Wilms' tumor in children aged below 10 years.

• Journal of Genetic Medicine
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• 제15권1호
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• pp.38-42
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• 2018

WAGR (Wilms tumor, aniridia, genitourinary anomalies, and mental retardation) syndrome is a rare contiguous gene deletion syndrome caused by deleting genes including WT1 and PAX6 genes in 11p13 region, which is characterized by Wilms tumor, aniridia, genitourinary abnormalities, and intellectual disability. We report the clinical and cytogenetic characteristics of one Korean patient with WAGR syndrome. The patient shows bilateral sporadic aniridia and genital anomalies at 2 months of age. A heterozygous 14.5 Mb interstitial deletion of 11p14.3p12 region was detected by array comparative genomic hybridization. At 2 years and 10 months of age, Wilms tumor is found through regularly abdominal ultrasonography and treated by chemotherapy, radiation therapy and surgery.

• Advances in pediatric surgery
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• 제3권1호
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• pp.71-76
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• 1997

A 3-year-old boy with a Wilms' tumor had unusually severe hypertension, polydipsia, polyuria and hypokalemia. Physical examination on admission was unremarkable except for the presence of a smooth, firm mass in the right abdomen. Computerized tomography showed a tumor occupying the upper two thirds of the right kidney. Plasma renin activity and aldosterone concentration were markedly elevated, 37.7 mg/ml/hour(normal in supine position 0.15-2.33 mg/ml/hour) and 120.1 ng/dL(normal in supine position 1 to 16 ng/dL), respectively. Hypertension varied from 150/90 mmHg to 240/180 mmHg, and was not effectively controlled by antihypertensive drugs. Right nephrectomy was performed on the sixth hospital day. At laparatomy, there was no evidence of mechenical compression of the renal artery by the tumor. The tumor, about 8 cm in diameter, was confined to the renal capsule without involvement of the renal blood vessels at the hilum. Histopathology was Wilms' tumor of favorable histology. On electron microscopy, tumor cells contained intracytoplasmic electron dense secreting graules, suggesting the possibility of renin secreting tumor cells. Shortly after nephrectomy, signs and symptoms were relieved dramatically, and plasma renin activity and aldosterone concentration were also decreased to normal.

• Advances in pediatric surgery
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• 제6권2호
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• pp.156-159
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• 2000

Neuroblastoma arises from the embryonic tissue of the adrenergic rest. It is commonly found in children and mostly in nonrenal tissue. We present a case of intrarenal neuroblastoma which was initially thought to be a Wilms' tumor. The patient was a 18 months-old girl treated with radical nephrectomy and adjuvant chemotherapy after operation. The neoplasm within the kidney in children cannot always indicate Wilms' tumor. Neuroblastoma of the adrenal gland or retroperitoneal tissue may often compress or invade the kidney directly or arise from the kidney. Clinical aspects that differentiate between neuroblastoma and Wilms' tumor are discussed with a review of the literatures.

• Clinical and Experimental Pediatrics
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• 제49권1호
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• pp.99-102
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• 2006

윌름씨 종양은 주로 1-5세에 복부에 생기는 종양으로 약 25% 이상에서 고혈압을 동반하지만 심각한 고혈압에 의하여 심부전을 보이는 경우는 매우 드물다. 저자들은 3세된 여자 아이가 윌름씨 종양으로 인한 고레닌 혈증으로 다음, 다뇨, 저나트륨성 고혈압과 심부전을 보였으나 수술적으로 종양을 제거한 후 증상 호전을 보였기에 문헌 고찰과 함께 보고하는 바이다.

• Clinical and Experimental Pediatrics
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• 제51권12호
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• pp.1355-1358
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• 2008

WAGR 증후군은 윌름즈 종양, 무홍채증, 비뇨 생식기계 기형, 정신지체 증상을 동반하는 증후군이다. 이는 윌름즈 종양 유전자인 WT1와 무홍채증 유전자 PAX6를 포함하는 11번 염색체 단완의 13째 부분의 결실에 의해 유발된다. 이에 저자들은 태어나서부터 양측성 무홍채증을 가졌고 복부팽만과 정신지체를 주소로 내원한 2세 여아에서 염색체 검사에서 11p11.2-13의 결실을 보인 국내 최초의 WAGR 증후군을 보고하는 바이다. 양측성 윌름즈 종양은 항암제와 수술로 성공적으로 치료하였고, 환아는 항암치료 종료 후 19개월째 정상적인 신기능을 보이며 생존하고 있다.

• Clinical and Experimental Pediatrics
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• 제45권3호
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• pp.390-394
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• 2002

Wilms 종양은 소아기에 발생하는 고형중양 중 6- 7%를 차지하며 소아에서 신장에 발생하는 고형종양 중 가장 흔한 것으로 진단시 정중연령은 3-5세이고 대부분 10세 이하에서 발견된다. 무증상성 복부종괴로 발견되는 경우가 많으며 하대정맥을 침범하는 경우는 4-10%에서 발견되며 우심방까지 침범한 경우도 0.5- 3%로 발견되나 우심실까지 침범한 경우는 아직 보고된 바가 없다. 2년 7개월 된 남자 환아로 내원 2개월 전부터의 간헐적 혈뇨 및 복통을 주소로 내원 하였다. 내원 당시 이학적 검사상 심잡음이 청진 되었으며 심한 복부팽만과 늑골하 종괴가 촉지 되었다. 이에 시행한 복부 초음파, 전산화 단층촬영 및 심에코상 우측 신장에서 기시하여 하대정맥을 통해 우심방과 우심실까지 침범한 종괴가 관찰되었다. 수술 전 항암 화학요법을 시행 받았으며, 항암 화학요법 후 20일째 수술을 하여 모든 종괴를 제거 후 조직학적 소견상 Wilms 종양을 확진 받았다. 환아는 수술후 방사선 치료 및 항암 화학 요법을 진행 중이다. 저자들은 우측 신장에서 기원하여 하대정맥을 통해 우심방 및 우심실까지 종양색전을 형성한 Wilms 종양 환아 1례를 경험하였기에 보고하는 바이다.