• Advances in pediatric surgery
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• v.3 no.1
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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.

• Korean Journal of Radiology
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• v.22 no.7
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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.

• Childhood Kidney Diseases
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• v.10 no.1
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• pp.1-7
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• 2006

Osteopontin (OPN) is a glycosylated phosphoprotein which mediates cell adhesion and migration, and is produced by bone, macrophages, endothelial cells, and epithelial cells. The many regulatory functions of OPN include bone remodeling, tumor invasion, wound repair, and promotion of cell survival. It is produced by renal tubular epithelial cells, and expression is upregulated in glomerulonephritis, hypertension, ischemic acute renal failure, renal ablation, and UUO. In this review, we discuss about osteopontin in general aspect, expression, role on the development and pathologic condition of neonatal kidney.

• Clinical and Experimental Pediatrics
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• v.59 no.sup1
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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.

• Clinical and Experimental Pediatrics
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• v.62 no.4
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• pp.144-147
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• 2019

The most common type of refractory hypertension found in children is secondary hypertension, which is a potentially curable disease. Reninoma, a renin-secreting juxtaglomerular cell tumor, is a rare cause of severe hypertension that is usually diagnosed in adolescents and young adults. Surgical resection of the tumor completely cures the hypertension of patients with reninoma. The typical clinical presentation of reninoma includes hypokalemia, metabolic alkalosis, and features secondary to the increased activation of the renin-angiotensin system without renal artery stenosis. We report a case of reninoma in a female adolescent with a typical clinical presentation, in which surgical removal of the tumor completely cured hypertension. We discuss here the clinical features, imaging studies, and immunohistochemical examination of the tumor used to establish the diagnosis of reninoma and for the management of the condition.

• Childhood Kidney Diseases
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• v.21 no.1
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• pp.31-34
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• 2017

Tumor lysis syndrome is a serious complication of malignancy, resulting from the massive and rapid release of cellular components into the blood. Generally, it occurs after initiation of chemotherapy. The onset of spontaneous tumor lysis syndrome (STLS) before anti-cancer treatment is rare and occurs mostly in Burkitt lymphoma and non-Hodgkin's lymphoma. There are only a few case reports in children. Here, we report a case of STLS secondary to T-cell acute lymphoblastic leukemia (ALL), which presented with urinary stone and subsequent acute kidney injury with severe hyperuricemia. Occult malignancy should be considered in case of unexplained acute kidney injury with extreme hyperuricemia.

• Advances in pediatric surgery
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• v.10 no.1
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• pp.52-55
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• 2004

A newborn male baby was transferred to our hospital with a left inguinal mass. The mass was huge measuring $10{\times}10cm$, engorged, and dark-blue colored as a result of internal hemorrhage. Unstable vital signs were combined with DIC and acute renal failure. Emergency operation was performed because of the suspicion of bowel perforation. The peritoneal cavity was full of ascitis and the distal jejunum had a 0.5 cm perforation. Segmental resection of the jejunum and incisional biopsy of the inguinal mass were performed. On pelvic and thigh MRI, the mass protruded into pelvic cavity and encircled large vessels and nerves of the thigh. Pathologic diagnosis was congenital infantile fibrosarcoma. Fifteen days after operation, primary tumor excision was undertaken. The second look operation, performed after 6 times VAC chemotherapy, revealed no remained malignant cell on microscopic section. The baby has been followed closely for the last eight months.

• Advances in pediatric surgery
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• v.7 no.2
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• pp.162-165
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• 2001

Clear Cell Sarcoma of the Kidney(CCSK) is a rare malignant childhood tumor with frequent metastasis to the bone. We report a case of right sided in a 5 month-old girl. A radical nephrectomy was performed. It was clinical stage III with renal capsular invasion and lymph node metastasis by the classification of NWTS-5. Histologic examination revealed the classic pattern of CCSK. Postoperative adjuvant chemotherapy with doxorubicin and radiotheraphy were applied.

• Asian Pacific Journal of Cancer Prevention
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• v.15 no.17
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• pp.7277-7280
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• 2014

This analysis was conducted to evaluate the efficacy and safety of carboplatin based chemotherapy in treating pediatric patients with Wilms tumors. Methods: Clinical studies evaluating the efficacy and safety of carboplatin based regimens on response and safety for pediatric patients with Wilms tumors were identified using a predefined search strategy. Pooled response rates (RRs) of treatment were calculated. Results: In carboplatin based regimens, 4 clinical studies which including 127 patients with advanced Wilms tumors were considered eligible for inclusion. With this carboplatin based chemotherapy, 2 clinical studies included carboplatin, ifosfamide and etoposide. Systemic analysis suggested that, in all patients, the pooled PR was 64.5% (82/127) in carboplatin based regimens. Thrombocytopenia and leukocytopenia were the main side effects. No grade III or IV renal or liver toxicity was observed. No treatment related death occurred with carboplatin based treatment. Conclusion: This systemic analysis suggests that carboplatine based regimens are associated with a reasonable response rate and accepted toxicities for treating pediatric patients with Wilms tumors.

• Childhood Kidney Diseases
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• v.5 no.2
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• pp.225-230
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• 2001

Multilocular cystic nephroma is a rare disease, noninherited benign renal neoplasm occurring in both children and adults. It is necessary to make a differential diagnosis from all renal diseases with a cystic component, such as Wilms tumor, harmatoma or polycystic dysplastic kidney in childhood. There are about only 200 case reports in the world since Walter Edmunds had described it first. We report a case of multilocular cystic nephroma presented with painless abdominal mass, treated with nephrectomy and confirmed with pathology. (J. Korean Soc Pediatr Nephrol 2001 ;5 : 219-24)