Renal tumours of childhood: an update (original) (raw)

Renal tumours of childhood: A review

Scripta Medica

Renal tumours of childhood are rare, although they are one of the most common solid tumours in children. They include numerous entities, which have different clinical, histological, molecular biological and prognostic features, so their precise diagnosis and staging are critical for appropriate treatment. The most common is Wilms' tumour (WT) with ~80-85 % of all cases, whereas other entities including mesoblastic nephroma, clear cell sarcoma, rhabdoid tumour, renal cell carcinoma, metanephric tumours and others are very rare (2-4 % each) which explains why they represent a big diagnostic challenge for diagnostic pathologists. They are subclassified into three risk groups - low, intermediate and high - which have different treatments and prognosis. There are two big study groups which have different approaches but remarkable similar outcomes. The International Society of Paediatric Oncology approach (followed in most of the world) is based on preoperative chemotherapy, followed ...

Surgical tactics in the treatment of malignant renal tumors in childhood

Pediatric Surgery International, 1997

The authors report the treatment results of 89 children with renal tumors (excluding carcinoma) seen from 1988 to 1992, 71 (79.8%) with favorable-histology Wilms' tumor (survival 86%) and 18 (20.2%) with unfavorable histology, including anaplastic nephroblastoma, clear-cell sarcoma, and rhabdoid tumor (survival 61%). Preoperative chemotherapy (ChT) was given to 46 patients (survival 84%); none was given to 43 with either tumors in the 1st year of life, massive hematuria, or tumor growth during ChT (survival 78%). There were 3 tumor ruptures in the latter group compared to 1 in the former group.

Childhood renal tumors: surgical treatment and results

Anatolian Current Medical Journal, 2022

Aim: Renal tumors in children are rare. Wilms' tumor (WT) is the most common renal tumor in childhood. The aim of this study is to investigate the data on the demographic factors, treatment, and follow-up results of pediatric patients who were operated on for renal tumors and determine the factors affecting mortality. Material and Method: Patients who were operated for renal tumor in our clinic in 2005-2020 were evaluated retrospectively. Age at diagnosis, gender, complaint, localization of the involved kidney, additional anomalies, tumor size, tru-cut biopsy performed, tumor stage, distant metastasis, applied treatments, pathology, treatment complications, and follow-up period were examined. Results: Of the 47 patients, 25 were female and 22 were male. The mean age was 45.46 (2-204) months. The tumor was located in the right in 21 cases, left in 23 cases and bilateral in three cases. The mean length of the tumor was 11.73 cm. At the time of diagnosis, there was metastasis in 15 cases (31.9%). While metastasis were the most common seen in lung (24.3%), bone-cell metastases were seen in clear cell carcinoma (33.3%) and brain metastases were seen in rhabdoid tumor (50%). Tru-cut biopsy was performed in 15 (31.9%) cases and biopsy was diagnostic in ten cases. Histopathologically, 41 cases had WT (87.23%), three cases were clear cell carcinoma (6.3%), two cases had anaplasia rhabdoid tumor (4.2%), and one case had metanephric adenoma. Relaps occured in nine cases (19.14%) during the follow-up period. Histopathologically, one case was a clear cell carcinoma, one case was a rhabdoid tumor, and seven cases were unilateral WT. The survival rate of our series was 89,4%. Conclusion: The most common renal tumor in childhood was WT. Surgery have no effect on survival; the most important survival factors were detecting the existence of rhabdoid tumors and anaplasia histopathologically. Therefore, during the followup and treatment of renal tumors in childhood, pathological examination should be of primary importance and followed up with the pediatric oncologists.

Part II: Treatment of primary malignant non-Wilms' renal tumours in children

The Lancet Oncology, 2007

Renal-cell carcinoma, clear-cell sarcoma, (congenital) mesoblastic nephroma, rhabdoid tumour, and renal medullary carcinoma form a heterogeneous group of childhood renal malignancies known as non-Wilms' tumours. Progress has been slow in improving the management of these tumours to decrease morbidity and increase survival. However, greater cooperation between national and international centres should engender specialisation, and an increased knowledge of the molecular biology of these tumours will inevitably lead to substantial progress over the next decade. This review is the second of two parts: the fi rst part provided an updated review of the clinical presentation, imaging, and pathology of non-Wilms' tumours and this second part provides an updated review of the treatment of these tumours.

Renal Tumors in Children Aged 10–16 Years: A Report From the United Kingdom Children's Cancer and Leukaemia Group

Pediatric and Developmental Pathology, 2011

Wilms tumor is the most common renal tumor of childhood. However, other epithelial, mesenchymal, and neuroectodermal neoplasms may also arise in the kidney during childhood, several of which show specific age distributions; in the 1st year of life, mesoblastic nephroma and rhabdoid tumor are more common, whereas renal cell carcinoma, primitive neuroectodermal tumor, and anaplastic Wilms tumors are relatively more frequent in older children and adolescents. The aim of this study is to describe the spectrum of renal tumors in children aged clinical trials of renal tumors in childhood. There were 67 (4.6%) tumors in children aged 10-16 years: 50 Wilms tumors (74.6%), 10 (14.9%) renal cell carcinomas, 3 (4.5%) renal medullary carcinomas, 2 (3%) primitive neuroectodermal tumors, 1 clear cell sarcoma of kidney, and 1 desmoplastic small round cell tumor. Fourteen percent of the Wilms tumors in this age group had diffuse anaplasia. Among the 10 renal cell carcinomas, 4 were associated with t(Xp-11.2), 3 were of papillary type II, 1 was papillary type I, 1 was clear cell type, and 1 was unclassified. Five-year overall survival for Wilms tumor was 63% (43% for anaplastic tumors), significantly lower than reported overall survival for all pediatric Wilms tumors. Only 40% of patients with renal cell carcinoma survived, and all patients with other tumors died.

Aggressive pediatric renal tumors

Seminars in Pediatric Surgery, 2019

This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Incidence, treatment, and outcomes of primary and recurrent Non-Wilms renal tumors in children: Report of 109 patients treated at a single institution

Journal of Pediatric Urology, 2020

This is a PDF file of an article that has undergone enhancements after acceptance, such as the addition of a cover page and metadata, and formatting for readability, but it is not yet the definitive version of record. This version will undergo additional copyediting, typesetting and review before it is published in its final form, but we are providing this version to give early visibility of the article. Please note that, during the production process, errors may be discovered which could affect the content, and all legal disclaimers that apply to the journal pertain.

Part I: Primary malignant non-Wilms' renal tumours in children

The Lancet Oncology, 2007

Non-Wilms' tumours form a small heterogeneous group of clinically signifi cant renal malignancies in children, including renal-cell carcinoma, clear-cell sarcoma, (congenital) mesoblastic nephroma, rhabdoid tumour, and renal medullary carcinoma. Good progress has been made in the assessment of these tumours, which has led to a greater understanding of the molecular changes that occur in their development. This review is the fi rst of two parts, and provides an updated review of the clinical presentation, imaging, and pathology of these tumours.