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A Protocol for Rapid Post-mortem Cell Culture of Diffuse Intrinsic Pontine Glioma (DIPG)
Published on: March 7, 2017
[High risk Wilms' tumor in children. Clinico-pathological correlation based on 320 patients registered in the Polish
K Sawicz-Birkowska1, S M Bagłaj, A Balcerska
1Katedra i Klinika Chirurgii Pediatrycznej, Akademia Medyczna we Wrocławiu, Poland.
Insights
Unfavorable histology (UH) Wilms' tumor in children presents significant challenges, with a high relapse rate (43%) observed in this study. Early identification and tailored treatment strategies are crucial for improving outcomes in pediatric oncology.
Area of Science:
- Pediatric Oncology
- Nephrology
- Cancer Histopathology
Context:
- Wilms' tumor is the most common pediatric renal malignancy.
- The first Wilms' Tumour Study (01-92 schedule) treated 320 patients.
- Unfavorable histology (UH) is associated with poorer prognoses.
Purpose:
- To analyze the characteristics and outcomes of pediatric patients with unfavorable histology (UH) Wilms' tumor.
- To identify specific histological subtypes and clinical stages within the UH group.
- To evaluate relapse rates and mortality in this patient cohort.
Summary:
- Out of 320 patients, 42 (13.86%) had unfavorable histology (UH) Wilms' tumors.
- Histological subtypes included diffuse anaplasia (61.9%), focal anaplasia (23.8%), clear cell sarcoma of the kidney (CCSK) (7.1%), and mixedรีnal tumor (MRT) (7.1%).
- Relapse occurred in 43% of patients, with 7 deaths attributed to complications or disease progression.
Impact:
- This study highlights the aggressive nature of UH Wilms' tumors and the need for intensive management.
- Findings can inform treatment protocols and risk stratification for pediatric patients with Wilms' tumor.
- Further research into targeted therapies for UH subtypes may improve survival rates.
Abstract:
The records of 320 patients treated for Wilms' tumour in the first Wilms' Tumour Study (01-92 schedule) were reviewed and 42 children (13,86%) with unfavourable histology (UH) tumours were identified. There were 18 boys and 24 girls. Diffuse anaplasia was found in 26 patients (61,9%), focal anaplasia in 10 children (23,8%), CCSK and MRT were diagnosed in 3 patients each (7,1%). Clinical stages were: CS I - 5 (11,9%) patients, CS II N(-) - 7 (16,7%), CS II N(+) - 9 (21,4%), CS III - 15 (35,7%), CS IV - 5 (11,9%) and CS V - 1 patient (2,4%). Local and metastatic relapses of the disease occurred in 18 patients (43%). Seven of the 42 patients died, in 2 cases due to complications and in 5 from progression of the disease.
