Childhood Renal Tumor: A Report from a Chinese Children’s Cancer Group

Cai, Jiaoyang; Pan, Ci; Lü, Qing; Yan, Jie; Ju, Xiuli; Ma, Fuyin; Zhu, Yiping; Liu, Qiuling; Sun, Lirong; Lian, Jiqin; Cao, Lizhi; Fu, Li; Liu, Zhigang; Qiao, Lijing; Huang, Dongsheng; Tian, Xin; Tang, Jingyan

doi:10.1155/2014/894341

Cited by 4 publications

(3 citation statements)

References 14 publications

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“…Shanghai is rich in medical resources, making radiotherapy and timely diagnosis accessible. Two previous reports in the literature have demonstrated that lack of RT was a factor contributing to poor survival [ 20 , 21 ]. Our findings are also consistent with a recent personal communication by the Shanghai CDC (Centers for Disease Prevention and Control) that described an 83% 5-year survival rate of childhood renal malignant tumors in Shanghai (2002–2016).…”

Section: Discussionmentioning

confidence: 99%

Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021)

Zhang,

Yuan,

Jiang

et al. 2024

PLoS ONE

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Background Although clear cell sarcoma of kidney (CCSK) is rare, it is the second most common renal tumor in children after Wilms’ tumor. NWTS and SIOP are two major groups which had made tremendous efforts on renal tumors, but the strategies are different, for NWTS follows the upfront surgery principle providing definite pathology and the SIOP follows the upfront chemotherapy principle, each has its own advantages. Here we aimed to evaluate the outcomes of CCSK in China following NWTS strategies to analyze the prognostic factors. Methods For this multicenter retrospective study, a total of 54 patients were enrolled from three children’s hospitals, between April 2003 and December 2021. Treatment comprised upfront radical nephrectomy, followed by radiotherapy and intensive chemotherapy. Clinical records were regularly updated. Prognostic factors and survival rates were evaluated. Results The 54 enrolled patients had a median age of 37 months (range, 4 months to 11.4 years). The stage distribution was 16% stage I (n = 9), 30% stage II (n = 16), 39% stage III (n = 21), and 15% stage IV (n = 8). Among stage IV, metastasis sites included the lung (n = 6), bone (n = 1), and intra-orbital/cervical lymph node (n = 1). After a median follow-up of 5.6 years, the 5-year event-free survival (EFS) was 82.4±5.4%, and overall survival was 88.1±4.6%. The EFS was 100% for stage I, 93.8 ±6.1% for stage II, 71.1±10.0% for stage III, and 68.6±18.6% for stage IV. Univariate analysis revealed that staging (III/IV), tumor rupture, and inferior vena cava tumor thrombus were inferior prognostic factors. Multivariate analysis revealed that tumor rupture was independent poor prognostic factor (P = 0.01, HR 5.9). Among relapsed patients, relapse occurred a median of 11 months after diagnosis (range, 4–41 months), and 50% (4/8) achieved a second complete remission after multiple treatment. None of the six lung metastasis patients received lung RT, only one patient developed a relapse and was salvaged by RT after relapse. Conclusions Tumor rupture was independent poor prognostic factor. Upfront surgery of NWTS strategies can make a definite pathology diagnosis, but how to reduce tumor rupture during surgery is important especially in developing countries. The outcomes of patients with stage I–III CCSK in China were comparable to findings in other developed countries. Better outcomes were achieved in stage IV CCSK by using an intensive chemotherapy regimen including carboplatin, which require further confirmation by AREN0321. Lung RT may be safely omitted in selected patients who achieve a compete radiographic response after 6 weeks of systemic treatment (including surgery). Treatment should be encouraged even in CCSK cases with metastasis and relapse.

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Section: Discussionmentioning

confidence: 99%

Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021)

Zhang,

Yuan,

Jiang

et al. 2024

PLoS ONE

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“…The mainland Chinese encountered a higher incidence of anaplastic histology and a correspondingly lower 5-year EFS of 72%. 20 The Taiwanese experience was intermediate, reporting a 5-year EFS of 78%. 21 Yet, in the latter publications it is not known how geographical distances and practice standards, or other confounders may have affected the reported outcomes.…”

Section: Discussionmentioning

confidence: 99%

Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

Loke

Wong

Tawng

et al. 2018

Intl Journal of Cancer

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Wilms tumor demonstrates significant interethnic epidemiological, histological and outcome differences, and is rare and poorly studied among Asians. We compared the clinicopathological, and loss of heterozygosity (LOH) profile and survival outcomes of Asian and non-Asian patients with Wilms tumor. Clinical charts and histological slides from patients with malignant renal tumors over a period of 20 years were retrospectively reviewed. We adapted a genotyping assay to determine 1p36 and 16q21-22 LOH in formalin-fixed paraffin-embedded (FFPE) specimens, and compared these characteristics between Asian and non-Asian patients. Fifty-three (79.1%) Asian and 14 (20.9%) non-Asian patients had Wilms tumors. Compared to non-Asians, Asians were younger (mean 4.6 and 4.0 years, respectively), had more equal gender distribution (female: male = 1.8 and 1.0, respectively), fewer tumors with unfavorable histology (25.0% and 4.1%, respectively, p = 0.05), and less advanced disease at presentation, yet similar nodal metastases rates (16.7% and 18.4%, respectively). No Asian patients had bilateral tumors. Our adapted genotyping assay accurately determined LOH in FFPE specimens <10 years post-fixation. Among 30 Asian patients, 1p and 16q LOH were each detected in 5 (16.7%) patients, respectively-similar to rates reported in other ethnicities. Yet after similar treatment with National Wilms Tumor Study regimens, 15-year event-free and overall survival for Asian patients was 95.7% and 96.3% respectively. In summary, despite similar nodal metastasis and LOH rates, Asian patients had fewer unfavorable histology tumors, lower-stage disease, and better survival outcomes. The bases for these differences and implications on treatment strategy for these patients warrant further study.

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“…Following initial surgery or biopsy, patients were recommended for chemotherapy protocol according to their primary tumor location and stages. Malignant rhabdoid tumors of the kidney (MRTK) that received initial resections would follow WT (4) protocol (Carboplatin, Cyclophosphamide, Doxorubicin, Vincristine, and Etoposide) proposed by the Fifth National Wilms Tumor Study Group (NWTSG-5) for 27 weeks (7,8). For those MRTK patients received biopsy only, the WT (5) protocol was employed and then patients were reevaluated for radical surgery.…”

Section: Treatments (Scmc Multimodal Protocol)mentioning

confidence: 99%

An analysis of the diagnosis, clinical characteristics, treatment, and survival outcomes of 36 extracranial malignant rhabdoid tumor patients

Shan¹,

Cai²,

Han³

et al. 2021

Transl Pediatr

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Background: Extracranial malignant Rhabdoid tumors (eMRTs) are rare but aggressive lesions in young children. This work aimed to review and analyze the diagnosis, clinical characteristics, treatment, and survival of eMRTs so as to summarize experience for future therapy.Methods: A total of 36 eMRT cases were treated between January 2008 and August 2019 according to Shanghai Children's Medical Center (SCMC) multimodal protocol of mixed surgery, radiation and chemotherapy involving vincristine, carboplatin, doxorubicin, etoposide, cyclophosphamide and ifosfamide.We collected information including: age at diagnosis, tumor location, disease stage, therapy, outcomes, etc. Overall survival (OS) and event free survival (EFS) were calculated and risk factors for survival were analyzed. Results:The patients had a median age of 1.80 years at diagnosis (range, 1.4 m-13.42 years), and were followed up for 9.17 months in median (range, 4 d-11.14 y). A total of 16 patients achieved complete remission (CR), and 7 survived without reoccurrence till December 2019. The 3-year EFS was 17.4% (95% CI: 11.0-23.8%) with a 3-year OS of 23.4% (95% CI: 15.8-31.0%). Recurrence was found only in children younger than the median age (1.80 y). Localized staging (Log Rank P=0.039 for OS and P=0.021) and older age (Log Rank P=0.016 for OS and P=0.002 for EFS) were associated with improved outcome. Younger age

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Childhood Renal Tumor: A Report from a Chinese Children’s Cancer Group

Cited by 4 publications

References 14 publications

Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021)

Outcomes of children with clear cell sarcoma of kidney following NWTS strategies in Shanghai China (2003–2021)

Clinical, pathological and loss of heterozygosity differences in Wilms tumors between Asian and non‐Asian children

An analysis of the diagnosis, clinical characteristics, treatment, and survival outcomes of 36 extracranial malignant rhabdoid tumor patients

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