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Retrospective analysis to determine outcomes of patients with bilateral Wilms tumor undergoing nephron sparing surgery: 15-year tertiary single-institution experience

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Abstract

Purpose

To describe our clinical experience with nephron sparing surgery (NSS) for bilateral Wilms tumor and evaluate the outcomes of patients treated at one of the largest pediatric medical centers in China.

Methods

Medical records of children with bilateral Wilms tumor undergoing NSS in the Children’s Hospital of Chongqing Medical University during a 15-year period were retrospectively analyzed. Data collected were composed of age at surgery, tumor response, tumor rupture during resection, final pathologic margins, use of radiation therapy, pathology reports, renal function, and patient survival.

Results

A total of 18 eligible patients (10 males, 8 females) with bilateral Wilms tumor at a mean age of 2.28 ± 1.12 years were identified. The administration of preoperative chemotherapy did not result in universally successful outcomes. All children underwent successfully unilateral or bilateral NSS, of which one had positive pathologic margins and five received radiation therapy postoperatively. The rates of tumor rupture and positive lymph nodes involvement were 11.1 and 19.4%, respectively. The pathological study showed favorable histology and unfavorable histology in 32 and 4 kidneys, respectively. The 4-year event-free survival and overall survival rates were 68.18 and 85.56%. In univariable analysis, tumor histology (p = 0.0028) and disease stage (p = 0.0303) appeared significantly associated with overall survival. After a median follow-up period of 41.5 months (range 10–89), three of the surviving patients were diagnosed with hypertension and one had renal insufficiency.

Conclusions

Our experience suggests that NSS has become a feasible and effective option with good oncologic outcomes. Further research, ideally in a multicenter randomized manner, is warranted to better assess the role of NSS in this challenging clinical scenario.

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Abbreviations

WT:

Wilms tumor

NSS:

Nephron sparing surgery

PN:

Partial nephrectomy

RN:

Radical nephrectomy

NWTSG:

National Wilms Tumor Study Group

COG:

Children’s Oncology Group

SIOP:

Société Internationale d’Oncologie Pédiatrique

eGFR:

Estimated glomerular filtration rate

VAD:

Vincristine, actinomycin D, and doxorubicin

OS:

Overall survival

EFS:

Event-free survival

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Funding

The authors did not receive any funding for this study. They have no financial disclosures.

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Authors

Contributions

Xiao-Hui Tan conceived and designed the study, read and analyzed documents, and drafted the paper. De-Ying Zhang and Xing Liu conducted the document search, read and analyzed the documents, and revised the manuscript. Da-Wei He, Xu-Liang Li, Tao Lin, and Guang-Hui Wei conceived and designed the study, advised on the search, read and analyzed documents. All authors take equal responsibility for guaranteeing the work.

Corresponding authors

Correspondence to Xing Liu or Da-Wei He.

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Tan, XH., Zhang, DY., Liu, X. et al. Retrospective analysis to determine outcomes of patients with bilateral Wilms tumor undergoing nephron sparing surgery: 15-year tertiary single-institution experience. Pediatr Surg Int 34, 427–433 (2018). https://doi.org/10.1007/s00383-018-4232-6

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