A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma

Stout, Thomas E.; Au, Jason; Hicks, John; Gargollo, Patricio C.

doi:10.1016/j.urology.2016.01.018

Cited by 7 publications

(11 citation statements)

References 12 publications

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“…A total of 108 patients presented with unilateral disease, while five presented with bilateral disease (4.4%) [8,[12][13][14][15], and none of the patients had metastatic disease. Three of the bilateral cases had CPDN in both kidneys [12,14,15]. Of the other two patients, one had CPDN with contralateral nephroblastomatosis [13] and one had CPDN with Wilms tumor, with focal anaplasia in the other kidney [8].…”

Section: Resultsmentioning

confidence: 99%

“…Stage I was recorded for 62 patients, stage II for one patient with tumor extension beyond the kidney [12], and stage III for nine patients. Stage III disease was based on invasion of the abdominal wall (n = 1) [17], pre-or intra-operative tumor rupture (n = 7) [8,12,18,19], or not specified [14]. In six patients, the tumor prolapsed into the ureter.…”

Section: Resultsmentioning

confidence: 99%

“…None of the pre-operatively treated patients showed response to chemotherapy on imaging or histological examination. In three of them, pre-operative imaging revealed an increase in tumor size after chemotherapy [14,15,23]. Seventy-eight patients underwent upfront surgery without chemotherapy.…”

Section: Treatment and Outcomementioning

confidence: 99%

“…Pre-operative chemotherapy administration was reported in 20 out of 98 patients with reported details on the pre-operative therapy and response (Table 2) [12,14,15,17,[19][20][21][22][23][24], including two of the five patients with bilateral disease [12,14]. None of the pre-operatively treated patients showed response to chemotherapy on imaging or histological examination.…”

Section: Treatment and Outcomementioning

confidence: 99%

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Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

Peer

Pleijte

Krijger

et al. 2021

Cancers

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In children presenting with a predominantly cystic renal tumor, the most likely diagnoses include cystic partially differentiated nephroblastoma (CPDN) and cystic nephroma (CN). Both entities are rare and limited information on the clinical and molecular characteristics, treatment, and outcome is available since large cohort studies are lacking. We performed an extensive literature review, in which we identified 113 CPDN and 167 CN. The median age at presentation for CPDN and CN was 12 months (range: 3 weeks–4 years) and 16 months (prenatal diagnosis–16 years), respectively. No patients presented with metastatic disease. Bilateral disease occurred in both entities. Surgery was the main treatment for both. Two/113 CPDN patients and 26/167 CN patients had previous, concomitant, or subsequent other tumors. Unlike CPDN, CN was strongly associated with somatic (n = 27/29) and germline (n = 12/12) DICER1-mutations. Four CPDN patients and one CN patient relapsed. Death was reported in six/103 patients with CPDN and six/118 CN patients, none directly due to disease. In conclusion, children with CPDN and CN are young, do not present with metastases, and have an excellent outcome. Awareness of concomitant or subsequent tumors and genetic testing is important. International registration of cystic renal tumor cohorts is required to enable a better understanding of clinical and genetic characteristics.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Treatment and Outcomementioning

confidence: 99%

Section: Treatment and Outcomementioning

confidence: 99%

See 2 more Smart Citations

Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

Peer

Pleijte

Krijger

et al. 2021

Cancers

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“…MCN is a very rarely seen benign entity characterized by multiple septations composed of differentiated tissue without blastemal elements slow-growing mesenchymal and epithelial neoplasm of kidney and there are more than 200 cases reported in the literature to the best of our knowledge (1) . Bimodal age distribution is often seen, accordingly it is mostly seen in children younger than 2 years and in the middle age (40-69 years) adults (male-to-female children ratio, 3:1 and adult female-to-male ratio, 9:1) (2) .…”

Section: Discussionmentioning

confidence: 99%

Adult Multiloculated Cystic Nephroma: A Case Report

Batmaz¹,

Uçar²,

Aktaş³

et al. 2020

Terh

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MRI Characteristics of Pediatric Renal Tumors: A SIOP‐RTSG Radiology Panel Delphi Study

Beek

Watson

Nievelstein

et al. 2021

Magnetic Resonance Imaging

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Background:The SIOP-Renal Tumor Study Group (RTSG) does not advocate invasive procedures to determine histology before the start of therapy. This may induce misdiagnosis-based treatment initiation, but only for a relatively small percentage of approximately 10% of non-Wilms tumors (non-WTs). MRI could be useful for reducing misdiagnosis, but there is no global consensus on differentiating characteristics. Purpose: To identify MRI characteristics that may be used for discrimination of newly diagnosed pediatric renal tumors. Study Type: Consensus process using a Delphi method. Population: Not applicable.

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A Case of Bilateral Cystic Partially Differentiated Nephroblastoma vs Cystic Wilms' Tumor: Highlighting a Diagnostic Dilemma

Cited by 7 publications

References 12 publications

Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

Clinical and Molecular Characteristics and Outcome of Cystic Partially Differentiated Nephroblastoma and Cystic Nephroma: A Narrative Review of the Literature

Adult Multiloculated Cystic Nephroma: A Case Report

MRI Characteristics of Pediatric Renal Tumors: A SIOP‐RTSG Radiology Panel Delphi Study

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