Clinical Considerations and Prognosis of Well-Differentiated Neuroendocrine Tumor Occurring Within a Renal Teratoma—A Case Series

Ungerer, Garrett; Steward, James; Akgul, Mahmut; Cheng, Liang; Sundaram, Chandru P.

doi:10.1016/j.clgc.2020.09.009

Cited by 6 publications

(16 citation statements)

References 26 publications

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“…In addition to horseshoe kidney, renal NETs have been found in mature cystic teratomas 12,16,20,22,35,39 . Ungerer et al 39 reviewed 10 cases of renal NET that had occurred within a teratoma and interestingly found half developing within a horseshoe kidney.…”

Section: Resultsmentioning

confidence: 99%

“…This, however, appears to be a relatively infrequent occurrence in renal NEN 16,20,27,30,40 occurring only in 12.7%–13.6% of cases 16,20 . Instead, renal NEN commonly present as an incidental finding on cross‐sectional imaging 31,32,37,39,48 with rates of asymptomatic cases ranging from 25% to 36.4% and reaching as much as 80% in an 11 patient case series 31 . In symptomatic patients, abdominal pain is the most common presenting symptom, followed by haematuria, abdominal mass, and constitutional symptoms such as weight loss and fever 1,11–14,16,17,24,27,30–33,37–46,48,49 .…”

Section: Resultsmentioning

confidence: 99%

“…Several case reviews, case series and review articles of primary renal NET show no significant sex predilection. 11,12,16,18,20,21,27,28,[30][31][32]39,42 However, in their literature review, Omiyale et al 13 noted that 62% of the 29 cases of renal NET occurred in females. Some other case series suggest a female predisposition to NET [34][35][36]40,50 ; however, these involved small patient numbers.…”

Section: Patient Demographics and Risk Factorsmentioning

confidence: 99%

“…In addition to horseshoe kidney, renal NETs have been found in mature cystic teratomas. 12,16,20,22,35,39 Ungerer et al 39 reviewed 10 cases of renal NET that had occurred within a teratoma and interestingly found half developing within a horseshoe kidney. This association was more common in older patients, with 72.7%-85.7% of cases occurring in the fourth to seventh decade.…”

Section: Patient Demographics and Risk Factorsmentioning

confidence: 99%

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Primary renal neuroendocrine neoplasms: A systematic literature review, report of four local cases, and original survival analysis of 63 patients from a national registry 2012–2018

Paisey

Weerasuriya

Palmer

et al. 2022

J Neuroendocrinology

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Primary renal neuroendocrine neoplasms (NEN) are rare. We aimed to conduct a systematic review, present local cases, and analyse data from the England's National Cancer Registration and Analysis Service (NCRAS) to provide comprehensive evidence on clinical experience, incidence, and survival to better characterize these tumours. First, a systematic review using the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) method; second, a synthesis of local cases; and, finally, a retrospective population-based cohort analysis of renal NEN recorded between 2012 and 2018 on NCRAS were performed. Kaplan-Meier estimator was used to calculate overall survival and Cox proportional hazard regression to identify prognostic factors. Systematic review identified 48 articles and the evidence was summarized and presented. We reported data from four local cases presenting with abdominal and back pain but without carcinoid syndrome. In population-based analysis, we identified 63 cases of renal NEN between 2012 and 2018 from the registry. Age-standardized incidence was 0.09-0.32 per million with a median age of 64 years (interquartile range = 48-72 years). Survival was worse in males and those aged 64 years and over. Five-year survival for renal neuroendocrine tumours (NET) was 69.8% (95% confidence interval = 66.6-72.7) and neuroendocrine carcinomas (NEC) was 38.4% (95% confidence interval = 34.6-42.0). No independent predictive factor was identified in the multivariable analyses. We have given a systematic review of evidence, published local experience, and reported incidence and survival of renal NEN in England for the first time. We have provided clinicians with evidence on diagnosis and proposed a treatment algorithm of theses rare tumours. The incidence and median age of presentation in England is similar to other published series. Renal NET has better survival than renal NEC as expected. A uniform classification system would reduce inconsistencies in reporting and standardize treatment decisions for this neoplasia.

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Section: Resultsmentioning

confidence: 99%

Section: Resultsmentioning

confidence: 99%

Section: Patient Demographics and Risk Factorsmentioning

confidence: 99%

Section: Patient Demographics and Risk Factorsmentioning

confidence: 99%

See 2 more Smart Citations

Primary renal neuroendocrine neoplasms: A systematic literature review, report of four local cases, and original survival analysis of 63 patients from a national registry 2012–2018

Paisey

Weerasuriya

Palmer

et al. 2022

J Neuroendocrinology

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“…6 In the minireview article "Clinical Considerations and Prognosis of Well-Differentiated Neuroendocrine Tumor Occurring Within a Renal Teratoma by Ungerer et al" collectively reported an extremely Editor's Preview rare disease entity that 11 cases have thus far been documented in the world. 7 In the article "Systemic Analysis and Review of Nivolumabipilimumab Combination as a Rescue Strategy for Renal Cell Carcinoma After Treatment With Anti-PD-1/PD-L1 Therapy by Carril-Ajuria et al" compiled 4 studies to address the utility of Ipilimumab plus Nivolumab combination in patients progressed on anti-PD-1/PD-L1 treatment. A total of 237 patients were included.…”

Section: Kidney Cancermentioning

confidence: 99%

The 2021 Variant Histology Issue

Hsieh¹

2021

Clinical Genitourinary Cancer

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Prognostic factors and treatment impact on overall survival in patients with renal neuroendocrine tumour

Omidele,

Connors,

Wainganker

et al. 2024

BJUI Compass

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BackgroundRenal neuroendocrine neoplasms (R‐NEN) are exceptionally rare tumours characterized by high mortality rates.ObjectiveThe objective of this study is to analyse prognostic factors and treatment impact on overall survival in patients with R‐NEN.Design, setting and participantsWe identified all patients with R‐NEN in the National Cancer Database (NCDB) from 2004 to 2019 and identified prognostic factors for improved survival.Results and limitationsOf 542 R‐NEN cases, 166 (31%) were neuroendocrine tumour grade 1 (NET‐G1), 14 (3%) were neuroendocrine tumour grade 2 (NET‐G2), 169 (31%) were neuroendocrine carcinoma (NEC‐NOS), 18 (3%) were large cell neuroendocrine carcinoma (LC‐NEC) and 175 (32%) were small cell neuroendocrine carcinoma (SC‐NEC). Median overall survival for all patients in the study was 44.88 months (SE, 4.265; 95% CI, 27.57–62.19). Median overall survival was 7.89 months (SE 0.67; 95% CI, 6.58–9.20) for patients without surgical intervention and 136.61 months (SE 16.44; 95% CI, 104.38–168.84, p < 0.001) for patients who underwent surgery. Increased age (HR, 1.05; 95% CI, 1.03–1.06; p < 0.001), T4 stage disease (HR, 3.17; 95% CI, 1.96–5.1; p < 0.001), NEC‐NOS histology (HR, 2.82; 95% CI, 1.64–4.86; p < 0.001), LC‐NEC histology (HR, 2.73; 95% CI, 1.04–7.17; p = 0.041) and SC‐NEC histology (HR, 5.17; 95% CI, 2.95–9.05; p < 0.001) were all positive predictors of worsening overall survival. The main limitation of the study is its retrospective design.ConclusionR‐NEN is an aggressive tumour characterized by high mortality rates. Surgery continues to be the mainstay of treatment and has shown to provide a survival benefit for most patients.Patient SummaryR‐NEN is composed of several tumour histologies that differ based on their aggressiveness with NEC‐NOS and SC‐NEC being the most lethal. Surgery, predominantly through minimally invasive approaches, is the mainstay of treatment and has a clear survival benefit.

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Clinical Considerations and Prognosis of Well-Differentiated Neuroendocrine Tumor Occurring Within a Renal Teratoma—A Case Series

Cited by 6 publications

References 26 publications

Primary renal neuroendocrine neoplasms: A systematic literature review, report of four local cases, and original survival analysis of 63 patients from a national registry 2012–2018

Primary renal neuroendocrine neoplasms: A systematic literature review, report of four local cases, and original survival analysis of 63 patients from a national registry 2012–2018

The 2021 Variant Histology Issue

Prognostic factors and treatment impact on overall survival in patients with renal neuroendocrine tumour

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