Urinary Neuroendocrine Neoplasms Treated in the "Modern Era": A Multicenter Retrospective Review
Bryan Khuong Le1, Patrick McGarrah2, Alan Paciorek3
1Department of Medicine, University of California, San Francisco, CA.
Background:
Primary urinary neuroendocrine neoplasms (U-NENs) are extremely rare thus optimal treatment is unknown. Grading and treatment are typically extrapolated from other primary sites. Since 2010, the clinical landscape for NENs has changed substantially. We performed a retrospective review of U-NENs to assess treatment patterns and oncologic outcomes of patients treated in the recent era of NEN therapy.
Patients And Methods:
A multicenter retrospective review of patients diagnosed after 2005 and alive after 2010. Time to treatment failure (TTF) was used to evaluate progression and toxicity for systemic therapy. Tumors were categorized as having either well-differentiated neuroendocrine tumor (WDNET) or poorly differentiated neuroendocrine carcinoma (PDNEC) histology.
Results:
A total of 134 patients from 6 centers were included in our analysis, including 94 (70%) bladder, 32 (24%) kidney, 2 (1.5%) urethra and 4 other urinary primaries (3.0%). Poorly-differentiated neuroendocrine carcinoma was more common in bladder (92%) than non-bladder tumors (8%). Median Ki-67 available in bladder primary was 90% (n = 24), kidney 10% (n = 23), ureter 95% (n = 1), urethra 54% (n = 2), and others 90% (n = 3). Patients received a median of 2 therapies (range 0-10). Median time to death was not reached in locoregional WDNETs versus 8.2 years (95% CI, 3.5-noncalculable) in metastatic WDNETs (predominantly renal primary). Median time to death was 3.6 years (95% CI, 2.2-9.2) in locoregional PDNECs versus 1 year (95% CI, 0.8-1.3) in metastatic PDNECs (predominantly bladder primary).
Conclusion:
This is the most extensive series examining treatment patterns in patients with U-NENs in the recent era of NEN therapy. The apparent inferior survival for bladder NENs is likely due to the preponderance of PDNECs in this group. As predicted, treatments for U-NENs mirrored that of other more common NENs. In our retrospective cohort, we observed that patients with WD-UNETs treated with peptide receptor radionuclide therapy (PRRT) and everolimus suggested potential activity for disease control in WD-UNETs. Prospective studies are needed to assess the activity of new oncology drugs in UNENs.
Insights
Primary urinary neuroendocrine neoplasms (U-NENs) are rare, and this study analyzed treatment patterns and outcomes in the recent era. Patients with well-differentiated U-NENs showed potential disease control with PRRT and everolimus.
Area of Science:
- Oncology
- Urology
- Medical Science
Background:
- Primary urinary neuroendocrine neoplasms (U-NENs) are exceptionally rare, leading to uncertainty in optimal treatment strategies.
- Current management often relies on extrapolations from NENs at other primary sites.
- The clinical landscape for neuroendocrine neoplasms (NENs) has evolved significantly since 2010.
Purpose of the Study:
- To retrospectively review U-NENs to assess treatment patterns.
- To evaluate oncologic outcomes for patients treated in the recent era of NEN therapy.
- To analyze treatment failure and toxicity for systemic therapies.
Main Methods:
- Multicenter retrospective review of patients diagnosed after 2005 and alive after 2010.
- Categorization of tumors into well-differentiated neuroendocrine tumor (WDNET) or poorly differentiated neuroendocrine carcinoma (PDNEC) histology.
- Utilized time to treatment failure (TTF) to assess progression and toxicity.
Main Results:
- 134 patients with U-NENs (bladder, kidney, urethra, others) were analyzed.
- Poorly-differentiated neuroendocrine carcinoma (PDNEC) was more prevalent in bladder primaries (92%).
- Median time to death varied significantly by histology and metastatic status, with PDNECs having poorer outcomes.
Conclusions:
- This study represents an extensive examination of U-NEN treatment patterns in the current NEN therapy era.
- Inferior survival in bladder NENs is likely linked to the high proportion of PDNECs.
- Peptide receptor radionuclide therapy (PRRT) and everolimus showed potential for disease control in well-differentiated U-NENs (WD-UNETs), warranting prospective investigation.
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