Small Cell Neuroendocrine Carcinoma of the Urinary Tract Successfully Managed with Neoadjuvant Chemotherapy

Abstract

Introduction. Small cell neuroendocrine carcinomas of the urinary tract is an extremely rare entity and very few cases have been reported in the literature. Small cell neuroendocrine carcinoma of the urinary tract (SCC-UT) is the association between bladder and urinary upper tract-small cell carcinoma (UUT-SCC). It characterized by an aggressive clinical course. The prognosis is poor due to local or distant metastases, and usually the muscle of the bladder is invaded. Case Presentation. We report a rare case of a 54-year-old Arab male native of moroccan; he is a smoker and was referred to our institution for intermittent hematuria. Following a diagnosis of small cell neuroendocrine carcinomas of the ureter and the bladder, thoracoabdominal-pelvic CT was done, and the staging of the tumor was done in the bladder (T2N0M0) and (T1N0M0) in the ureter. Neoadjuvant alternating doublet chemotherapy with ifosfamide/doxorubicin and etoposide/cisplatin was realized, and nephroureterectomy associated to a cystoprostatectomy was carried out. After 24 months of followup, no local or distant metastasis was detected. Conclusion. The purpose of this review is to present a rare case of pure small cell neuroendocrine carcinoma of the urinary tract and review the literature about the place of neoadjuvant chemotherapy in this rare tumors.

Clinicopathologic characteristics, therapy and outcomes of patients with primary ureteral small cell carcinoma: a case series and systematic review of the literature

“… 30 Our data have confirmed that the incidence of ureteral SCC is highest in Asian countries, which may be attributed to genetic susceptibility, dietary habits or environmental factors. 22 Consistent with previous reports, 21 the most commonly presenting symptoms were gross hematuria and flank pain. To date, no patient presented with paraneoplastic syndrome due to inappropriate hormone secretion.…”

Neuroendocrine Tumor of the Ureter: A Zebra Among Horses

“…These case reports utilizing Cisplatin-based neoadjuvant chemotherapy followed by surgery have reported disease-free survival times of 24 to 38 months, 13,14 whereas a prior study of 39 patients with small cell carcinoma of the upper urinary tract reported an overall survival time of only 15 months in patients managed with surgery alone or surgery with adjuvant chemotherapy. 5 Although validation in larger cohorts is needed, taken together these findings suggest that early institution of chemotherapy may be pivotal in improving survival in these patients.…”

Analysis of Clinicopathological Features and Prognostic Factors in 39 Cases of Bladder Neuroendocrine Carcinoma

“…For treatment, most scholars suggest combining radical bladder dissection and chemotherapy to improve clinical effectiveness. Of course, there are also some scholars adopting neoadjuvant chemotherapy, which has been shown to have good efficacy (4). In recent years, the development of molecular targeted therapy for malignant tumors has advanced and some investigators suggest that molecular targeted therapy of BNEC using epidermal growth factor receptor (EGFR), B-cell lymphoma 2 (Bcl-2) and CD56 might hold promise, potentially bringing significant improvement to the survival rate of BNEC patients (17).…”