Primary large-cell neuroendocrine carcinoma of the upper ureter

Abstract

Rationale: The incidence of primary large-cell neuroendocrine carcinoma (LCNEC) is extremely rare in the urinary tract. In the present study, we investigated a case of primary LCNEC associated with the upper ureter. Patient concern: A 58-year-old Korean female patient with right ureter mass, which was accidentally detected. An enhancing mass measuring 3.3 cm in size was found in the computed tomography (CT) scan. No definitive evidence of lymph node and distant metastasis was observed. Diagnosis: Histopathological analysis revealed large atypical epithelial cells in upper ureter mass, based on neuroendocrine morphology. Immunohistochemistry was positive for synaptophysin, CD 56, and cytokeratin. Accordingly, the upper ureter mass was finally diagnosed as LCNEC stage III, pT3 cN0 cM0. Intervention: Right nephroureterectomy was conducted. Outcomes: Multiple metastatic lesions were detected in the right adrenal, paracaval, and right pararenal space of the patient in a CT scan 3 months post-surgery. The patient chemotherapy and radiation therapy were proceeded for metastatic and recurred mass. But patient died by multiorgan failure Lessons: In summary, this case study demonstrated that LCNEC can develop even in the upper ureter for the first time, despite the absence of neuroendocrine cells in the normal urothelium. The occurrence of LCNEC in the ureter is still very rare but is possible. Therefore, further studies are needed to establish appropriate treatment strategies.

Urinary Tract Large Cell Neuroendocrine Carcinoma: Diagnostic, Prognostic and Therapeutic Issues

“…NE tumors rarely occur in the ureter; indeed, only 4 cases have been reported so far (Table IV) (59,(82)(83)(84). Usual presenting symptoms are ureteral obstruction or gross hematuria (85), as well as hematuria and leukocyturia in the urine sediment; since these tumors are unilateral, the occurrence of oligoanuria in a binephric patient should raise suspicion of a bladder primary causing bilateral ureteral obstruction rather than an upper urinary tract lesion.…”

Neuroendocrine Neoplasms of the Female Genitourinary Tract: A Comprehensive Overview

“…Large-cell NEC constitutes <0.5% of all the bladder urothelial carcinomas and is usually reported in male (77.1% vs. 23%), elderly patients (>60 years) [24,71,72]. In the urinary tract, the bladder is the most common site for the origin of large-cell NEC [73,74]. The urinary bladder (79.2%), followed by urethra (13%), pelvis (5%), and ureter (3%) are the most common sites of paraganglioma in the genitourinary tract [75,76].…”

“…Small-cell NEC of the ureter is usually observed in elderly patients, and around 15 cases have been observed in females so far [2,102,103]. As per our knowledge, less than 10 cases of large-cell NEC of the ureter [73,74] have been reported in the literature. Neuroendocrine cells are not usually found in the urinary tract.…”