Primary Upper Urinary Tract Small Cell Carcinoma: A Case Series and Literature Review

Abstract

Background: Primary upper urinary tract small cell carcinoma (SCC) is exceedingly rare with < 30 cases reported in the literature. Little is known about the incidence, diagnosis, treatment, and outcomes in these patients. We present a series of three patients with primary upper tract SCC. Case Presentation: Patient 1 is an 89-year-old Caucasian male who presented with hydroureteronephrosis and a mass in the proximal right ureter. Biopsy revealed SCC. Without further intervention, the patient died 2 months after his diagnosis. Patient 2 is a 67-year-old Caucasian female who underwent left laparoscopic nephroureterectomy for primary distal ureteral SCC, pT4N1M0. She developed lymphadenopathy and completed external beam radiation to the pelvis and four courses of cisplatin-based chemotherapy. She died from metastatic disease 7 months after diagnosis. Patient 3 is a 45-year-old female who underwent open right radical nephrectomy, retroperitoneal lymph node dissection, and hepatic metastasectomy for metastatic primary upper tract SCC, pT3N1M1. She underwent two subsequent retroperitoneal debulking procedures for recurrence followed by treatment with octreotide. She developed widespread metastasis and was treated with temozolomide and capecitabine before her death 80 months after diagnosis. Conclusion: This series contributes to the limited knowledge of the management and natural course of primary upper tract SCC. Patient 1 represents the first disease-specific mortality reported in a patient who received no therapy. Patient 3 represents the longest reported survival with metastatic disease, and the first treated with octreotide. The patient was managed with aggressive repeat surgical resection and exhibited 2 years of progression-free survival on octreotide. Emphasis should be placed on aggressive resection of all visible disease combined with the use of multimodal adjuvant chemoradiation for selected patients in this rare disease.

Keywords: Goblet Sign; neuroendocrine carcinoma; small cell carcinoma; upper tract; ureter.

FIG. 1.

Axial noncontrast CT section from Case 1 depicting a proximal right ureteral mass (white arrow, A). Coronal noncontrast CT section from Case 2 depicting a distal left ureteral mass (white arrow, B). Axial section of excretory phase-contrasted CT from Case 3 depicting a large, heterogeneous, enhancing right renal mass (C).

FIG. 2.

Retrograde pyelogram characterized by the classic “Goblet Sign” with filling defect (asterisks) indicative of ureteral obstruction from Case 1 (A). Hematoxylin and eosin-stained section revealing clusters of small palisading cells with hyperchromatic nuclei and crushed artifact (600 × , B). Cam 5.2 staining specific for neuroendocrine differentiation (600 × , C).