Abstract

BackgroundExtrapulmonary small cell carcinomas have been reported in a variety of organs, and their incidence in the genitourinary tract is second only to that in the gastrointestinal tract. To date, however, only a few cases of small cell carcinoma of the ureter have been reported. Because the extreme rarity of this type of carcinoma, its clinical behaviour, diagnostic methods, and effective treatment modalities have not yet been determined.Case presentationA 59-year-old man presented with a 1-month history of painless gross haematuria. Urine cytopathology revealed a urothelial carcinoma and computed tomography revealed left hydronephroureterosis with a distal ureteral stone and a mildly enhanced fungating mass just below the stone-impacted site. The preoperative TNM stage was T2N0M0. The patient underwent simultaneous diagnostic ureterorenoscopy and left laparoscopic nephroureterectomy with bladder cuff resection. Gross examination showed a 3.5 × 3.0 × 0.8 cm white, partly yellow mass in the left distal ureter. Light microscopy showed a small cell carcinoma, overlaid on a urothelial carcinoma in situ, invading the ureter and external lateral resection margins. The small cell carcinoma was diffusely positive for neuron-specific enolase, and exhibited focal positivity for CD 56, synaptophysin, chromogranin and cytokeratin 20. The patient was treated with adjuvant chemotherapy, consisting of cisplatin and etoposide, and radiation therapy, and has been well, without evidence of tumour recurrence or metastasis in the 10 months after surgery.ConclusionSmall cell carcinoma of the ureter is rare. Although its clinical behaviour and diagnostic modalities have not been determined and it has yet to be diagnosed immunohistopathologically, multimodality treatment including surgery, chemotherapy and radiotherapy may improve patient survival.

Highlights

  • Extrapulmonary small cell carcinomas have been reported in a variety of organs, and their incidence in the genitourinary tract is second only to that in the gastrointestinal tract

  • Background most small cell carcinomas (SCCs) originate in the pulmonary system, extrapulmonary Small cell carcinoma (SCC) (ESCCs) have been reported in a variety of organs [1], with genitourinary Extrapulmonary small cell carcinomas (ESCC) being second in incidence only to gastrointestinal ESCCs [2]

  • We describe the clinical characteristics, pathological features and immunohistochemical findings of a primary malignant SCC combined with urothelial carcinoma

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Summary

Conclusion

We report a rare case of primary malignant SCC combined with urothelial carcinoma in the ureter. The clinical behaviour and methods of diagnosis of SCC of the ureter have not been established, preventing diagnosis prior to immunohistopathological examination of biopsied or resected samples, multimodal therapy including surgery, chemotherapy and radiotherapy may improve patient survival. Urologists should be aware of the possibility of SCC in the ureter in patients presenting with a ureteral mass. Further prospective studies and standardized diagnostic criteria are needed to clarify the epidemiology, diagnostic tools and most effective treatment protocol for SCC of the ureter. Consent Written informed consent was obtained from the patient for publication of this case report and any accompanying images. All authors read and approved the final manuscript

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