Contemporary management of upper tract urothelial cell carcinoma

Abstract

Upper tract urothelial cell carcinoma (UTUCC), formerly known as transitional cell carcinoma of the upper urinary tract, is a rare oncologic disease in Western countries. Thus its disease process and its management are not as well defined as other urologic cancers. We are reviewing the current evidence based literature available to develop a plan for the treatment of UTUCC. A PubMed search was completed using the key words “upper tract urothelial cell carcinoma”, “epidemiology”, “risk factor”, “treatment” and “prognosis”. Six hundred fifty two articles were found. We narrowed our search to articles published between January 2004 and June 2016 for a more contemporary review of the topic. Four hundred seventy articles were then available for review. Further detailed search was performed for relevance on the topic and hundred one articles were selected for the review. Many risk factors have been found to be associated with the development of UTUCC, including tobacco use. Patients are often asymptomatic and may only present with microscopic or gross hematuria. Tumor grade and stage are pivotal in determining the treatment options for UTUCC. Advancements in endoscopic techniques have aided in the diagnosis, grading and treatment of this disease. Treatment options include topical therapy, with combinations of methotrexate, vinblastine, doxorubicin and cisplatin or gemcitibine or cisplatin, endoscopic resection, segmental ureterectomy and ureteral implantation, and nephroureterectomy, including bladder cuff. Treatment recommendations depend on tumor grade and stage, renal function, tumor location and the patient’s prognosis. There are currently no tissue or blood-based biomarkers available to accurately monitor the disease. Further studies of gene expression and biomarkers may hopefully improve the management of this disease. Although rare in many countries, UTUCC is becoming more prevalent due to exposure to carcinogenic herbal remedies and other identifiable risk factors. Numerous treatment modalities, both surgical and chemotherapeutic, have been utilized to treat both low and high grade UTUCC tumors. Additional clinical trials are necessary to further develop methods for screening, treatment, and surveillance to improve management.

Keywords: Urothelial cell carcinoma, Diagnosis, Upper tract, Epidemiology, Risk factors, Treatment, Prognosis

Core tip: To review the current literature on upper tract urothelial cell carcinoma (UTUCC) and provide a contemporary management plan for treatment based on best available evidence. Large randomized controlled trials are lacking in UTUCC due to the fortunately rare occurrence of the disease. Treatment recommendations for the primary lesion, imaging, and follow-up in this review are based on the stage and grade of the tumor. Early diagnosis and aggressive treatment remains the mainstay of therapy for UTUCC.