Granulocyte Colony-stimulating Factor-producing Upper Urinary Tract Carcinoma: Systematic Review of 46 Cases Reported in Japan

Abstract

AimsGranulocyte colony-stimulating factor (G-CSF)-producing upper urinary tract carcinoma is extremely rare, and we do not yet have a comprehensive understanding of the disease. This study was carried out to determine the characteristics of G-CSF-producing upper urinary tract carcinoma. Materials and methodsA systematic MEDLINE and ICHUSHI WEB (Japan Medical Abstract Society) search was carried out to identify articles and conference proceedings describing patients with G-CSF-producing upper urinary tract carcinoma. The final cohort included 46 patients: eight studies were published in English, 16 in Japanese and there were 18 Japanese conference proceedings. ResultsThe average age of patients was 67 years and the male to female ratio was 2.5 to 1. The mean white blood cell count was as high as 33 900/μl (range 10 000–121 000) in these patients. Pretreatment serum G-CSF levels were measured in 23 patients, all of which were higher (range 55–1220 pg/ml) than normal levels. Metastasis was detected in 29 patients (63%) and lymph node and lung metastases were well observed. The most commonly reported primary treatment was surgery (33 patients), but the median survival period for these patients was short (4.5 months). Multivariate analysis showed that lymph node and/or distant metastasis (hazard ratio 2.92, P = 0.020) and the absence of adjuvant therapy (hazard ratio 3.20, P = 0.014) were independent risk factors for mortality. A total of only seven patients survived more than 1 year and most had a history of neoadjuvant/adjuvant chemotherapy and/or radiation therapy. ConclusionWe believe that the presence of G-CSF-induced leukocytosis represents a distinct and highly aggressive form of upper urinary tract carcinoma. However, the results of our systematic review indicate that a multidisciplinary approach including surgery, neoadjuvant or adjuvant chemotherapy and radiotherapy may have the potential to control the disease, although we cannot provide definitive recommendations from this retrospective study.