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Cancer Treat Res Commun. 2018;17:23-30. doi: 10.1016/j.ctarc.2018.10.003. Epub 2018 Oct 9.

Systematic review and two new cases of primary upper urinary tract neuroendocrine carcinomas.

Author information

1
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: take_nkst@yahoo.co.jp.
2
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: ayahrmt@med.showa-u.ac.jp.
3
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: hyperzector@med.showa-u.ac.jp.
4
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: y-unoki@med.showa-u.ac.jp.
5
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: hshimoy@med.showa-u.ac.jp.
6
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: oshikazu@med.showa-u.ac.jp.
7
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: moritajun@med.showa-u.ac.jp.
8
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: ymaeda@med.showa-u.ac.jp.
9
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: naoemichio@med.showa-u.ac.jp.
10
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: kfuji@med.showa-u.ac.jp.
11
Department of Information Science, Faculty of Arts and Sciences at Fujiyoshida, Showa University, 4562 Kamiyoshida, Fujiyoshida City, Yamanashi 403-0005, Japan. Electronic address: ogawayos@med.showa-u.ac.jp.
12
Department of Pathology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: mayumi-h@med.showa-u.ac.jp.
13
Department of Pathology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: onizuka@med.showa-u.ac.jp.
14
Department of Pathology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: takimoto@med.showa-u.ac.jp.
15
Department of Urology, Showa University, School of Medicine, 1-5-8, Hatanodai, Shinagawa-ku, Tokyo 142-8666, Japan. Electronic address: ogura@cas.showa-u.ac.jp.

Abstract

BACKGROUND:

Upper urinary tract neuroendocrine carcinoma (UUT-NEC) is extremely rare and has a poor prognosis. Although a few cases of successful treatment have been reported, no treatment has shown established efficacy.

PATIENTS AND METHODS:

We analyzed 70 UUT-NEC patients, including 68 with small cell neuroendocrine carcinoma (SCNEC) and large cell neuroendocrine carcinoma (LCNEC) reported between 1985 and 2017 and 2 treated at our hospital.

RESULTS:

Median patient age was 66 years, 58.6% were men, and 60% were of Asian descent. Most UUT-NECs were SCNEC (68; 95.7%), whereas LCNEC was very rare (2; 2.9%). More than half of the patients had accompanying other histological components, the most common being urothelial carcinoma (51.5%), whereas 41.4% had NEC alone. Of the 70 patients, 27 underwent additional therapy (e.g., chemotherapy and radiotherapy) along with surgery. Median survival was 15 months. In univariate analysis, stages T1-2 disease showed better prognosis than stages T3-4 (P < 0.001). Additional treatment (e.g., chemotherapy and radiotherapy) significantly improved prognosis (P = 0.014). Moreover, platinum-based chemotherapy also was associated with improved prognosis (P = 0.017). For platinum-based chemotherapy, multicollinearity with additional treatments was strong, and, thus, these data were not included in the analysis. Multivariate analysis revealed pathological stage (T1-2 vs. T3-4; P = 0.003) and additional treatment (P = 0.028) to be independent predictors of improved prognosis.

CONCLUSION:

Although UUT-NEC has a poor prognosis, additional treatment along with surgery and therapeutic intervention and stage T1-2 disease are independent factors to improve prognosis.

KEYWORDS:

Large cell neuroendocrine carcinoma; Neuroendocrine carcinoma; Renal pelvis; Small cell neuroendocrine carcinoma; Systematic review; Ureter

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