High-grade carcinoma with neuroendocrine differentiation of the urinary bladder: A case report of a rare variant

Shashank Bansal; Prutha Jinwala; Aditya Elhence; SP Shrivastava; Vikas Asati; Rajesh Patidar; Prakash G Chitalkar

doi:10.4103/aort.aort_20_22

CASE REPORT

Year : 2022 | Volume : 2 | Issue : 2 | Page : 104-106

High-grade carcinoma with neuroendocrine differentiation of the urinary bladder: A case report of a rare variant

Shashank Bansal, Prutha Jinwala, Aditya Elhence, SP Shrivastava, Vikas Asati, Rajesh Patidar, Prakash G Chitalkar
Department of Medical Oncology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India

Date of Submission	17-Jul-2022
Date of Decision	05-Aug-2022
Date of Acceptance	09-Aug-2022
Date of Web Publication	18-Nov-2022

Correspondence Address:
Dr. Shashank Bansal
Department of Medical Oncology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh
India

Source of Support: None, Conflict of Interest: None

DOI: 10.4103/aort.aort_20_22

Abstract

Neuroendocrine bladder carcinoma is considered a rare variant (<1%) with highly aggressive potential. Because of the rarity of its presentation, available data are mainly from published case reports. A 40-year-old male presented with painless hematuria for the past 3 months. After evaluation, he underwent radical cystectomy with pelvic lymph node dissection with urinary diversion. Histopathology from the postoperative specimen was suggestive of high-grade carcinoma with neuroendocrine differentiation (CK-focal positive, synaptophysin-focal positive, GATA-3, CD-56 positive and Ki-67 85%–90% chromogranin A-negative) stage pT4bN2M0. The case was discussed in the multidisciplinary tumor board and was planned for adjuvant chemotherapy with cisplatin (75 mg/m²) and etoposide (100 mg/m²) Repeated every 21 days. He completed six cycles of adjuvant chemotherapy and was on follow-up. Within 3 months of completing adjuvant therapy, he presented again with a complaint of hematuria and on evaluation found to have a localized recurrence, following which he received radical chemoradiotherapy and is now on follow-up. Our case provides evidence to think us in new dimension, with chemotherapy in adjuvant and radiation in salvage settings instead of adjuvant settings.

Keywords: Chemoradiotherapy, neuroendocrine carcinoma, urinary bladder cancer

How to cite this article:
Bansal S, Jinwala P, Elhence A, Shrivastava S P, Asati V, Patidar R, Chitalkar PG. High-grade carcinoma with neuroendocrine differentiation of the urinary bladder: A case report of a rare variant. Ann Oncol Res Ther 2022;2:104-6

How to cite this URL:
Bansal S, Jinwala P, Elhence A, Shrivastava S P, Asati V, Patidar R, Chitalkar PG. High-grade carcinoma with neuroendocrine differentiation of the urinary bladder: A case report of a rare variant. Ann Oncol Res Ther [serial online] 2022 [cited 2023 Mar 25];2:104-6. Available from: http://www.aort.info/text.asp?2022/2/2/104/361493

Introduction

Bladder cancer ranks the 10^th among cancers worldwide, and its incidence is steadily rising, especially in developed nations.^[1] It accounts for 1.6% of the total cancer incidence and 1.3% of all cancer deaths in India.^[1] Urothelial cancer, which represents more than 90% of bladder cancer, is mainly found in its pure form. However, urothelial cancer is known to show variant histologic features. Other common nonurothelial epithelial malignancies of the bladder are squamous cell carcinoma and adenocarcinoma. The neuroendocrine bladder carcinoma is considered a rare variant (<1%) with highly aggressive potential. Neuroendocrine carcinoma is subdivided into small-cell carcinoma, large-cell neuroendocrine carcinoma (LCNC), well-differentiated neuroendocrine tumor, and paraganglioma.^[2] Because of the rarity of its presentation, available data are mainly from published case reports. In this article, we present a rare case of neuroendocrine cancer of the urinary bladder.

Case Report

A 40-year-old male with no comorbidity and addiction presented with painless hematuria and difficulty passing urine for the past 3 months. Initially, he underwent transurethral resection of bladder tumor. Histopathological examination showed low-grade urothelial carcinoma with muscle invasion. A preoperative computed tomography (CT) scan showed a mass involving lateral bladder wall with perivesical extension with obscured fat planes with the lateral pelvic wall and enlarged lymph nodes at the internal and external iliac region. He underwent radical cystectomy with pelvic lymph node dissection with urinary diversion. Postoperative histopathological examination and immunohistochemistry showed high-grade carcinoma with neuroendocrine differentiation (CK-focal positive, synaptophysin-focal positive [Figure 1]a, CD-56 positive [Figure 1]b, GATA-3 positive [Figure 1]c, and Ki-67 85-90% chromogranin A-negative) stage pT4bN2M0 (AJCC 8^th Edition). He has received adjuvant chemotherapy with cisplatin (75 mg/m²) and etoposide (100 mg/m²) Repeated every 21 days. He completed six cycles of adjuvant chemotherapy and was kept on follow-up. After a period of 3 months, he presented with complaints of hematuria, and on evaluation, localized measureable disease was present on CT scan, after which he was planned for chemoradiotherapy (60 Gy in 30 fractions and 2 Gy per fraction with 5 fractions per week for 6 weeks along with concurrent cisplatin 35 mg/m²). He tolerated the treatment well and is on follow-up; the total follow-up period since diagnosis is 15 months till now.

Figure 1: (a) Immunohistochemistry positive for synaptophysin (b) CD56 (c) GATA3

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Discussion

Neuroendocrine tumors in the urinary bladder are rare, and most publications are case reports. Clinically, the most common complaint at presentation is painless hematuria and obstructive urinary symptoms.^[3],[4] Tumors are usually aggressive and present in the older age, and smoking is the most commonly associated risk factor.^[5] Our patient is nonsmoker adult. The case reported by Akdeniz et al. also had younger, nonsmoker patient.^[6] Due to the paucity of a randomized trials, the appropriate management strategy is yet to be formulated. Hata and Tasaki treated patients with early LCNC of the urinary bladder using transurethral resection alone and observed no recurrence at the 8^th postoperative month.^[7] Dowd et al. applied transurethral resection + radiotherapy + chemotherapy to patients with poorly differentiated neuroendocrine tumors and observed no recurrence in the 1^st year postoperatively.^[8] Colarossi et al. treated LCNC patients using neoadjuvant chemotherapy, followed by cystectomy + hysterectomy + lymphadenectomy followed by adjuvant chemotherapy and reported that patients died in the 7^th month postoperatively.^[9] In our case, R0 resection of the bladder tumor was done and received 6 cycles of adjuvant cisplatin and etoposide; the disease relapsed at 3 month following chemotherapy and received radical chemoradiotherapy. He is now asymptomatic and is on follow-up, 15 months from the diagnosis. Martín et al. in a bibliographic review showed that about half of all patients were alive after a follow-up of 20 months.^[10] In our case, the disease progressed after a period of 8 months, following which he has received radical chemoradiotherapy. Integrating radiotherapy up front in this disease with a propensity of locoregional recurrences may prove beneficial.

Conclusion

In absence of an adequate evidence base, large-cell neuroendocrine tumors of the bladder will be treated with a variety of regimes; adjuvant concurrent chemoradiation/radiotherapy as a first-line therapy has achieved a median survival of 20 months. Radiotherapy could be reserved for a progression, as in our case.

Declaration of patient consent

The authors certify that they have obtained all appropriate patient consent forms. In the form, the patient has given his consent for his images and other clinical information to be reported in the journal. The patient understands that his name and initials will not be published and due efforts will be made to conceal identity, but anonymity cannot be guaranteed.

Financial support and sponsorship

Nil.

Conflicts of interest

There are no conflicts of interest.

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