• Users Online: 58
  • Print this page
  • Email this page


 
 Table of Contents  
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


Department of Medical Oncology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh, India

Date of Submission17-Jul-2022
Date of Decision05-Aug-2022
Date of Acceptance09-Aug-2022
Date of Web Publication18-Nov-2022

Correspondence Address:
Dr. Shashank Bansal
Department of Medical Oncology, Sri Aurobindo Institute of Medical Sciences, Indore, Madhya Pradesh
India
Login to access the Email id

Source of Support: None, Conflict of Interest: None


DOI: 10.4103/aort.aort_20_22

Rights and Permissions
  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 2022 Nov 29];2:104-6. Available from: http://www.aort.com/text.asp?2022/2/2/104/361493


  Introduction Top


Bladder cancer ranks the 10th 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 Top


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 8th 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

Click here to view



  Discussion Top


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 8th postoperative month.[7] Dowd et al. applied transurethral resection + radiotherapy + chemotherapy to patients with poorly differentiated neuroendocrine tumors and observed no recurrence in the 1st 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 7th 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 Top


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.



 
  References Top

1.
Cancer Today. Available from: http://gco.iarc.fr/today/home. [Last accessed on 2022 Jun 17].  Back to cited text no. 1
    
2.
Humphrey PA, Moch H, Cubilla AL, Ulbright TM, Reuter VE. The 2016 WHO classification of tumours of the urinary system and male genital organs-part B: Prostate and bladder tumours. Eur Urol 2016;70:106-19.  Back to cited text no. 2
    
3.
Pasquier D, Barney B, Sundar S, Poortmans P, Villa S, Nasrallah H, et al. Small cell carcinoma of the urinary bladder: A retrospective, multicenter rare cancer network study of 107 patients. Int J Radiat Oncol Biol Phys 2015;92:904-10.  Back to cited text no. 3
    
4.
Pusıol T, Zorzi MG, Morichetti D, Sari A. “Pure” primary large cell neuroendocrine carcinoma of the urinary bladder. Turk Patoloji Derg 2013;29:241-5.  Back to cited text no. 4
    
5.
Bhatt VR, Loberiza FR Jr., Tandra P, Krishnamurthy J, Shrestha R, Wang J. Risk factors, therapy and survival outcomes of small cell and large cell neuroendocrine carcinoma of urinary bladder. Rare Tumors 2014;6:5043.  Back to cited text no. 5
    
6.
Akdeniz E, Bakirtas M, Bolat MS, Akdeniz S, Özer I. Pure large cell neuroendocrine carcinoma of the bladder without urological symptoms. Pan Afr Med J 2018;30:134.  Back to cited text no. 6
    
7.
Hata S, Tasaki Y. A case of the large cell neuroendocrine carcinoma of the urinary bladder. Case Rep Med 2013;2013:804136.  Back to cited text no. 7
    
8.
Dowd K, Rotenberry C, Russell D, Wachtel M, de Riese W. Rare occurrence of a poorly differentiated neuroendocrine tumor of the bladder. Case Rep Med 2017;2017:1-4.  Back to cited text no. 8
    
9.
Colarossi C, Pino P, Giuffrida D, Aiello E, Costanzo R, Martinetti D, et al. Large cell neuroendocrine carcinoma (LCNEC) of the urinary bladder: A case report. Diagn Pathol 2013;8:19.  Back to cited text no. 9
    
10.
Martín IJ, Vilar DG, Aguado JM, Perelló CG, Aliaga MR, Argente VG, et al. Large cell neuroendocrine carcinoma of the urinary bladder. Bibliographic review. Arch Esp Urol 2011;64:105-13.  Back to cited text no. 10
    


    Figures

  [Figure 1]



 

Top
 
 
  Search
 
Similar in PUBMED
   Search Pubmed for
   Search in Google Scholar for
 Related articles
Access Statistics
Email Alert *
Add to My List *
* Registration required (free)

 
  In this article
Abstract
Introduction
Case Report
Discussion
Conclusion
References
Article Figures

 Article Access Statistics
    Viewed38    
    Printed2    
    Emailed0    
    PDF Downloaded5    
    Comments [Add]    

Recommend this journal


[TAG2]
[TAG3]
[TAG4]