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Rare neuroendocrine tumor decades after bladder exstrophy repair with enterocystoplasty
Journal of Pediatric Surgery Case Reports 31 (2018) 10–12
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Rare neuroendocrine tumor decades after bladder exstrophy repair with enterocystoplasty
T
Sabine Rouxa,∗, Marie-Laurence Poli-Merola,b, Nicolas Merzeaua, Stéphane Larréa, Priscilla Leona a b
Department of Urology, Robert Debre University Hospital, Reims, France Department of Pediatric Surgery, American Memorial Hospital, Reims, France
A R T I C L E I N F O
A B S T R A C T
Keywords: Bladder exstrophy Neuroendocrine carcinoma Urinary bladder neoplasms
Bladder exstrophy is associated with an increased risk of bladder cancer. Adenocarcinoma is the main histological type found in the bladder exstrophy, accounting for 95% of cases, whereas squamous cell carcinoma accounts for 3–5% cases. We present here the rare case of the development of a metastatic neuroendocrine tumor on a bladder exstrophy, 26 years after enterocystoplasty enlargement. We describe the presentation of the cancer and the results after treatment by surgery and chemotherapy.
1. Introduction Bladder exstrophy is a rare congenital anomaly and it exists 1% of carcinological risk due to the presence of digestive mucosa in the bladder wall [1]. We report the extremely rare case of a 34-year-old woman, multi-operated for bladder exstrophy, who developed a rare kind of neuroendocrine tumor 26 years after an enterocystoplasty enlargement. 2. Case report A 34-year-old patient was admitted in our urology department after the discovery of a neuroendocrine bladder tumor. She had a history of complete bladder exstrophy associated to vulva and vagina bifidity. The bladder exstrophy was closed four months after birth. At the age of 7 an enterocystoplasty enlargement with a sigmoidian patch was carried out. When she was 30 morbid obesity was managed with gastric band. At 34 years old, she presented a right obstructive pyelonephritis and acute renal failure. A scan showed then a vesical tumor on the right bladder side face (Fig. 1). In February 2016, a transurethral resection of the bladder tumor revealed a neuroendocrine carcinoma infiltrating the muscle with small aggressive cells, and a proliferation index Ki67 between 90 and 100% (AE1/AE3+, chromogranine+, synatophysine+). The stadification imaging with a FDG scintigraphy found a hypermetabolic state of the right vesicoureteral tumor and multiple adenopathy (Fig. 2). A chemotherapy associating Etoposide and Cisplatin was started and an evaluation scintigraphy was performed after 3 cycles, showing a decreased hyper metabolism on the hepatic right lesion and a complete regression of the left iliac wing hyper metabolism. ∗
There was a regression in number and size of the latero vesical hyper metabolism. A regression of the postero-inferior mediastinal, lomboaortic and pelvic nodes was also found; meanwhile several suspicious hypermetabolic bone areas appeared. After 6 cycles of chemotherapy associating Cisplatin (180 mg) and Etoposide (190 mg) performed from February to June 2016 the scintigraphy showed a dissociated answer. An abdominal-pelvic scan confirmed the presence of lomboaortic nodes, a 13 mm hepatic lesion and a 17 mm thickening of the right bladder wall. In August 2016, a closing surgery was decided consisting in a total neocystectomy and lombo-aortic nodes dissection. An injury of the right extern iliac artery during the complicated dissection led to an arterial bypass with prosthesis. Thus, a Bricker surgery was contraindicated and a total cystectomy with bilateral ureterostomy was performed. Histology revealed an infiltration of the bladder wall including the right lateral side, the dome and the back, with an endocrine carcinoma with small cells (Ki67 at 70%, TTF1+), that extended to the muscle and N+. 3 months after surgery, the patient received a MVAC chemotherapy (30mg/m2 of Methotrexate, 3mg/m2 of Vinblastine, 30mg/m2 of Doxorubicin and 70 mg/m2 of Cisplatin). In February 2017, after her 4th cycle of MVAC, a FDG scintigraphy showed a partial answer with a decrease of most of the secondary lesions and the apparition of a hypermetabolic signal at the extremity of the right jugular PICC Line. In all she received 6 cycles of MVAC, the last one was in March 2017. The patient was treated after with Endoxan. She died in September 2017. 3. Discussion Bladder exstrophy is associated with an increased risk of bladder
Corresponding author. Department of Urology, Robert Debre University Hospital, 51, rue du Général Köenig, 51100, Reims, France. E-mail address: [email protected] (S. Roux).
https://doi.org/10.1016/j.epsc.2017.11.018 Received 19 November 2017; Accepted 21 November 2017 Available online 24 November 2017 2213-5766/ © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
cancer. Adenocarcinoma is the main histological type found in bladder exstrophy, accounting for 95% of cases, whereas squamous cell carcinoma accounts for 3–5% cases [2]. N. Smeulders et al. studied the incidence of cancers in patients suffering from bladder exstrophy through a cohort of 103 patients. Patients were divided in 2 groups: high or low risk to develop a tumor depending on the type of surgery. 42 patients were in the high-risk group with urinary and faecal stream mixing surgery. In the low-risk group 61 patients had an ileal or a colic conduit after a bladder closing surgery or ureterostomy. There was also an incidence of 17, 5% of cancer (18/103), 38% (16/42) in the high-risk group and 3,3% (2/61) in the low-risk group. Authors also suggested a potential intrinsic predisposition to a malignant transformation. Patients with bladder exstrophy have 700-fold incidence of bladder cancer than the general population at the same age. The early cystectomy does not seem to be a protective factor [3]. The urinary diversions, which mix urinary and faecal stream, have a carcinogenic potential established in the literature [4]. There are no guidelines regarding these patients' follow up. Austen et al. [5] propose an annual endoscopy from the 3rd postoperative year. The last endoscopic evaluation of the present case was approximatively 4 years before presentation. Indeed, the patient's tumor could have been diagnosed earlier with more regular endoscopy. Surgery probably avoided pelvic complications for our patient, but other treatments, such as chemotherapy or radiotherapy
11
B.P. Kerfoot et al. [6] Current case
Bladder exstrophy non operated
Di Lauro G et al. [10] J.S. Ko et al. [4]
- Bilateral ureterosigmoidostomy on bladder exstrophy before the age of 10, - Ileal conduit after sigmoidostomy for benign polype at 18 Ileal diversion and cystectomy for bladder adenocarcinoma Enterocystoplasty Enlargement with a sigmoidian patch at 7 years old
Bladder exstrophy non operated
Neuroendocrine carcinoma
Undifferentiated high-grade adenocarcinoma Ileon's adenocarcinoma of 6 cm, extented to the left ureter, the colon and to the left iliac artery Carcinoid tumor
Papillary adenocarcinoma
55-year-old man
42-year-old man
Tumoral resection including the left kidney, the ureter, the ileal conduct, the sigmoid, then coloproctostomy and enterostomy Ileal segmentectomy and ileal conduit reconstruction Cystectomy with bilateral ureterostomy, 6 cycles of MVAC chemotherapy, Endoxan
Patient won't accept treatment
59-year-old man (41 years after ileal conduit and 1 year after the last unremarkable colonoscopy) 61-year-old woman (9 years after diversion) 34-year-old woman (26 years after diversion)
Squamous cell carcinoma at a site of remnant bladder plate
Cystectomy and ureterosigmoidostomy in the childhood on bladder exstrophy-epispadias complex
S. Shwetha et al. [9]
34-year-old man
Squamous cell carcinoma
Radical cystectomy
Penectomy with radical prostatectomy, bilateral pelvic and inguinal lymph nodes dissection Chemotherapy: 4 cycles of Gemcitabine (1,4g) et Cisplatin (70 mg) After no response: 2 cycles of Docetaxel (120 mg) Surgery
36-year-old woman (17 years after surgery)
Squamous cell carcinoma on the exposed bladder mucosa
Repair of the exstrophy defect at 19, then 6 months after ileal conduit to prevent leakage of urine from the lower abdominal wall Bladder exstrophy non operated
Pramod Kumar Sharma et al. [2] Sachin Patil et al. [7] Lane GI et al. [8]
Treatment
Fig. 1. Frontal section of CT abdomen in January 2016 showing a vesical tumor on the right bladder side face extended to the ureteral meatus and pelvic ureter compression with iliac pelvic, interaorticocaval adenopathy and nodular lesion of the hepatic unit IV.
78-year-old man (73 years after surgery)
Age at cancer diagnosis
Histological type
Type of Diversion
Fig. 2. FDG scintigraphy in February 2016 showing an hypermetabolic state of the right vesicoureteral tumor, a lombo-aortic, pelvic, left subclavicular, posteroinferior mediastinal lymph node involvement and an hypermetabolic area of the right liver and of the left iliac wing.
Study
Table 1 Types of tumors after urinary diversion.
Dead in September 2017 (20 months after cancer diagnosis)
Still alive in February 2015, but with an increase of the growth No recurrence at 6-month follow-up No recurrence at 2- month follow-up
Free of disease 41 months after resection
No follow-up
Still alive 5 months after surgery
Survival after cancer diagnosis
S. Roux et al.
Journal of Pediatric Surgery Case Reports 31 (2018) 10–12
Journal of Pediatric Surgery Case Reports 31 (2018) 10–12
S. Roux et al.
agencies in the public, commercial, or not-for-profit sectors.
could be discussed, since Bricker was unmanageable. Carcinoid tumors are not common after bladder exstrophy. B.P. Kerfoot et al. [6] explained the case of a carcinoid tumor developed on an ileal derivation after radical cystectomy for a bladder adenocarcinoma in a 61-year-old woman. These tumors are rare. Besides, the authors described the case of a 55-year-old woman who presented a carcinoid tumor after urinary derivation and post pelvic radiotherapy for cervical cancer. Carcinoid tumors represent 19% of digestive tumors and 90% of the small tumors from the ileon [6]. Carcinogenesis is a multifactorial phenomenon where the urine contact with the different diversions promotes the tumor development. Bladder tumors development in patients with bladder exstrophy is frequent, especially after urinary diversion. However, our patient's case, which developed a neuroendocrine tumor on an ectopic bladder after enterocystoplasty enlargement, is extremely rare. We do not have long term data concerning enterocystoplasty benefit on bladder exstrophy. However, closure of the exstrophy bladder at birth should be discussed to protect patients. The various histological types of bladder tumors developing on bladder exstrophy could explain the absence of guidelines to manage these patients (Table 1).
Conflicts of interest The authors have nothing to disclose. Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx. doi.org/10.1016/j.epsc.2017.11.018. References [1] Mouriquand P, Vidal I. Complexe extsrophie épispade (CEE). EMC - Urol 2012;5(1):1–18. [Article 18-208-A-10]. [2] Sharma PK, Pandey PK, Vijay MK, Bera MK, Singh JP, Saha K. Squamous cell carcinoma in exstrophy of the bladder. Korean J Urol 2013;54:555–7. [3] Smeulders N, Woodhouse CR. Neoplasia in adult exstrophy patients. BJU Int 2001 May;87:623–8. [4] Ko JS, Di Carlo HN, Gupta AD, Ross AE, Eckhauser FE, Bivalacqua TJ. Adenocarcinoma of the ileal conduit in a patient born with classic bladder exstrophy. Urol Case Rep 2013;1:5–6. [5] Austen M, Kälble T. Secondary malignancies in different forms of urinary diversion using isolated gut. J Urol 2004;172:831–8. [6] Kerfoot BP, Steele GS, Datta MW, Richie JP. Carcinoid tumor in an ileal conduit diversion. J Urol 1999;162:1685. [7] Patil S, Jain SK, Kaza R, Rao S. Squamous cell carcinoma in bladder exstrophy: a rare entity. Singap Med J 2012;53:254–7. [8] Lane GI, Kwaan M, Lewis J. Pediatr Surg Int 2015;31:1107. [9] Shwetha S, Ghalige HS, Goyal L, Jain P, Fakhruddin. J Clin Diagnostic Res Sept 2015;9(9):XD04–5. [10] Di Lauro G, Iacono F, Ruffo A, Romis L, Mordente S, Pane U, et al. Presenting a case of a mucinous adenocarcinoma of an exstrophic bladder in an adult patient and a review of literature. BMC Surg 2013;13(2):S36.
4. Conclusion There is a need to find out which surgery at birth could be protective and maintain these patients' quality of life. A close follow-up seems necessary for patients with bladder exstrophy history to treat them early, though the frequency of endoscopies and the scheduling of the first one are unclear in the literature. Funding This research did not receive any specific grant from funding
12
Contents lists available at ScienceDirect
Journal of Pediatric Surgery Case Reports journal homepage: www.elsevier.com/locate/epsc
Rare neuroendocrine tumor decades after bladder exstrophy repair with enterocystoplasty
T
Sabine Rouxa,∗, Marie-Laurence Poli-Merola,b, Nicolas Merzeaua, Stéphane Larréa, Priscilla Leona a b
Department of Urology, Robert Debre University Hospital, Reims, France Department of Pediatric Surgery, American Memorial Hospital, Reims, France
A R T I C L E I N F O
A B S T R A C T
Keywords: Bladder exstrophy Neuroendocrine carcinoma Urinary bladder neoplasms
Bladder exstrophy is associated with an increased risk of bladder cancer. Adenocarcinoma is the main histological type found in the bladder exstrophy, accounting for 95% of cases, whereas squamous cell carcinoma accounts for 3–5% cases. We present here the rare case of the development of a metastatic neuroendocrine tumor on a bladder exstrophy, 26 years after enterocystoplasty enlargement. We describe the presentation of the cancer and the results after treatment by surgery and chemotherapy.
1. Introduction Bladder exstrophy is a rare congenital anomaly and it exists 1% of carcinological risk due to the presence of digestive mucosa in the bladder wall [1]. We report the extremely rare case of a 34-year-old woman, multi-operated for bladder exstrophy, who developed a rare kind of neuroendocrine tumor 26 years after an enterocystoplasty enlargement. 2. Case report A 34-year-old patient was admitted in our urology department after the discovery of a neuroendocrine bladder tumor. She had a history of complete bladder exstrophy associated to vulva and vagina bifidity. The bladder exstrophy was closed four months after birth. At the age of 7 an enterocystoplasty enlargement with a sigmoidian patch was carried out. When she was 30 morbid obesity was managed with gastric band. At 34 years old, she presented a right obstructive pyelonephritis and acute renal failure. A scan showed then a vesical tumor on the right bladder side face (Fig. 1). In February 2016, a transurethral resection of the bladder tumor revealed a neuroendocrine carcinoma infiltrating the muscle with small aggressive cells, and a proliferation index Ki67 between 90 and 100% (AE1/AE3+, chromogranine+, synatophysine+). The stadification imaging with a FDG scintigraphy found a hypermetabolic state of the right vesicoureteral tumor and multiple adenopathy (Fig. 2). A chemotherapy associating Etoposide and Cisplatin was started and an evaluation scintigraphy was performed after 3 cycles, showing a decreased hyper metabolism on the hepatic right lesion and a complete regression of the left iliac wing hyper metabolism. ∗
There was a regression in number and size of the latero vesical hyper metabolism. A regression of the postero-inferior mediastinal, lomboaortic and pelvic nodes was also found; meanwhile several suspicious hypermetabolic bone areas appeared. After 6 cycles of chemotherapy associating Cisplatin (180 mg) and Etoposide (190 mg) performed from February to June 2016 the scintigraphy showed a dissociated answer. An abdominal-pelvic scan confirmed the presence of lomboaortic nodes, a 13 mm hepatic lesion and a 17 mm thickening of the right bladder wall. In August 2016, a closing surgery was decided consisting in a total neocystectomy and lombo-aortic nodes dissection. An injury of the right extern iliac artery during the complicated dissection led to an arterial bypass with prosthesis. Thus, a Bricker surgery was contraindicated and a total cystectomy with bilateral ureterostomy was performed. Histology revealed an infiltration of the bladder wall including the right lateral side, the dome and the back, with an endocrine carcinoma with small cells (Ki67 at 70%, TTF1+), that extended to the muscle and N+. 3 months after surgery, the patient received a MVAC chemotherapy (30mg/m2 of Methotrexate, 3mg/m2 of Vinblastine, 30mg/m2 of Doxorubicin and 70 mg/m2 of Cisplatin). In February 2017, after her 4th cycle of MVAC, a FDG scintigraphy showed a partial answer with a decrease of most of the secondary lesions and the apparition of a hypermetabolic signal at the extremity of the right jugular PICC Line. In all she received 6 cycles of MVAC, the last one was in March 2017. The patient was treated after with Endoxan. She died in September 2017. 3. Discussion Bladder exstrophy is associated with an increased risk of bladder
Corresponding author. Department of Urology, Robert Debre University Hospital, 51, rue du Général Köenig, 51100, Reims, France. E-mail address: [email protected] (S. Roux).
https://doi.org/10.1016/j.epsc.2017.11.018 Received 19 November 2017; Accepted 21 November 2017 Available online 24 November 2017 2213-5766/ © 2017 The Authors. Published by Elsevier Inc. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/BY-NC-ND/4.0/).
cancer. Adenocarcinoma is the main histological type found in bladder exstrophy, accounting for 95% of cases, whereas squamous cell carcinoma accounts for 3–5% cases [2]. N. Smeulders et al. studied the incidence of cancers in patients suffering from bladder exstrophy through a cohort of 103 patients. Patients were divided in 2 groups: high or low risk to develop a tumor depending on the type of surgery. 42 patients were in the high-risk group with urinary and faecal stream mixing surgery. In the low-risk group 61 patients had an ileal or a colic conduit after a bladder closing surgery or ureterostomy. There was also an incidence of 17, 5% of cancer (18/103), 38% (16/42) in the high-risk group and 3,3% (2/61) in the low-risk group. Authors also suggested a potential intrinsic predisposition to a malignant transformation. Patients with bladder exstrophy have 700-fold incidence of bladder cancer than the general population at the same age. The early cystectomy does not seem to be a protective factor [3]. The urinary diversions, which mix urinary and faecal stream, have a carcinogenic potential established in the literature [4]. There are no guidelines regarding these patients' follow up. Austen et al. [5] propose an annual endoscopy from the 3rd postoperative year. The last endoscopic evaluation of the present case was approximatively 4 years before presentation. Indeed, the patient's tumor could have been diagnosed earlier with more regular endoscopy. Surgery probably avoided pelvic complications for our patient, but other treatments, such as chemotherapy or radiotherapy
11
B.P. Kerfoot et al. [6] Current case
Bladder exstrophy non operated
Di Lauro G et al. [10] J.S. Ko et al. [4]
- Bilateral ureterosigmoidostomy on bladder exstrophy before the age of 10, - Ileal conduit after sigmoidostomy for benign polype at 18 Ileal diversion and cystectomy for bladder adenocarcinoma Enterocystoplasty Enlargement with a sigmoidian patch at 7 years old
Bladder exstrophy non operated
Neuroendocrine carcinoma
Undifferentiated high-grade adenocarcinoma Ileon's adenocarcinoma of 6 cm, extented to the left ureter, the colon and to the left iliac artery Carcinoid tumor
Papillary adenocarcinoma
55-year-old man
42-year-old man
Tumoral resection including the left kidney, the ureter, the ileal conduct, the sigmoid, then coloproctostomy and enterostomy Ileal segmentectomy and ileal conduit reconstruction Cystectomy with bilateral ureterostomy, 6 cycles of MVAC chemotherapy, Endoxan
Patient won't accept treatment
59-year-old man (41 years after ileal conduit and 1 year after the last unremarkable colonoscopy) 61-year-old woman (9 years after diversion) 34-year-old woman (26 years after diversion)
Squamous cell carcinoma at a site of remnant bladder plate
Cystectomy and ureterosigmoidostomy in the childhood on bladder exstrophy-epispadias complex
S. Shwetha et al. [9]
34-year-old man
Squamous cell carcinoma
Radical cystectomy
Penectomy with radical prostatectomy, bilateral pelvic and inguinal lymph nodes dissection Chemotherapy: 4 cycles of Gemcitabine (1,4g) et Cisplatin (70 mg) After no response: 2 cycles of Docetaxel (120 mg) Surgery
36-year-old woman (17 years after surgery)
Squamous cell carcinoma on the exposed bladder mucosa
Repair of the exstrophy defect at 19, then 6 months after ileal conduit to prevent leakage of urine from the lower abdominal wall Bladder exstrophy non operated
Pramod Kumar Sharma et al. [2] Sachin Patil et al. [7] Lane GI et al. [8]
Treatment
Fig. 1. Frontal section of CT abdomen in January 2016 showing a vesical tumor on the right bladder side face extended to the ureteral meatus and pelvic ureter compression with iliac pelvic, interaorticocaval adenopathy and nodular lesion of the hepatic unit IV.
78-year-old man (73 years after surgery)
Age at cancer diagnosis
Histological type
Type of Diversion
Fig. 2. FDG scintigraphy in February 2016 showing an hypermetabolic state of the right vesicoureteral tumor, a lombo-aortic, pelvic, left subclavicular, posteroinferior mediastinal lymph node involvement and an hypermetabolic area of the right liver and of the left iliac wing.
Study
Table 1 Types of tumors after urinary diversion.
Dead in September 2017 (20 months after cancer diagnosis)
Still alive in February 2015, but with an increase of the growth No recurrence at 6-month follow-up No recurrence at 2- month follow-up
Free of disease 41 months after resection
No follow-up
Still alive 5 months after surgery
Survival after cancer diagnosis
S. Roux et al.
Journal of Pediatric Surgery Case Reports 31 (2018) 10–12
Journal of Pediatric Surgery Case Reports 31 (2018) 10–12
S. Roux et al.
agencies in the public, commercial, or not-for-profit sectors.
could be discussed, since Bricker was unmanageable. Carcinoid tumors are not common after bladder exstrophy. B.P. Kerfoot et al. [6] explained the case of a carcinoid tumor developed on an ileal derivation after radical cystectomy for a bladder adenocarcinoma in a 61-year-old woman. These tumors are rare. Besides, the authors described the case of a 55-year-old woman who presented a carcinoid tumor after urinary derivation and post pelvic radiotherapy for cervical cancer. Carcinoid tumors represent 19% of digestive tumors and 90% of the small tumors from the ileon [6]. Carcinogenesis is a multifactorial phenomenon where the urine contact with the different diversions promotes the tumor development. Bladder tumors development in patients with bladder exstrophy is frequent, especially after urinary diversion. However, our patient's case, which developed a neuroendocrine tumor on an ectopic bladder after enterocystoplasty enlargement, is extremely rare. We do not have long term data concerning enterocystoplasty benefit on bladder exstrophy. However, closure of the exstrophy bladder at birth should be discussed to protect patients. The various histological types of bladder tumors developing on bladder exstrophy could explain the absence of guidelines to manage these patients (Table 1).
Conflicts of interest The authors have nothing to disclose. Appendix A. Supplementary data Supplementary data related to this article can be found at http://dx. doi.org/10.1016/j.epsc.2017.11.018. References [1] Mouriquand P, Vidal I. Complexe extsrophie épispade (CEE). EMC - Urol 2012;5(1):1–18. [Article 18-208-A-10]. [2] Sharma PK, Pandey PK, Vijay MK, Bera MK, Singh JP, Saha K. Squamous cell carcinoma in exstrophy of the bladder. Korean J Urol 2013;54:555–7. [3] Smeulders N, Woodhouse CR. Neoplasia in adult exstrophy patients. BJU Int 2001 May;87:623–8. [4] Ko JS, Di Carlo HN, Gupta AD, Ross AE, Eckhauser FE, Bivalacqua TJ. Adenocarcinoma of the ileal conduit in a patient born with classic bladder exstrophy. Urol Case Rep 2013;1:5–6. [5] Austen M, Kälble T. Secondary malignancies in different forms of urinary diversion using isolated gut. J Urol 2004;172:831–8. [6] Kerfoot BP, Steele GS, Datta MW, Richie JP. Carcinoid tumor in an ileal conduit diversion. J Urol 1999;162:1685. [7] Patil S, Jain SK, Kaza R, Rao S. Squamous cell carcinoma in bladder exstrophy: a rare entity. Singap Med J 2012;53:254–7. [8] Lane GI, Kwaan M, Lewis J. Pediatr Surg Int 2015;31:1107. [9] Shwetha S, Ghalige HS, Goyal L, Jain P, Fakhruddin. J Clin Diagnostic Res Sept 2015;9(9):XD04–5. [10] Di Lauro G, Iacono F, Ruffo A, Romis L, Mordente S, Pane U, et al. Presenting a case of a mucinous adenocarcinoma of an exstrophic bladder in an adult patient and a review of literature. BMC Surg 2013;13(2):S36.
4. Conclusion There is a need to find out which surgery at birth could be protective and maintain these patients' quality of life. A close follow-up seems necessary for patients with bladder exstrophy history to treat them early, though the frequency of endoscopies and the scheduling of the first one are unclear in the literature. Funding This research did not receive any specific grant from funding
12