neuroendocrine tumor of bladder - Journal of University Medical
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54 JUMDC Vol. 3, Issue 1, Jan-Jun 2012 Case Report NEUROENDOCRINE TUMOR OF BLADDER Muhammad Akram Malik 1 , Sadia Hameed 2 , Nayab Alia 3 , Muhammad Sohail 4 , Iftikhar Ahmed 5 1 Associate Professor, Deptt. of Urology, Univ. of Medical & Dental College, Faisalabad. 2 Associate Professor, Deptt. of Pathology, Univ. of Medical & Dental College, Faisalabad. 3 Asstt. Professor, Deptt. of Radiology, Univ. of Medical & Dental College, Faisalabad. 4 Registrar, Deptt. of Urology, Univ. of Medical & Dental College, Faisalabad. 5Assistant Professor, Quaid-e-Azam Medical College, Bahawalpur. ABSTRACT Neuroendocrine tumor is a rare and aggressive tumor of urinary bladder with poor prognosis. It often presents late and the commonest presentation is haematuria. Cystectomy, partial cystectomy, TURBT with adjuvent radio/chemotherapy, radiotherapy or chemotherapy alone are various treatment options. We present a case of primary neuroendocrine tumor of urinary bladder in a 57 years old lady presented with haematuria. TURBT was performed and patient was reffered to oncology department for further management as she was not willing for cystectomy. Keyword: Bladder tumor, Neuroendocrine tumors, Small cell carcinoma INTRODUCTION Most common (90%) tumors of urinary bladder are transitional cell carcinoma. Adenocarcinoma and squmous cell carcinoma are less common. Primary neuroendocrine tumor of urinary bladder is a rare (<1%) however very aggressive malignancy having poor prognosis. It usually presents late as compared to other urothelial malignancies of the bladder. Haematuria is the most common presentation although it may present with metastasis of liver, lungs, lymph nodes etc. Neuroendocrine carcinoma is classified histologically into small cell carcinoma (SCC) and large cell carcinoma which is extremely rare in the urinary bladder. SCC of urinary bladder comprises of 0.5% to 1.0% primary bladder carcinomas which commonly presents in the seventh decade with a male to female ratio of 3:1. 1,2 A number of treatments options are being employed to deal with this rare malignancy. Transurethral resection (TURBT), with adjuvent and neo-adjuvent chemotherapy and radiotherapy, cystectomy, partial cystectomy, radiotherapy and chemotherapy alone are various modalities of treatment with poor prognosis. 3 We present a case of neoroendocrine carcinoma in a 57 years old female at Madina Teaching Hospital/University Medical & Dental College Faisalabad, Pakistan. CASE REPORT A 57 years old female was admitted through out patient department with history of painless gross haematuria for 60 days. Ultrasound (USG) of abdomen and pelvis showed irregular 7 cm×4 cm mass on right lateral wall and base of bladder (Fig I). Computerized Tomography (CT) of abdomen and pelvis showed 7 cm×4 cm irregular mass not extending beyond bladder wall (Fig. II). There was no lymphadenopathy and no metastasis in liver or lungs. Transurethral resection of tumor was performed under spinal anesthesia. Histopathology of tissue proved to be neuroendocrine tumor with invasion into detrusor muscle (Figs. III, IV). Tumor stage was T2 N0 M0. Post operative Magnetic Resonance Imaging (MRI) showed clearance of tumor with no evidence of metastasis (Fig. V). Patient was not willing for cystectomy so she was referred to oncologist for adjuvent radio/chemotherapy. Corresponding Author: Dr. Muhammad Akram Malik, Urology Department, UMDC, Faisalabad. Email: [email protected]
Transcript of neuroendocrine tumor of bladder - Journal of University Medical
54 JUMDC Vol. 3, Issue 1, Jan-Jun 2012
Case Report
NEUROENDOCRINE TUMOR OF BLADDER
Muhammad Akram Malik1, Sadia Hameed2, Nayab Alia3, Muhammad Sohail4, Iftikhar Ahmed5 1Associate Professor, Deptt. of Urology, Univ. of Medical & Dental College, Faisalabad. 2Associate Professor, Deptt. of Pathology, Univ. of Medical & Dental College, Faisalabad. 3Asstt. Professor, Deptt. of Radiology, Univ. of Medical & Dental College, Faisalabad. 4Registrar, Deptt. of Urology, Univ. of Medical & Dental College, Faisalabad. 5Assistant Professor, Quaid-e-Azam Medical College, Bahawalpur.
ABSTRACT
Neuroendocrine tumor is a rare and aggressive tumor of urinary bladder with poor prognosis. It often presents late and the commonest presentation is haematuria. Cystectomy, partial cystectomy, TURBT with adjuvent radio/chemotherapy, radiotherapy or chemotherapy alone are various treatment options. We present a case of primary neuroendocrine tumor of urinary bladder in a 57 years old lady presented with haematuria. TURBT was performed and patient was reffered to oncology department for further management as she was not willing for cystectomy.
Keyword: Bladder tumor, Neuroendocrine tumors, Small cell carcinoma
INTRODUCTION Most common (90%) tumors of urinary bladder are transitional cell carcinoma. Adenocarcinoma and squmous cell carcinoma are less common. Primary neuroendocrine tumor of urinary bladder is a rare (<1%) however very aggressive malignancy having poor prognosis. It usually presents late as compared to other urothelial malignancies of the bladder. Haematuria is the most common presentation although it may present with metastasis of liver, lungs, lymph nodes etc. Neuroendocrine carcinoma is classified histologically into small cell carcinoma (SCC) and large cell carcinoma which is extremely rare in the urinary bladder. SCC of urinary bladder comprises of 0.5% to 1.0% primary bladder carcinomas which commonly presents in the seventh decade with a male to female ratio of 3:1.1,2
A number of treatments options are being employed to deal with this rare malignancy. Transurethral resection (TURBT), with adjuvent and neo-adjuvent chemotherapy and radiotherapy, cystectomy, partial cystectomy,
radiotherapy and chemotherapy alone are various modalities of treatment with poor prognosis.3 We present a case of neoroendocrine carcinoma in a 57 years old female at Madina Teaching Hospital/University Medical & Dental College Faisalabad, Pakistan. CASE REPORT
A 57 years old female was admitted through out patient department with history of painless gross haematuria for 60 days. Ultrasound (USG) of abdomen and pelvis showed irregular 7 cm×4 cm mass on right lateral wall and base of bladder (Fig I). Computerized Tomography (CT) of abdomen and pelvis showed 7 cm×4 cm irregular mass not extending beyond bladder wall (Fig. II). There was no lymphadenopathy and no metastasis in liver or lungs. Transurethral resection of tumor was performed under spinal anesthesia. Histopathology of tissue proved to be neuroendocrine tumor with invasion into detrusor muscle (Figs. III, IV). Tumor stage was T2 N0 M0. Post operative Magnetic Resonance Imaging (MRI) showed clearance of tumor with no evidence of metastasis (Fig. V). Patient was not willing for cystectomy so she was referred to oncologist for adjuvent radio/chemotherapy.
Corresponding Author:
Dr. Muhammad Akram Malik,
Urology Department, UMDC, Faisalabad. Email: [email protected]
55 JUMDC Vol. 3, Issue 1, Jan-Jun 2012
MALIK MA, HAMEED S, et al. NEUROENDOCRINE TUMOR OF BLADDER
Figure I
Figure II
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Figure III
Figure IV
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Figure V
DISCUSSION
Primary small cell carcinoma (Neuroendocrine) is rare malignancy of urinary bladder. The differential diagnosis of a high grade urothelial carcinoma, a small cell carcinoma of the bladder, lymphoma and metastatic lesions especially from lungs should be kept in mind as lymphomas has better prognosis out of these malignancies. At cystoscopy neuroendocrine tumor cannot be differentiated morphologically from urothelial carcinoma. Histopathology confirm the diagnosis which is further supported by immunostaining. Treatment is dependent upon stage of tumor at time of presentation and patient’s general condition as it commonly presents in 7th decade. Treatment algorithm could not be established due to its low incidence. Cystectomy, partial cystectomy, transurethral resection and radiotherapy have been used to treat the localized disease. Neoadjuvent and adjuvent chemotherapy have also been tried. TURBT alone is usually not curative and have only 3–6 months survival of patients. TURBT is reserved only for those patients, which are not fit for more aggressive therapies. Patients
who were managed by TURBT and radiotherapy had 5–6.5 months median survival rate.4 Cystectomy and partial cystectomy is reserved for early stage (Stage II) disease.5 Choong and colleagues have reported 75% cure in patients who underwent cystectomy for stage 2 disease. Quek and associates6 have reported significant improvement in overall survival and reoccurrence free survival in patients who received adjuvent and neoadjuvent chemotherapy along with cystectomy as compared to those patients who underwent cystectomy alone. Siefker-Radtke7 and colleagues has published similar results with neoadjuvent chemotherapy. Chenge and colleagues8 has reported no survival benefit with multimodality approaches. On the other hand Choong and co-authors has reported 75% cure rate with radical cystectomy alone in stage 2 disease and has recommended radical cystectomy alone in stage I and II disease. Bladder sparing approaches using TURBT with radiotherapy/chemotherapy has been advocated by clinicians.9 Lohrisch and colleagues10 has reported 70% 2-years survival and 44% 5-
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years overall survival in patients treated with TURBT and chemo/radiation. Bex and colleagues11 also reported success with bladder sparing approaches; however reoccurrence rate of 20 to 60% has been reported in the literature.12 Review of literature regarding neuroendocrine tumor of urinary bladder showed that there is no consensus on treatment guidelines to manage this aggressive disease due to its low occurrence rate. Bladder sparing approaches are attractive options with risk of reoccurrence or residual disease in preserved bladder. CONCLUSION
Neuroendocrine tumor of urinary bladder is rare and aggressive disease with worst prognoses. Due to its low occurrence no authoritative treatment guidelines are available. Various treatment options are cystectomy, partial cystectomy, TURBT alone, TURBT with chemotherapy or radiotherapy. Prospective studies are required to establish the most effective treatment with better out come and less complications. REFERENCES
1. Jemal A, Murray T, Ward E, et al. Cancer
statistics, 2005. CA Cancer J Clin. 2005; 55: 10–30.
2. Mazzucchelli R, Morichetti D, Lopez-Beltran A, et al. Neuroendocrine tumours of the urinary system and male genital organs: clinical significance. BJU Int.
2009; 103: 1464–1470. 3. Naturale RT and Maclennan GT. Small cell
carcinoma of the bladder. J Urol. 2006; 176: 781.
4. Church DN and Bahl A. Clinical review–small cell carcinoma of the bladder. Cancer Treat Rev. 2006; 32: 588–593.
5. Choong NW, Quevedo JF and Kaur JS. Small cell carcinoma of the urinary bladder. The Mayo Clinic experience. Cancer. 2005; 103: 1172–1178.
6. Quek ML, Nichols PW, Yamzon J, et al. Radical cystectomy for primary neuroendocrine tumors of the bladder: the University of Southern California experience. J Urol. 2005; 174: 93–96.
7. Siefker-Radtke AO, Dinney CP, Abrahams NA, et al. Evidence supporting preoperative chemotherapy for small cell carcinoma of the bladder: a retrospective review of the M.D. Anderson cancer experience. J Urol. 2004; 172: 481–484.
8. Cheng L, Pan CX, Yang XJ, et al. Small cell carcinoma of the urinary bladder: a clinicopathologic analysis of 64 patients. Cancer. 2004; 101: 957–962.
9. Bastus R, Caballero JM, González G, et al. Small cell carcinoma of the urinary bladder treated with chemotherapy and radiotherapy: results in five cases. Eur Urol. 1999; 35: 323–326.
10. Lohrisch C, Murray N, Pickles T and Sullivan L. Small cell carcinoma of the bladder: long term outcome with integrated chemoradiation. Cancer. 1999; 86: 2346–2352.
11. Bex A, Nieuwenhuijzen JA, Kerst M, et al. Small cell carcinoma of bladder: a single-center prospective study of 25 cases treated in analogy to small cell lung cancer. Urology. 2005; 65: 295–299.
12. Shailen S Sehgal, Alan J Wein, Zhanyong Bing, S. Bruce Malkowicz, and Thomas J Guzzo. Neuroendocrine Tumor of the Bladder. Rev Urol. 2010; 12(4): e197–e201.
Submitted for publication: 06-02-2012 Accepted for publication: 20-02-2012
