Bladder Paraganglioma: Experiences in National Taiwan University Hospital

1. Bladder Paraganglioma: Experiences in National Taiwan University Hospital Yi-Kai Chang, Chao-Yaun Huang, Yeong-Shiau Pu, Chung-Hsin Chen, Shuo-Meng Wang,Yuan-Ju Lee, Huai-Ching Tai, Hong-Jeng Yu Department of Urology, National Taiwan University Hospital, College of Medicine, National Taiwan University

2. Introduction • Pheochromocytomas are catecholamine-secreting tumors, derived from the adrenal gland and sympathetic nervous system. • Extra-adrenal pheochromocytomas, known as paragangliomas, account for about 15 % of all pheochromocytomas. • Extremely rare less than 0.06% of all bladder tumors , < 1% of all pheochromocytomas • Micturition attack

3. Introduction • Definition of malignant paraganglioma Benign in nature but some cases with malignant behavior • No Definite pathology criteria for malignancy  local invasion  distant metastasis

4. Materirals and Methods • We retrospectively searched the pathological and clinical database of National Taiwan University Hospital with ICD-9 and diagnosis in recent 15 years. • 6 cases of bladder paraganglioma were collected.

5. Results Results • Case 1 • 21 y/o male • TUR-BT  paraganglioma • CT: 5.5*5.6 cm bladder tumor • Partial cystectomy in 2001 0828 • Pathology: malignant paraganglioma with perivesical fat invasion • Cystoscopy and abdominal and pelvic MRI followup 122 months so far and still alive

6. Results Case 2 • 53 y/o female • Bladder blood clot tamponade • CT: huge bladder tumor with uterus/ vaginal invasion • Partial cystectomy, ATH+BSO • Patholgy: bladder malignant paraganglioma with uterus, bil ovary and vaginal invasion • V-V fistula was noted 3 months after operation • Died 21 months postoperatively of severe sepsis

7. Results • Case 6 • 68 y/o male, BPH with LUTS • TUR-P+ TUR-BT 2011 04 • Pathology Bladder paraganglioma • CT: 6 mm enhanced lateral wall tumor • TUR-BT 201208, • Pathology: bladder paraganglioma

8. Table 1 Clinical characteristics of 6 patients with bladder paraganglioma

9. Table 2 tumor characteristics, surgical procedures and pathology data

10. Results • The specimens of two malignant bladder paragangliomas were largepolypoid tumors, both rich in blood supply and were broad based tumors. • The specimens of other four benign paragangliomas were all smallround or ovoid tumors

11. Discussion • Doctor Dana[1]reported 236 extra-adrenal benign paragangliomas, only 2 were bladder origins, 1 prostate paraganglioma • Our data A trend of female predominance [1]Erickson D, Benign paragangliomasJ Clin Endocrinol Metab. 2001 Nov;86(11):5210-6. [2]Leestma JE, Price EB: Paraganglioma of the urinary bladder. Cancer 1971; 28:1063-1073 [3]Paraganglioma of the urinary bladder first presented by bladder bloody tamponade: two case reports and review of the literatures. Tsai CC, Kaohsiung J Med Sci. 2011 Mar;27(3):108-13. Epub 2011 Feb 16.

12. Discussion [2]Leestma JE, Price EB: Paraganglioma of the urinary bladder. Cancer 1971; 28:1063-1073 [3]Paraganglioma of the urinary bladder first presented by bladder bloody tamponade: two case reports and review of the literatures. Tsai CC, Kaohsiung J Med Sci. 2011 Mar;27(3):108-13. Epub 2011 Feb 16.

13. Factors Suggesting Malignancy [4]Chin J Cancer. 2010 Aug;29(8):729-34. Functional paragangliomas of the urinary bladder: a report of 9 cases. Deng JH

14. Surgical Intervention and Outcome in Taiwan

15. TUR or partial cystectomy Defects in TUR: • Involve the muscularis propria of the bladder wall • Easy to recur if the tumor cannot be resected completely • Higher malignancy rate than pheochromocytoma [5]Diagnosis and treatment of pheochromocytoma in urinary bladder LIU Yong, J Zhejiang Univ Sci B. 2007 June; 8(6): 435–438

16. Discussion • Post operative follow-up protocol should include annual cystoscopy, plasma or urine catecholamine analysis and an 131I-MIBGscan. • Annual biochemical testing [6] • Life-long follow-up is necessary to detect late recurrences as previous studies suggested. [6]Young WF. Paragangliomas: clinical overview. Ann N Y Acad Sci 2006;1073:21e9

17. Conclusion • Female predominant • Painless gross hematuria  most common presenting symptom • Large, multiple, polypoid tumors  malignant • For small-sized lesion, TUR-BT but regular followup (image, biochemical testing, cystoscopy ) • For large-sized lesion, partial / radical cystectomy • Life long followup

18. Thanks for your attention

21. Pre-OP prophylaxis? • adrenergic blockade should be started at least 7 days preoperatively to allow for expansion of the contracted blood volume Young WF. Paragangliomas: clinical overview. Ann N Y Acad Sci 2006;1073:21e9.

22. Post OP followup? • 1–2 weeks after surgery, 24-h urinary fractionated catecholamines and metanephrines • If the levels are normal, the resection of the biochemically active paraganglioma should be considered complete • Annual biochemical testing metastasis, recurrence [6]Young WF. Paragangliomas: clinical overview. Ann N Y Acad Sci 2006;1073:21e9.

23. Table 4 Tumor Characteristics and Malignant Potential 謝佳秀

24. Surgical intervention in China

25. Table 3 Gender and Bladder Paraganglioma

26. Table5 Surgical Intervention and Outcome in Taiwan