血尿罕見的鑑別診斷- 副神經瘤案例報告與文獻回顧

黃英哲¹ 、陳彥達¹

¹高雄長庚紀念醫院外科部泌尿科

Rare differential diagnosis of hematuria: A case report and review of paraganglioma

Ying-Che Huang¹、Yen-Ta Chen¹

¹Divisions of Urology, Department of Surgery, Kaohsiung Chang Gung Memorial Hospital

Case description: A 68-year-old male presented to our outpatient department with a chief complaint of mild intermittent hematuria for one week. The patient’s medical history is significant for 1) End stage renal disease on peritoneal dialysis since 2014, 2) Sinus arrhythmia with premature atrial complexes, 3) Spondylolisthesis of L4/5, with thecal sac narrowing, 4) Left knee advanced osteoarthritis with gout, post total knee replacement in 2015. He had no history of diabetes, hypertensive, hypothyroid, or hyperlipidemia. He was not known to have headaches, palpitations, or dizziness associated with micturition or postural changes. Physical examination did not find a palpable kidney, lower extremity edema, or right costovertebral angle tenderness. A complete blood count revealed a normal white blood cell count of 5.3 × 103/µl, with a hemoglobin of 9.9 g/dL and platelets 168 × 103/ul. Blood chemistry showed sodium to be 128 mEq/l; potassium, 4.4 mEq/l; serum creatinine, 9.96 mg/dl. Urinalysis cannot be done due to oliguria. The flexible cystoscope found a bladder stone in the bladder. Under the impression of hematuria with a bladder stone, he was admitted for endoscopic cystolitholapaxy. However, intraoperatively, the cystoscope found a small bladder stone and a 1 cm-sized bladder tumor located in the right lateral wall with round appearance and sessile base. Transurethral resection of the bladder tumor (resection to the depth of the deep muscle layer) was done and the specimen was sent for histopathological examination.

Pathological reports showed tumor cells, bearing round nuclei and abundant cytoplasm, were arranged in a nested growth pattern. The tumor did not invade muscularis propria. Immunohistochemical studies revealed paraganglioma (PG) as positive for synaptophysin, and chromogranin, but negative for CK7. The postoperative course was uneventful and was discharged in the following days. Currently, the patient receives follow-ups in our Urology outpatient department for further evaluation in our hospital.

Discussion: PG is an extra-adrenal pheochromocytoma and is a rare type of bladder tumor. Bladder PGs only account for 0.06% of bladder tumors and 10% of extra-adrenal pheochromocytoma¹. Bladder PGs can be functional or non-functional based on the catecholamine level. According to previous studies, 83% of them are functional², with clinical features including persistence or paroxysmal hypertension, hypertensive crisis during micturition, headache, palpitation, and painless gross hematuria³. However, less than a third of bladder PGs can be successfully diagnosed before the surgery and non-functional bladder PGs may be misdiagnosed as urothelial carcinoma (UC)⁴. Histologically, it is characterized by nested-pattern cells, with granular cytoplasm and surrounded by abundant vessels⁵. But they can be hard to differentiate from UC because of muscularis propria infiltration and procedure-related artifact.^6,7 Thus, immunohistochemistry is useful in definitive diagnosis. PG expresses neuroendocrine markers, like synaptophysin, chromogranin, and CD56, while UC expresses cytokeratin⁴. TURBT or partial cystectomy for complete tumor removal is the most effective way of treatment. Long-term follow-up is recommended and further chemoradiotherapy might be needed for metastases⁸.

Conclusions: PG is a rare neoplasm and may mimic UC in the bladder. Although image studies and laboratory catecholamine tests might be helpful in diagnosis, a definite diagnosis usually relies on the immunohistochemistry of the tumor. Surgical resection is generally an effective treatment, but further outpatient follow-up is needed for those patients.

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6.     Menon S, Goyal P, Suryawanshi P, et al. Paraganglioma of the urinary bladder: a clinicopathologic spectrum of a series of 14 cases emphasizing diagnostic dilemmas. Indian J Pathol Microbiol. Jan-Mar 2014;57(1):19-23. doi:10.4103/0377-4929.130873

7.     Zhou M, Epstein JI, Young RH. Paraganglioma of the urinary bladder: a lesion that may be misdiagnosed as urothelial carcinoma in transurethral resection specimens. Am J Surg Pathol. Jan 2004;28(1):94-100. doi:10.1097/00000478-200401000-00011

8.     Saha A, Saha K, Sarkar N, Geelani IA. Paraganglioma of Urinary Bladder: An Uncommon Entity in Uropathology. Cureus. Aug 2021;13(8):e17265. doi:10.7759/cureus.17265