原發性腎臟副神經節細胞瘤 – 案例報告
潘柏勳1、張延驊1,2,3、黃志賢1,2,3
1台北榮民總醫院 泌尿部；2陽明大學醫學院泌尿學科；3書田泌尿學研究中心
Primary renal paraganglioma: a Case Report
Po-Hsun Pan1, Yen-Hwa Chang1,2,3, William J.S. Huang1,2,3
1Department of Urology, Taipei Veterans General Hospital, Taipei, Taiwan
2Department of Urology, School of Medicine, National Yang-Ming University, Taipei, Taiwan
3Shu-Tien Urological Institute, National Yang-Ming University, Taipei, Taiwan
Introduction:
Paraganglioma is an uncommon catecholamine secreting neuroendocrine tumor which derived from chromaffin cells of adrenal medulla (known as pheochromocytoma) and extra-adrenal sympathetic paraganglia. Due to the high secretion of catecholamine, it might cause some tumor specific symptoms such as refractory hypertension, chest pain, headache, sweating, palpitation or tremors. Most of the tumors arise from adrenal gland with only about 10 percent of paraganglioma are located at extra-adrenal organs, and which occurs in genitourinary system such as urinary bladder, prostate, and kidney are even rare. Here we would like to report a rare case of primary renal paraganglioma.
Case Report:
A 29-year-old male patient has no any systemic medical disease. He was incidentally found to have a right renal mass by abdominal sonography during physical checkup. According to the patient’s statement, he did not have any specific symptoms such as abdominal pain, flank pain or hematuria. No history of hypertension, headache, tremors or shortness of breath. Physical examination showed no abdominal palpable mass, costovertebral angle knocking pain or abdominal tenderness. CT scan disclosed one 3.2 cm soft tissue mass at right kidney with homogenous enhancement. Invasion of renal sinus fat cannot be ruled out. MRI revealed a 2.8 cm poor enhancing lesion at upper pole of right kidney and directly attached to the collecting system. No abnormal enlarged lymph nodes and bilateral renal vein and inferior vena cava were patent. Due to the first impression of renal cell carcinoma, the patient received nephron sparing open partial nephrectomy on 2019-06-24. There was no specific surgical related event such as blood pressure surges during the whole course of operation. The tumor was resected completely and the collecting system was found not invaded by tumor. The resection tumor was checked microscopically which made a final diagnosis of renal paraganglioma. We post operatively check his hormone profile, 24 hours urine VMA was 38.7 mg/day (normal range < 7mg/day), 24 hours urine catecholamine were in normal limit: epinephrine - 8.8 mcg/day; norepinephrine – 22.4 mcg/day; dopamine – 162.3 mcg/day and blood catecholamine were also in normal range. We followed up 24 hours urine VMA 1 month post-operatively and showed the data dropped to 4.1 mg/day. Followed up whole body PET showed small foci of increased FDG uptake in the right retroperitoneal region, however, no abnormal hormone data was found. So closely follow up might be indicated.
Conclusions:
We described a rare case of paraganglioma which primarily located at kidney. Though the diagnosis are hardly to made, the surgical outcome could be pretty good.