雙擺位達文西手術技巧運用於同時發生在膀胱及腎上腺的遺傳性副神經節瘤-嗜鉻細胞瘤症候群
溫晨越、余家政、蔡政諭、陳逸軒、林仁泰
高雄榮民總醫院 外科部 泌尿外科
Dual Docking Robotic Surgical Technique for Hereditary Paraganglioma-pheochromocytoma Syndrome of Synchronous Tumors in Bladder and Adrenal Gland
Chen-Yueh Wen, Chia-Cheng Yu, Jeng-Yu Tsai, I-Hsuan Alan Chen, Jen-Tai Lin
Divisions of Urology, Department of Surgery, Kaohsiung Veterans General Hospital, Kaohsiung, Taiwan
Background:
Surgical resection of all tumors in cases of hereditary paraganglioma-pheochromocytoma (PGL/PCC) syndromes is the standard treatment for excess adrenaline production and is important because of tendency for metastasis. Herein we present a rare case of hereditary PGL/PCC syndromes treated by partial cystectomy and right adrenalectomy at the same time with dual docking robotic surgical technique.
Case Presentation:
A 32-year-old woman was incidentally found to have a urinary bladder mass due to epigastric pain in 2019/10. Abdominal computed tomography demonstrated a mass up to 4.5 cm over anterior wall of the bladder and a nodule over right adrenal gland. Transurethral resection of the bladder tumor (TURBT) was performed and pathology confirmed the diagnosis of bladder paraganglioma with bladder muscular invasion. Therefore, she was prompted a referral to our institution for further management. Tracing back history, episodes of sudden onset of palpitations, chest tightness and headache after voiding were noted on and off over the past 6 years. We advised her to receive robotic partial cystectomy and right adrenalectomy simultaneously. First, the patient was placed in a modified lithotomy with steep Trendelenburg position for conducting partial cystectomy. Then, she was placed in modified lateral decubitus position. Right adrenalectomy was successfully performed via transperitoneal approach. The docking time for both procedures were 145 minutes and 80 minutes respectively. Total blood loss was 50 ml. No immediate intraoperative complication was noted. The duration of hospital stay was 4 days. With reference to the pathology report, immunohistochemical stain with SDHB is performed for surveillance due to synchronous existence of tumors, which reveals negative result of the tumor cells. It may suggest the presence of a germline pathogenic variant of one of the SDH subunits. Bladder and adrenal tumors compatible with hereditary PGL/PCC syndrome caused by SDHx mutations.
Conclusion:
Our dual docking robotic technique is a feasible option for patients with hereditary PGL/PCC syndromes of synchronous tumors in bladder and adrenal gland. It could not only prevent from second surgery but be safely performed without compromising disease control.