後腹腔副神經節瘤之病例報告與文獻回顧

張賀翔、陳建旭

高雄長庚紀念醫院 泌尿科

A case of retroperitoneal paraganglioma - Case report and literature review

Chang Ho Hsiang, Chen Chien Hsu

Department of Urology, Kaohsiung Chang Gung Memorial Hospital, Taiwan

Case presentation:
This 47-year-old woman with past history of adenomyosis was initially presented with nocturia and poor sleep quality. She had visited urological clinic for her bothersome problem. However, sonography revealed a left retroperitoneal mass. There were no symptoms such as sweating, hot flushing, nausea/vomiting, or blurred vision. Physical examination did not showed any positive sign. Therefore, she was referred to our hospital for further evaluation. Abdominal CT revealed (1)one mixed cystic and solid component mass(3.2x3.5cm) in left retroperitoneal region (figure1). DDx: metastatic lymph node, neurogenic tumor, sarcoma (2)Hypodense lesion 5.7cm in pelvic cavity in perirectal region, DDx: loculated ascites, mucinous tumor (3)Cystic lesion in left adnexal region, DDX: functional cysts or cystic tumor. The left adnexal tumor favored benign lesion on GYN sonography. The left retroperitoneal mass and a cystic tumor near middle rectum were removed by Da vinci robotic surgery system. The postoperative samples were shown in figure2 . The morphology: (1) the left retroperitoneal mass was encapsulated, size measuring 3.0 x 2.5 x 2.2 cm (2) the middle rectal cystic lesion was sized 5.5 x 2.5x 1.0 cm. The pathology: (1) the left retroperitoneal mass was paraganglioma, and the margin was uninvolved by tumor. No lymph node found. The pathologic staging was T1Nx , AJCC 8th Pathological stage: I. Hemorrhage and cystic change also noted on this tumor. Immunohistochemical stain showed PHOX2B:(+), synaptophysin: (+), chromogranin A:(+), focal weak, AE1/AE3: (-), SDHB: (+), retained (2) the middle rectal lesion was mesothelial cyst. Now, she is regularly followed up at our urology department. The patient had no discomfort or symptoms when she was discharged from the hospital. Abdominal CT will be arranged six months later.
Discussion:
The paraganglioma is a rare disease, also as known as extra-adrenal pheochromocytoma. The is about 0.6 cases per 100,000 person-­years. Surgical resection of paraganglioma is the mainstream management for the paraganglioma. Besides, preoperative preparation is important, including blood pressure control before surgery, water expansion during operation, and anesthetic consultation. Life time follow-up is necessary to detect any sign of recurrence. If any evidence of distant metastasis noted, systemic chemotherapy was eligible according to the previous study. The most commonly used protocol was CVD (cyclophosphamide + vincristine + dacarbazine), with an objective response rate of 33%.

Conclusion: