腎上腺偶見瘤的高級模仿者:後腹腔神經節細胞瘤之案例分享與文獻回顧

李翊維¹  王世鋒^1,2

¹國泰綜合醫院外科部 泌尿科  ²汐止國泰綜合醫院外科部 泌尿科

Retroperitoneal ganglioneuroma mimicking adrenal incidentaloma – Case report and literature review

Yi-Wei Lee¹   Shih-Feng Wang^1,2

¹Division of Urology, Department of Surgery, Cathay General Hospital, Taipei City, Taiwan

²Division of Urology, Department of Surgery, SiJhih Cathay General Hospital, New Taipei City, Taiwan

Ganglioneuromas are tumors originating from neural crest cells. They tend to occur in adolescents and young adults. Neuroblastoma, ganglioneuroblastoma, and ganglioneuroma can be conceptualized as three maturational manifestations of a common neoplasm. Ganglioneuromas are highly differentiated benign tumors which do not contain neutrophils, intermediate cells, or mitotic figures. Ganglioneuromas are compatible with long-term, disease-free survival even with unsatisfactory surgical treatment.

A 34-year-old male patient came to our gastroenterology clinic that complained of left lower quardrant of abdomen pain accompanied with intermittent constipation and diarrhea for 1 week. Physical examination revealed left lower quardrant of abdomen tenderness with rebound pain. Abdominal computed tomography was arranged and showed an ovoid tumor about 4cm-sized at right suprarenal region just behind intrahepatic IVC. Thus, he was referred to endocrinology clinic for adrenal tumor survey that the adrenal function test were within normal range. A pre-operative evaluation led to the possible diagnosis of primary retroperitoneal tumor. A laparoscopic resection was scheduled for the non-functioning retrocaval tumor.

Herein, we reported a patient with pulmonary embolism due to severely invaded right clear cell carcinoma into right atrium, right ventricle, even pulmonary arteries. After surgery, he was recovered smoothly and uneventfully. During laparoscopic exploration the mass was found in close relation to, and behind, the renal veins, inferior vena cava. Total resection of the tumor was performed. The resected specimen was 6.0 * 6.0 * 2.0 cm in size. The histological examination showed that the lesion was an extra-adrenal retroperitoneal ganglioneuroma composed of mature ganglion cells and nerve fibers without any malignant features. The patient’s total hospitalization lasted 4 days, and the postoperative course was uneventful.

Ganglioneuroma is a rare benign tumor of the sympathetic nervous system. Few reports have been published of surgical resection of this rare tumor using a laparoscopic approach. Ganglioneuroma can arise from the adrenal medulla; however, in the present case, it had originated from the retrocaval sympathetic chain and mimicked an adrenal mass on imaging.