成熟型神經節細胞瘤：非功能性腎上腺腫瘤之罕見病例報告

何岩¹、溫玉清^1,2

臺北市立萬芳醫院-委託財團法人臺北醫學大學辦理 泌尿科¹

臺北醫學大學醫學院醫學系 泌尿學科²

Mature-Type Ganglioneuroma: A Rare Case Report of Non-Functional Adrenal Tumor

Yen Ho¹, Yu-Ching Wen^1,2

Department of Urology, Taipei Municipal Wan Fang Hospital¹

Department of Urology, School of Medicine, College of Medicine, Taipei Medical University²

Introduction: The incidence of ganglioneuroma is reported around one case per million population, and retroperitoneal GN accounts for 0.72 to 1.6% of all primary retroperitoneal tumors. While the features of adrenal ganglioneuroma as elucidated through computed tomography and magnetic resonance imaging have been extensively documented, the precise diagnosis remains a challenging endeavor. Ganglioneuromas, upon surgical resection, demonstrate a highly favorable prognosis. In this report, we present a case of an incidental discovery of adrenal ganglioneuroma in a male patient, notably characterized by elevated standardized uptake values in a positron emission tomography scan.

Case Presentation: In a case involving a 43-year-old female with a surgical history of right oophorectomy for a right ovarian cyst, the patient presented with a gradual onset of lower leg swelling and soreness persisting for several weeks. Suspected left iliac vein compression syndrome led her to seek evaluation at the lymphovascular surgery clinic. Magnetic resonance imaging (MRI) confirmed the presence of iliac vein compression syndrome and incidentally discovered a 5.8 cm right adrenal mass with mild enhancement. Subsequent evaluation entailed an abdomen and pelvis computed tomography (CT) scan, which revealed a 5.7 x 3.5 cm right adrenal lesion exhibiting linear calcification and inhomogeneous enhancement. Laboratory analysis disclosed a low aldosterone level (12.16 pg/mL), normal plasma renin activity (0.52 ng/mL/hr), and a normal adrenocorticotropic hormone (ACTH) level (8.78 pg/mL).

The patient subsequently underwent robotic-assisted laparoscopic right adrenalectomy, which transpired without major complications. Gross examination of the resected specimen revealed a well-defined, elastic, and brown tumor, partially enveloped by adrenal tissue. Microscopically, the tumor displayed characteristic features consistent with a mature type of ganglioneuroma, primarily composed of spindle Schwann cells organized in fascicles. The patient recovered from the surgery smoothly.

Conclusion: In summary, adrenal ganglioneuroma warrants consideration in the differential diagnosis of any adrenal or retroperitoneal mass. Accurate diagnosis remains a formidable challenge, with histological examination representing the sole definitive means of confirmation. Nevertheless, the overall prognosis for adrenal ganglioneuromas is generally favorable, and recurrence following surgical resection is a rare occurrence.