合併鈣化與非典型內分泌表現之雙側腎上腺腫瘤：病例報告

吳哲安、鄒凱亦

衛生福利部台北醫學大學雙和醫院 泌尿科

Bilateral Adrenal Tumors With Calcification and Atypical Endocrine Findings: A Case Report

Che-An Wu, Kai-Yi Tzou

Division of Urology, Taipei Medical University Shuang Ho Hospital, New Taipei City, Taiwan

Background: Primary pigmented nodular adrenocortical disease (PPNAD) is a rare adrenal disorder that can be difficult to distinguish from other adrenal tumors, especially when radiologic findings suggest malignancy. Its clinical and endocrine manifestations may overlap with other adrenal diseases, making preoperative diagnosis challenging.

Case Presentation: A 50-year-old woman presented with chronic left flank soreness for 6 months. Abdominal computed tomography revealed bilateral lobulated suprarenal tumors measuring up to 5.6 cm, with mild enhancement, coarse calcification, feeding vessels, and focal fat components, initially favoring adrenocortical carcinoma. Endocrine evaluation demonstrated markedly low morning serum cortisol (<1.0 μg/dL), low adrenocorticotropic hormone (6.8 pg/mL), elevated plasma renin activity (9.29 ng/mL/hr), and near-normal aldosterone (25.64 ng/dL). She received preoperative steroid and mineralocorticoid replacement with dexamethasone and fludrocortisone. Because of persistent symptoms and concern for malignancy, laparoscopic left adrenalectomy was performed on March 12, 2026. Perioperative hydrocortisone (100 mg intravenously) was administered immediately after surgery. The postoperative course was uneventful, without adrenal crisis, severe hypotension, or major complications, and the patient was discharged on March 15, 2026.

Pathological Findings: Histopathological examination showed nodular adrenocortical proliferation composed of sharply circumscribed but unencapsulated nodules of eosinophilic, lipid-poor cells with abundant lipofuscin pigment, raising suspicion for Primary Pigmented Nodular Adrenal Dysplasia (PPNAD). Immunohistochemical staining showed diffuse Melan-A positivity in cortical cells, focal weak inhibin-α positivity, and a low Ki-67 proliferation index (4%). A coexisting myelolipoma with extensive ossification and calcification was also identified. No histologic evidence of adrenocortical carcinoma was found, and the modified Weiss score was 0.

Conclusion: This case highlights that PPNAD may present as bilateral adrenal masses with atypical endocrine findings and radiologic features mimicking adrenocortical carcinoma. In such cases, definitive diagnosis depends on histopathological examination. Careful perioperative steroid replacement is essential to prevent adrenal insufficiency–related complications.