An oncocytic adrenocortical tumor initially classified as uncertain malignant potential progressed to metastatic adrenocortical carcinoma within nine months post-resection.
Oncocytic adrenocortical tumors of uncertain malignant potential can recur and metastasize despite complete resection, necessitating long-term surveillance.
Absolute Event Rate: 0% vs 0%
Abstract Introduction Oncocytic adrenocortical neoplasms (AONs) are rare tumors, comprising 5% of adrenal cortical neoplasms. Most are benign, but a subset classified as oncocytic adrenocortical neoplasms of uncertain malignant potential (AONUMP) may recur or metastasize. Clinical Case A 43-year-old woman was referred to the Faiha Specialized Diabetes, Endocrine and Metabolism Center (FDEMC), Basrah, south of Iraq, in June 2022 for evaluation of an incidentally detected left adrenal mass during investigation of loin pain from a renal stone. She reported a seven-month history of weight gain (13 kg), progressive hirsutism, and amenorrhea. More recently, she developed polyuria, polydipsia, nocturia, and was diagnosed with diabetes mellitus and hypertension. Examination revealed obesity (BMI 41.2 kg/m²), blood pressure 171/106 mmHg, facial plethora, acanthosis nigricans, and marked hirsutism (Ferriman–Gallwey score 24/36). There were no abdominal striae or organomegaly. Biochemical evaluation demonstrated elevated DHEA, suppressed ACTH, cortisol non-suppression after dexamethasone, hyperandrogenemia, and impaired glucose metabolism. CT scan revealed a large left adrenal mass (75 × 73 × 80 mm, 40 HU). In July 2022, she underwent left adrenalectomy, removing a 10 × 10 cm, 350 g adrenal mass. Histopathology confirmed oncocytic adrenocortical tumor of uncertain malignant potential based on Lin–Weiss–Bisceglia (LWB) criteria. At four months follow-up, symptoms improved: menses resumed, diabetes and hirsutism resolved, weight decreased by 9 kg, and biochemical parameters normalized. However, nine months postoperatively (April 2023), she presented with recurrent hirsutism, amenorrhea, uncontrolled hypertension and diabetes, bilateral loin pain, and bone pain. Biochemistry showed recurrent hypercortisolism and androgen excess. CT demonstrated para-aortic lymphadenopathy, and excisional biopsy confirmed metastatic adrenocortical carcinoma. She underwent lymph node dissection and was referred for targeted chemotherapy. Despite treatment, she relapsed again in July 2025. PET scan revealed multiple hypermetabolic nodules involving the left suprarenal region, abdominal peritoneum (SUVmax 8.6), right iliac fossa, and paracolic gutter, consistent with disseminated disease. Conclusion We report malignant progression of an oncocytic adrenocortical tumor initially classified as uncertain malignant potential. This case underscores that, despite complete resection and initial improvement, recurrence and metastasis may occur. Long-term surveillance, comprehensive hormonal evaluation, and refined prognostic markers are essential for optimal management.Figure 1:Left adrenal mass gross spciemen and radiologyLeft lower image represent axial CT image demonstrating a large heterogeneous left adrenal lesion with well-defined margins., upper images demonstrating Gross specimen of the resected left adrenal mass, measuring approximately 10 cm, showing heterogeneous cut surface. Right lower image demonstrating coronal CT image showing left adrenal metastatic nodules (measuring up to ∼3 cm), upper image demonstrating excised nodules, consistent with metastatic deposits. Table 1:Timeline of Key Investigations and FindingsTable 1 summarizes the key investigations, hormonal evaluations, imaging findings, and histopathology of the patient at different time points during presentation, post-adrenalectomy follow-up, recurrence, and relapse. Laboratory values are reported with standard clinical units. Imaging details include adrenal mass size, density (HU), and lymph node or metastatic lesions. The timeline highlights disease progression, management interventions, and biochemical changes over time.
Zuhairi et al. (Thu,) reported a other. An oncocytic adrenocortical tumor initially classified as uncertain malignant potential progressed to metastatic adrenocortical carcinoma within nine months post-resection.
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