Background: Adrenal cortical carcinoma (ACC) is a rare and aggressive malignancy; diagnostic tools for a pathologic confirmation of ACC are currently limited to scoring systems such as the modified Weiss criteria, the Helsinki score, and criteria proposed by Bisceglia et al. There is lack of consensus on appropriate monitoring and therapeutic strategies for suspicious lesions that do not fulfill all criteria for malignancy or metastatic potential. This is a case of a previously resected localized adrenal neoplasm with progression to metastatic ACC 1 year later.
Clinical Case: A 63-year-old male with hypertension and type 2 diabetes was referred to Endocrinology due to fragility fractures, weight gain, and uncontrolled hypertension concerning for cortisol excess. Workup revealed a 24-hour urinary free cortisol of 283 μg [0–50 μg/24hr], a post-1 mg dexamethasone suppression cortisol of 24.6 μg/dL [<1.8 μg/dL], and a random ACTH of <1.1 pg/mL. CT adrenals revealed a left 3.3 cm nodule measuring 40 Hounsefield units and 27% washout. The patient underwent left adrenalectomy with clear margins. Pathology reported an oncocytic adrenal cortical neoplasm with a Ki-67 of 4.3%, an elevated mitotic activity of up to 12 mitoses per 50 high-power fields, and large nuclei with prominent nucleoli. There was no evidence of necrosis, capsular or vascular invasion. The final pathologic diagnosis was “adrenal cortical neoplasm with worrisome features”, and close follow-up was recommended. 1 year later, the patient presented with recurrent Cushing’s syndrome; he was found to have metastatic ACC with lesions in the adrenalectomy bed, liver, and lungs. Despite maximal medical therapy, the patient suffered severe sequelae of Cushing’s and transitioned to palliative measures.
Clinical Lessons: There are several pathologic scoring systems used to diagnose and predict prognosis in ACC. The challenge of this case is balancing fulfilling the highly-sensitive Bisceglia criteria for ACC [1 “major criterion” of high mitotic rate favors oncocytic ACC] with the other aspects that suggest a more positive outcome: clear surgical margins and a Helsinki score of 7.3 [≥8.5 favors metastatic potential]. His diagnosis fell into an intermediate area of “neoplasm with worrisome features”, and he did not receive adjunctive mitotane therapy. Current guidelines do not recommend adjuvant mitotane therapy for indeterminate lesions or for cases of confirmed ACC with Ki-67 <10% after complete resection, and there is limited guidance regarding monitoring of such lesions. Retrospectively, it is unclear if our patient would have had an improved outcome with adjunctive mitotane therapy or closer surveillance for recurrence.
Bisceglia M, Ludovico O, Di Mattia A, Ben-Dor D, Sandback J, Pasquinelli G, Lau SK, Weiss LM. Adrenocortical Oncocytic Tumors: Report of 10 Cases and Review of the Literature. Int J Surg Pathol. 2004 Jul; 12(3)231–43