MON-496 - A Red Herring: An Unusual Presentation of Bilateral Adrenal Masses

Background:
The workup for bilateral adrenal masses is broad. We discuss a patient found with new incidental bilateral adrenal masses initially confused as a genetic syndrome.

Clinical

Case:
A 30-year-old male with PMH of cardiac myxoma presented 4 months after resection with shortness of breath and was referred for heart transplant. MRI obtained as part of transplant workup revealed new bilateral adrenal masses (8.3 cm R and 3.5 cm L) with radiology concerned for Carney complex due to the history of cardiac myxoma. Notably, there was no mention of adrenal nodules on abdominal imaging 6 months ago at an outside hospital. A CT abdomen demonstrated bilateral heterogenous lesions HU > 20 throughout with retroperitoneal nodules. Physical exam demonstrated palpable nodules in the right leg and left shoulder without signs of hypercortisolism. Laboratories showed TSH 4.28 (0.5-5 mIU/L), FT4 1.33 (0.8-1.9 ng/dL), aldosterone 6 (3.1 - 35.4 ng/dL)​, renin 11.51 (0.5–4.0 ng/mL/hr)​, PM ACTH 5 (10-60 pg/mL)​, LH 4.8 (1.7-8.6 mlU/mL)​, prolactin 21.2 (4-15.2 ng/mL), IGF-1 100 ng/mL (Z score –0.9), free testosterone 12PM 36.3​, metanephrines 0.12 ( < 1.1pg/mL), and normetanephrine 1.2​ ( < 0.9 pg/mL). Low-dose dexamethasone suppression test was 3.0 mcg/dL and 24-hour urine free cortisol (UFC) was 17.1 (4-50 mcg/24 hr). Biopsy of the leg nodule was pursued, resulting as leiomyosarcoma. Further biopsy of the adrenal lesions was deferred as it would not affect staging and would delay systemic therapy for leiomyosarcoma with oncology.

Discussion:
Due to the history of cardiac myxoma, new adrenal masses, and non-suppressed cortisol, there was initial concern for Carney complex. However, given the acuity of the new adrenal masses, physical exam without hypercortisolism, external nodules, and radiologic findings, the differential seemed not as clear.

This case highlights the workup in a patient with bilateral adrenal nodules, including systemic infection, genetic syndromes, or malignancy. Imaging of choice is typically a CT scan for evaluation of density and size as well as characterization if the adrenal masses appear uniform or heterogenous in appearance. In Carney complex, the typical presentation of adrenal disease is primary pigmented nodular adrenal dysplasia, which appears as multiple micronodules in the adrenal gland on imaging and clinically with hypercortisolism. In contrast, primary adrenal leiomyosarcoma is an extremely rare finding and usually associated with unilateral disease. We suspect given the patient’s prior retroperitoneal masses noted several months prior and no unilateral adrenal disease, the patient had leiomyosarcoma of the retroperitoneum and subsequent bilateral adrenal metastasis given the radiologic and physical exam findings, which itself is a rare finding. Our case highlights the importance of avoiding anchoring the diagnosis on prior history for workup of adrenal masses.

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