Abstract
Introduction: Adrenocortical Carcinoma (ACC) is a rare disease, with an incidence of 0.7–2 cases per million people. Approximately 80% of ACC tumors are functional, with the majority of tumors secreting glucocorticoids, however a small proportion concurrently secrete androgens in addition to glucocorticoids. Here, we describe a case of ACC presenting with feminization, without clinical evidence of Cushing’s syndrome.
Case Description: A 35 year old male with no significant history presented with sudden onset non-radiating, 7/10, abdominal pain 4 days prior to admission. On exam, the patient had diffuse tenderness to palpation in the right upper quadrant without other abdominal findings. He also was noted to have palpable breast tissue bilaterally. Upon persistent questioning, he reported an 8-month history of gradual breast development, decreased libido and decreased morning erections. He had no history of hypertension, headaches, diabetes, palpitations, flushing, sweating, weight gain, striae, bruising, or muscle weakness. Diagnostic CT imaging of the abdomen and pelvis showed a 14 cm right adrenal mass without invasion or lymphadenopathy. Laboratory testing showed a total testosterone of 37 ng/dL (reference range:249–846 ng/dL), estradiol of 181 pg/mL (8–43 pg/mL), prolactin of 14.5 ng/ml (4.0–15.2), and FSH and LH were both <0.1 mIU/mL. Serum metanephrines and normetanephrines, renin and aldosterone showed no evidence of excess secretion. Urine free cortisol measurement was 111mcg over 24 hrs (<50 mcg/24hr) and ACTH was <1.0 pg/mL (7.2–63.3 pg/mL). Surgical resection was performed with histopathologic diagnosis of an 18cm ACC confined to the adrenal gland. Post-surgical prophylactic hydrocortisone replacement was given. At outpatient follow-up, testing revealed normalization of his estradiol and testosterone levels. The patient had no clinical evidence of adrenal insufficiency and plans were made to assess endogenous adrenal function.
Conclusion: This case highlights the importance of discussing symptoms of hypogonadism, virilization or feminization with patients in detail when evaluating for ACC. Careful history and physical exam may be key to identifying sex hormone excess and prompting preoperative workup. In this case of a feminizing ACC, pre-surgical assessment allows for estrogen to be as a marker of tumor progression. A sex steroid producing ACC should be evaluated for cortisol secretion as prompt detection can avoid post-operative adrenal insufficiency.
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