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Feminizing estrogen-secreting adrenocortical carcinomas (ACCs) are exceedingly rare and carry a poor prognosis. The most common presenting trait is gynecomastia, but enlarged breasts are also a frequent clinical finding in healthy men. Biochemical evaluation may be challenging. As such, there is a high risk of delayed diagnosis and treatment opport...
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... confirmed bilateral gynecomastia with tender, firm mounds of tissue subareolar bilaterally, predominantly on the right side (Fig. 1). No palpable lymph nodes in the axillae, collum or groin were found. The testes had no palpable nodules and were of normal size, the right testicle slightly larger than the left, 5.3 × 1.6 × 3 vs 4.3 × 1.6 × 3 cm, confirmed by ultrasound. His weight was 78 kg and height was 179 cm (BMI 24.3 kg/m 2 ) with normal fat distribution. Blood ...
Citations
... A literature review of 21 cases of estrogen-secreting adrenal tumors in men published between 1970 and 2014 reported that 71% were malignant, and 2 cases of ACC were initially misclassified as adenoma, similar to our case (4). Since 2014, 5 additional cases of estrogen-secreting tumors in men were published, with 3 of 4 with optimal follow-up being malignant, and 1 case of reportedly benign adenoma that was followed for 6 months only (6)(7)(8)(9). Histopathology is considered the gold standard for diagnosing ACC. Guidelines recommend review of all suspected ACC specimens by an expert adrenal pathologist because of the frequent risk of misdiagnosis: 26 (9%) of cases were misdiagnosed in 1 study of 300 patients with ACC and in another study of 161 patients with ACC, initial diagnosis was revised by reference pathologist in 21 (13%) patients (10). ...
Estrogen-secreting adrenocortical carcinoma (ACC) is exceedingly rare, representing 1% to 2% of all ACCs. We present a case of a 65-year-old man diagnosed with an estrogen-secreting, 4.3-cm right adrenal mass discovered during work-up for bilateral gynecomastia. Gynecomastia and hyperestrogenism resolved after laparoscopic adrenalectomy, and pathology was reported as adrenocortical adenoma. However, 5 years later, he again developed bilateral gynecomastia because of recurrent hyperestrogenism. Imaging revealed multiple metastases in the abdomen. Urine steroid profiling demonstrated increased androgen precursors, androgen metabolites, and glucocorticoid precursors. Ultrasound-guided biopsy of one of the metastases confirmed ACC. Initial therapy included debulking surgery with removal of metastatic lesions. Mitotane therapy was initiated 4 weeks later along with hydrocortisone for anticipated mitotane-induced adrenal insufficiency. Histopathology from the adrenalectomy specimen 5 years earlier was rereviewed and confirmed ACC. Estrogen-secreting adrenal tumors are exceedingly rare, and the majority are malignant. This case underlines the importance of making an initial accurate diagnosis of adrenal malignancy that allows better surgical planning and appropriate monitoring. Indeterminate imaging characteristics of the adrenal mass, as well as the presentation with estrogen excess, suggested an elevated risk for ACC. Initial pathology-based misdiagnosis illustrates the need for an expert adrenal pathologist to review these rare tumors.
