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The
nd Annual Meeting of March 27-28, 2021 The Endocrine Society and The Diabetes Association of the R.O.C. (Taiwan)
PE01
ECTOPIC ADRENOCORTICAL CARCINOMA IN THE OVARY: A CASE REPORT 1
WEN-HSUAN TSAI, 2TZE-CHIEN CHEN, 3SHUEN-HAN DAI, 1YI-HONG ZENG
1
Division of Endocrinology and Metabolism, Department of Internal Medicine, Mackay Memorial Hospital, Taipei, Taiwan (ROC); 2 Department of Obstetrics and Gynecology, Mackay Memorial Hospital, Taipei, Taiwan (ROC); 3 Department of Pathology, Mackay Memorial Hospital, Taipei, Taiwan (ROC)
Adrenocortical carcinoma (ACC) is a rare malignancy with an incidence of 0.7–2.0 cases/ million habitants/year. Ectopic adrenal adenomas are uncommon and ectopic ACCs are rarely seen. We presented a 59-year-old woman who suffered from abdominal fullness for 6 months and increased abdominal circumstance. A huge pelvic tumor was noted. She received debulking operation. Initial diagnosis was ovarian neuroendocrine carcinoma with omentum, lymph nodes, lung and peritoneum metastasis. Pathology report revealed local tumor necrosis and prominent lympho-vascular invasion. The Ki-67 proliferation labeling index is very high > 50%. Immunohistochemically, tumor cells are focally positive for CD56, synaptophysin but also focally positive for calretinin. She received 3 cycles of etoposide, cisplatin and bevacizumab. 2 months later, pathologist confirmed diagnosis of ectopic ACC by positivity for synaptophysin, alpha-inhibin, TFE-3, calretinin (focal), and CD56 (focal), and high Ki-67-labeling proliferating index (> 80%). Non-functioning tumor was diagnosed according to hormone profile. She received mitotane 6 months after operation but died of cachexia 3 months later. There was limited knowledge of the incidence and prognosis of ectopic ACC. Diagnosing ACC from ectopic organ can be tricky and comprehensive IHC may shed light on the correct diagnosis.
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