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Extra-Adrenal, Non-Functional Adrenocortical Carcinoma Presenting With Acute Abdomen: A Case Report Publisher Pubmed



Mirsharifi A1 ; Vasei M2 ; Sadeghian E1 ; Ghorbaniabdehgah A1 ; Naybandi Atashi S3
Authors
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Authors Affiliations
  1. 1. Department of General Surgery, Research Center of Surgical Outcomes and Procedures, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  2. 2. Cell-Based Therapies Research Center, Digestive Disease Research Institute, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran
  3. 3. Department of Radiology, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran

Source: Journal of Medical Case Reports Published:2020


Abstract

Background: Adrenocortical carcinoma is a rare malignancy, with 43% being non-functional. These may arise from adrenal rest anywhere in the embryonic pathway of the adrenal glands. In the context of extra-adrenal and retroperitoneal tumors, the exact pathologic diagnosis is challenging. The case reported here, to the best of our knowledge, is the seventh reported case of extra-adrenal non-functional adrenocortical carcinoma. Case presentation: We report a case of extra-adrenal non-functional adrenocortical carcinoma in a 15-year-old Persian boy who presented with an acute abdomen. He underwent surgical resection. Pathologic findings based on immunohistochemistry and cellular morphology confirmed adrenocortical carcinoma. He was treated with mitotane for 24 months. During a follow-up period of 30 months, no recurrence or metastases were found. Conclusion: Despite the rarity of extra-adrenal adrenocortical carcinoma, presentation with an acute abdomen may occur, and the tumor may be found anywhere in the adrenal embryonic pathway. On the other hand, tumor behavior and prognosis in children may be different from what we expect in adults. © 2020 The Author(s).