Extra-adrenal, non-functional adrenocortical carcinoma presenting with acute abdomen: a case report

Alireza Mirsharifi,Ali Ghorbani-Abdehgah,Mohammad Vasei,Ehsan Sadeghian,Sara Naybandi Atashi

doi:10.1186/s13256-020-02408-7

Alireza Mirsharifi, Ali Ghorbani-Abdehgah + Show 3 more

Open Access

https://doi.org/10.1186/s13256-020-02408-7

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Abstract

BackgroundAdrenocortical 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 presentationWe 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.ConclusionDespite 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.

Highlights

ConclusionDespite 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
Adrenocortical carcinoma (ACC) is a rare malignancy with an annual incidence of 1–2 in one million [1, 2]
Various hormone oversecretions can be detected in 40–60% of ACCs, leading to a wide range of clinical presentations [1]

Summary

Conclusion

ACCs may present with an acute abdomen. This tumor may be found anywhere in the adrenal embryonic pathway. It has been suggested to start mitotane for cases with lymph node metastasis, it seems to be rational to prescribe it in ruptured ACCs even with no lymph node involvement or in extra-adrenal ACCs. Malignant features of adrenocortical tumors in histopathologic findings should be interpreted cautiously in pediatric tumors, because adult malignancy criteria may not be applicable in children

Introduction

Discussion

Findings

Hilum of left kidney cm Radical nephrectomy