Department of General Surgery, Research Center of Surgical Outcomes and Procedures, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
Cell-Based Therapies Research Center, Digestive Disease Research Institute, Shariati Hospital, Tehran University of Medical Sciences, Tehran, Iran.
J Med Case Rep. 2020 Jul 8;14(1):107. doi: 10.1186/s13256-020-02408-7.
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.
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.
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.
肾上腺皮质癌是一种罕见的恶性肿瘤，其中 43%为无功能性肿瘤。这些肿瘤可能起源于肾上腺胚胎发育过程中任何部位的肾上腺残余组织。在发生于肾上腺外和腹膜后肿瘤的情况下，准确的病理诊断具有挑战性。据我们所知，本报告所描述的病例是第七例报道的肾上腺外无功能性肾上腺皮质癌。
我们报告了一例发生于 15 岁的波斯男孩的肾上腺外无功能性肾上腺皮质癌病例，该患者以急性腹痛为首发表现。他接受了手术切除。基于免疫组化和细胞形态学的病理发现证实为肾上腺皮质癌。他接受了为期 24 个月的米托坦治疗。在 30 个月的随访期间，未发现复发或转移。
尽管肾上腺外肾上腺皮质癌较为罕见，但可能会以急性腹痛为首发表现，且肿瘤可能出现在肾上腺胚胎发育过程中的任何部位。另一方面，儿童的肿瘤行为和预后可能与我们在成人中所预期的不同。
