Spontaneously metachronous ruptures of adrenocortical carcinoma and its contralateral adrenal metastasis

Adrenocortical carcinomas (ACCs) are rare neoplasms. In spite of its rarity, ACCs are the second most lethal endocrine cancer after anaplastic thyroid carcinomas. Currently, the only chance for a cure is an early diagnosis and a radical surgical resection. We present the case of a previously unreported bilateral adrenal hemorrhage occurring in a 59-year-old Caucasian male who was admitted to our surgical division with the diagnosis of a right retroperitoneal spontaneous hemorrhage. Imaging revealed a 10-cm ruptured right adrenal mass with no other abdominal lesions, endocrine screening results were normal, and a right adrenalectomy was performed. Pathology revealed a ruptured ACC. The postoperative period was uneventful and the patient was discharged. While recovering, 3 weeks after the operation, the patient showed the same symptoms on the contralateral side. Imaging once again revealed a retroperitoneal hemorrhage due to a 5-cm ruptured left adrenal mass. Endocrine screening showed a frank peripheral hypercortisolism and imaging showed a huge metastatic dissemination to the liver, lungs, and retroperitoneal space. An urgent left adrenalectomy was performed and pathology showed a metastatic ruptured ACC. The patient was placed in substitutive therapy but never recovered and died of penta lobar pneumonia on postoperative day 31. An extensive review of the current literature on the issue was performed. ACC is confirmed to be a lethal cancer. Rupture is the rarest clinical presentation and appears to be caused by the tumor’s growth rate more than the tumor dimensions itself. The use of endocrine screening on such hemodynamically unstable patients is questionable.