Recurrent oncocytic adrenocortical carcinoma: implementing diagnostic criteria in a case report with the longest survival

Introduction: Oncocytic adrenocortical tumors represent a subtype of the adrenal cortex neoplasms. These tumors can be divided into oncocytomas, oncocytic neoplasms of uncertain malignancy and carcinomas (OACs). To date, only 34 cases of OAC have been reported. Case experience: We reported a case of a 54-year-old male patient with a history of laparoscopic right adrenalectomy for an OAC and subsequent chemotherapy for a recurrence. He was referred to our emergency room for diffuse abdominal pain, vomit, change in bowel habits, fever, asthenia. He underwent a laparotomy and a complete excision of the known bilobate lesion. The histopathological findings matched the features of a recurrent OAC. No chemotherapy was administered after surgery and the patient was disease-free after a follow-up of twenty-eight months. Results - discussion: The most questionable issue in treating adrenocortical oncocytic neoplasms is the determination of malignancy. According to the Helsinki Score, which is the best prognostic system, the primary lesion was an OAC. We also implemented the score systems to the recurrent lesion, that seemed to be malignant. We believe that the adjuvant treatment can delay a recurrent lesion development, but finally, radical surgical excision is necessary. Moreover, we reported the longest survival after the primary adrenalectomy. Conclusions: This study described the first case of recurrent oncocytic adrenocortical carcinoma with the longest followup. Adrenocortical oncocytoma is an extremely rare tumor of the adrenal gland with variable biological behavior without definitive consensus about diagnostic criteria. This was also the first case in which different histopathological criteria have been implemented in a recurrence. Key words: Oncocytic adrenocortical carcinoma, Surgical oncology, Survival.