Abstract

Abstract Description Adrenocortical carcinoma (ACC) is a rare and aggressive endocrine malignancy with an incidence of 0.7–2.0 cases/million/year. We present a case of ACC that we followed for a long period of approximately 5.5 years. Clinical Case A 50-year-old female patient was referred to our hospital because of a left adrenal mass. She had no complaints except left flank pain. There were no features on her resume. There were no findings related to hypercortisolemia on physical examination. Hormone levels were examined (Table 1). In the upper abdomen tomography, a 9×13×10.8 cm sized, sharply defined, lobulated contoured, heterogeneous-enhancing mass lesion with central cystic components was observed in the left adrenal region (Figure 1). The patient underwent left adrenalectomy and left partial nephrectomy. Pathology was reported as adrenocortical carcinoma. Weis score was 6/9, and the Ki-67 index was 15-20%. She was evaluated as a stage 2 disease according to ENSAT staging and followed up with thoracoabdominal CT. A 30*15 mm recurrent mass lesion was detected in the first-year postoperative follow-up. The patient underwent implant excision, left nephrectomy, and left paraaortic lymphadenectomy. Then the patient was given 6 cycles of Cisplatin-Etoposide-Adriamycin chemotherapy. On follow-up imaging after chemotherapy, a mass of approximately 13 mm in diameter was detected adjacent to the small intestine. Bridectomy and malignant soft tissue resection were performed on the patient. Mitotane treatment was started after the operation. After 6 weeks, the target mitotane level was reached. The patient was followed closely for glucocorticoid needs. Oral hydrocortisone treatment was started in the patient who complained of fatigue and nausea in the 8th month of mitotan. Since there was no hyponatremia/hyperkalemia and clinically no hypotension/orthostatic hypotension, mineralocorticoid therapy was not initiated. While under mitotane treatment, liver function tests, thyroid function tests, complete blood count and lipid parameters were evaluated intermittently. She was hospitalized 4 times in total with the clinic of infection and adrenal insufficiency. At that time, mitotane treatment was interrupted and parenteral steroid treatment was started. The patient received 4.5 years of mitotane therapy. No recurrence was detected in the follow-up scans during mitotane treatment and 6 months after the treatment was discontinued. PET-CT imaging was performed in the 1st year while the patient was in the follow-up without treatment. In the retroperitoneal region, 38×21 mm focal increased activity involvement was observed (SUV Max: 17.82). Left hemicolectomy operation was performed on the patient and chemotherapy was planned. Conclusion In conclusion, we presented a case that we followed for a long time without recurrence under mitotane treatment. It can be thought that mitotane treatment keeps ACC disease under control. Figure 1. 9×13×10.8 cm left adrenal mass. Table 1

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