The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane

• Published in: Endocrine-related cancer Vol. 17; no. 1; pp. 265 - 272
• Main Authors: Wängberg, B, Khorram-Manesh, A, Jansson, S, Nilsson, B, Nilsson, O, Jakobsson, C E, Lindstedt, S, Odén, A, Ahlman, H
• Format: Journal Article
• Language: English
• Published: England Society for Endocrinology 01-03-2010; BioScientifica
• Subjects: Adrenal Cortex Neoplasms - drug therapy; Adrenal Cortex Neoplasms - mortality; Adrenal Cortex Neoplasms - surgery; Adrenocortical Carcinoma - drug therapy; Adrenocortical Carcinoma - mortality; Adrenocortical Carcinoma - surgery; Adult; Aged; Aged, 80 and over; Algorithms; Antineoplastic Agents, Hormonal - therapeutic use; Combined Modality Therapy; Endocrine Surgical Procedures - methods; Female; Humans; Male; MEDICAL AND HEALTH SCIENCES; MEDICIN OCH HÄLSOVETENSKAP; Middle Aged; Mitotane - therapeutic use; Monitoring, Physiologic; Regular papers; Survival Analysis; Survivors - statistics & numerical data; Time Factors; Young Adult
• ISSN: 1351-0088; 1479-6821; 1479-6821
• DOI: 10.1677/ERC-09-0190

Adrenocortical carcinoma (ACC) is a rare tumour disease with sinister prognosis also after attempts to radical surgery; better prognosis is seen for low-stage tumours. Adjuvant treatment with the adrenolytic drug mitotane has been attempted, but not proven to prevent from recurrence. The drug may offer survival advantage in case of recurrence. The aim of this single-centre study (1979–2007) of 43 consecutive patients was to evaluate the long-term survival after active surgical treatment combined with monitored mitotane (to reduce side effects of the drug). The series is unique, since all patients were offered a period of mitotane as adjuvant or palliative treatment; six patients refused mitotane. Despite a high proportion of high-stage tumours (67%), the complete resection rate was high (77%). The disease-specific 5-year survival was high (64.1%); very high for patients with low-stage tumours without evident relation to mitotane levels. Patients with high-stage tumours had a clear survival advantage with mitotane levels above a threshold of 14 mg/l in serum. The hazard ratio for patients with high mitotane levels versus all patients indicates a significant effect of the drug. The results indicate that adjuvant mitotane may be the standard of care for patients with high-stage ACC after complete resection.