title: The long-term survival in adrenocortical carcinoma with active surgical management and use of monitored mitotane.
abstract: 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.
authors.affiliation: Department of Surgery, Sahlgrenska University Hospital, University of Gothenburg, 413 45 Gothenburg, Sweden.
authors.forename: B
authors.initials: B
authors.lastname: Wängberg
authors.forename: A
authors.initials: A
authors.lastname: Khorram-Manesh
authors.forename: S
authors.initials: S
authors.lastname: Jansson
authors.forename: B
authors.initials: B
authors.lastname: Nilsson
authors.forename: O
authors.initials: O
authors.lastname: Nilsson
authors.forename: C E
authors.initials: CE
authors.lastname: Jakobsson
authors.forename: S
authors.initials: S
authors.lastname: Lindstedt
authors.forename: A
authors.initials: A
authors.lastname: Odén
authors.forename: H
authors.initials: H
authors.lastname: Ahlman
mesh_terms: D000306:Adrenal Cortex Neoplasms / Q000188:drug therapy* / Q000401:mortality* / Q000601:surgery*; D018268:Adrenocortical Carcinoma / Q000188:drug therapy* / Q000401:mortality* / Q000601:surgery*; D000328:Adult; D000368:Aged; D000369:Aged, 80 and over; D000465:Algorithms; D018931:Antineoplastic Agents, Hormonal / Q000627:therapeutic use; D003131:Combined Modality Therapy; D013507:Endocrine Surgical Procedures / Q000379:methods; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D008939:Mitotane / Q000627:therapeutic use*; D008991:Monitoring, Physiologic; D016019:Survival Analysis; D017741:Survivors / Q000706:statistics & numerical data; D013997:Time Factors; D055815:Young Adult
chemical_list: D018931:Antineoplastic Agents, Hormonal; D008939:Mitotane