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Adrenocortical carcinoma: surgery and mitotane for treatment and steroid profiles for follow-up.

Journal article
Authors Amir Khorram-Manesh
Håkan Ahlman
Svante Jansson
Bo Wängberg
Ola Nilsson
C E Jakobsson
B. Eliasson
Sven Lindstedt
Lars-Eric Tisell
Published in World journal of surgery
Volume 22
Issue 6
Pages 605-11; discussion 611-2
ISSN 0364-2313
Publication year 1998
Published at Institute of Surgical Sciences
Institute of Laboratory Medicine, Dept of Pathology
Institute of Laboratory Medicine, Dept of Clinical Chemistry/Transfusion Medicine
Institute of Surgical Sciences, Department of Surgery
Pages 605-11; discussion 611-2
Language en
Links www.ncbi.nlm.nih.gov/entrez/query.f...
Keywords 17-Hydroxycorticosteroids, urine, 17-Ketosteroids, urine, Adrenal Cortex Neoplasms, drug therapy, surgery, therapy, urine, Adult, Aged, Antineoplastic Agents, Hormonal, administration & dosage, therapeutic use, urine, Combined Modality Therapy, Disease-Free Survival, Female, Follow-Up Studies, Humans, Male, Middle Aged, Mitotane, administration & dosage, therapeutic use
Subject categories Surgery

Abstract

Adrenocortical carcinoma (ACC) is a rare disease with a poor prognosis. It has been difficult to establish a strict treatment program for ACC, and better treatment alternatives and diagnostic tools must be sought. Even though surgery is the treatment of choice, the role of surgery in advanced disease has been questioned. Eighteen consecutive patients were treated at our unit over a 22-year period (1975-1997). All patients underwent surgery and were followed by our protocol, which includes urinary steroid profiles, clinical examinations, analysis of steroid hormones, and radiologic investigations. Twelve patients received mitotane with drug concentration measurements to deliver an effective, nontoxic dose. The median duration of mitotane treatment was 12 months. Few side effects were observed. Four patients with low-stage tumors underwent second-look operations with no pathologic findings. Five patients were subjected to repeat operations, and the mean duration of the disease-free interval before repeat surgery for these patients was 59 months. There was a significant positive correlation between the disease-free interval and the observed survival after repeat surgery. Eleven patients with intentionally curative surgery had their urinary steroid profiles tested several times postoperatively. For five patients preoperative urine samples were also available. Steroid profiles indicated recurrent disease despite normal radiologic findings in two of these five patients. The follow-up ranged from 6 weeks to 24 years. The predicted 5-year survival was 58% according to the Kaplan-Meier method. We conclude that monitoring serum concentrations of mitotane makes long-term treatment possible with few side effects; steroid profile analysis can be used for early detection of tumor recurrence; and repeat surgery for recurrence is of value for patients with long disease-free intervals.

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