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Andrenocortical Carcinomas: Twelve-year Prospective Experience

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Abstract

Adrenocortical carcinoma (AC) is a rare tumor with poor prognosis. Twenty-two patients (14 F, 8 M; age 22 to 59 years; median, 43 years) with AC were evaluated prospectively in a single center: tumor stage was I–II in 12 cases and III–IV in 10. The overall survival in our cohort was 41.6 ± 42 months; 16 subjects are still alive. Curative surgery was followed by longer survival than debulking or no surgery (p < 0.0001). The first relapse was highly predictive for further recurrences. Recurrent ACs were progressively more aggressive, and they occurred with variable but ever shorter intervals. At diagnosis, 14 patients (63.5%) presented with features of clear adrenocortical hyperactivity. Despite the absence of clinical signs of hormonal excess, all other patients presented some abnormalities of steroid secretion. The most common clinical finding was a recent diagnosis of moderate-to-severe hypertension (68%), poorly controlled by pharmacological treatment, often associated with multiple cardiovascular risk factors. High mitotic rate and undifferentiated polymorph cellular pattern were associated with worse prognosis. Response to treatments other than surgery (mitotane chemotherapy) was better in patients treated early after the first surgery. In conclusion, curative surgery was the most effective treatment. Monitoring arterial pressure, endocrine parameters, and metabolic parameters can be helpful for the early detection of AC recurrences.

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Acknowledgments

The authors thank the many health professionals who cared for the patients with adrenocortical carcinoma over these years. They are also indebted to Dr Melania Pulcrano for organizing the database, Prof. Fimmanò, and Dr. Nicola Gennarelli for their help in the surgical care of some patients, and Mr. Angelo Di Francia for his skilled technical support.

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Authors and Affiliations

  1. Department of Molecular and Clinical Endocrinology and Oncology, “Federico II” University of Naples, Via S. Pansini 5, 80131, Naples, Italy

    Libuse Tauchmanovà M.D., Ph.D., Annamaria Colao M.D, Ph.D., Annalisa Rossi M.D., Giovanna Palmieri M.D., Gaetano Lombardi M.D., Ph.D. & Riccardo Rossi M.D.

  2. Department of Molecular and Clinical Endocrinology and Oncology, Section of Endocrine and General Surgery,, “Federico II” University of Naples, Via S. Pansini 5, 80131, Naples, Italy

    Luigi Antonio Marzano M.D. & Ettore Marzano M.D.

  3. Department of Anatomical Pathology and Cytology, “Federico II” University of Naples, Via S. Pansini 5, 80131, Naples, Italy

    Lucianna Sparano M.D. & Guido Pettinato M.D., Ph.D.

  4. Department of Biomorphological and Functional Sciences, “Federico II” University of Naples, Via S. Pansini 5, 80131, Naples, Italy

    Luigi Camera M.D. & Marco Salvatore M.D., Ph.D.

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  1. Libuse Tauchmanovà M.D., Ph.D.
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  2. Annamaria Colao M.D, Ph.D.
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  3. Luigi Antonio Marzano M.D.
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  4. Lucianna Sparano M.D.
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  5. Luigi Camera M.D.
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  6. Annalisa Rossi M.D.
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  7. Giovanna Palmieri M.D.
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  8. Ettore Marzano M.D.
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  9. Marco Salvatore M.D., Ph.D.
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  10. Guido Pettinato M.D., Ph.D.
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  11. Gaetano Lombardi M.D., Ph.D.
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  12. Riccardo Rossi M.D.
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Correspondence to Libuse Tauchmanovà M.D., Ph.D..

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Tauchmanovà, L., Colao, A., Marzano, L. et al. Andrenocortical Carcinomas: Twelve-year Prospective Experience. World J. Surg. 28, 896–903 (2004). https://doi.org/10.1007/s00268-004-7296-5

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