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Cited by 717Adrenal and Paraganglionic TumorsAdrenal Hormones and DisordersNeuroendocrine Tumor Research Advances

Clinical features of adrenocortical carcinoma, prognostic factors, and the effect of mitotane therapy

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Luton, Jean-Pierre, Cerdas, Sonia, Billaud, Line, Thomas, Guy, Guilhaume, Brigitte, Bertagna, Xavier, Laudat, Marie-Hélène, Louvel, Albert +4 more·1990·New England Journal of Medicine
DOI 10.1056/nejm199004263221705
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Abstract

Adrenocortical carcinoma is a rare tumor, and only limited information is available about its natural history and the effects of therapy. We studied 105 patients (75 female and 30 male; mean age, 46 years) with adrenocortical carcinoma who were referred to us between 1963 and 1987. The average duration of symptoms before diagnosis was 8.7 months. At the time of diagnosis, 68 percent of the patients had endocrine symptoms, and 30 percent had distant metastases. Hormonal studies showed that 79 percent of the tumors were functional. Eighty patients underwent surgery, and 59 also received the adrenal cytotoxic agent mitotane. The median disease-free interval after surgery was 12.1 months (range, 1 to 175). Tumor dissemination occurred in 82 percent of the patients, most commonly to the lung, liver, and adjacent organs. The median survival time was 14.5 months (range, less than 1 to 175), and the five-year survival was 22 percent. Age over 40 years and the presence of metastases at the time of diagnosis were the only factors recognized as indicating a poor prognosis. Mitotane controlled hormonal secretion in 75 percent of the patients. Eight mitotane-treated patients had partial tumor regression, but the drug did not have a significant effect on survival. We conclude that adrenocortical carcinoma carries a poor prognosis. Mitotane therapy may offer transient benefits, particularly in controlling endocrine symptoms.

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  • A large single center series in France reported a median age of 46 years, consistent with the German registry.

    “This is in accordance with a median age of 46 years in a large single center series in France (10).”

    From Adrenocortical carcinoma (2014) · cites this paper as [10]

  • In both adult and pediatric populations, there is a predilection for the female gender, with a female-to-male ratio ranging from 1.5-2.5:1.

    “In the adult as well as in the pediatric population, there is a predilection for the female gender (the ratio of female to male ranges from 1.5–2.5:1) (10, 17).”

    From Adrenocortical carcinoma (2014) · cites this paper as [10]

  • A role of estrogens in ACC has been suggested by the observation of a probable relative increase of diagnosis during pregnancy.

    “A role of estrogens has also been suggested by the observation of a probable relative increase of diagnosis of ACC during pregnancy (10, 19).”

    From Adrenocortical carcinoma (2014) · cites this paper as [10]

  • For 40% to 60% of ACC patients, the major presenting complaints are symptoms and signs of hormone excess.

    “For 40% to 60% of patients, the major presenting complaints are symptoms and signs of hormone excess (3, 9, 10).”

    From Adrenocortical carcinoma (2014) · cites this paper as [10]

  • Another third of ACC patients present with nonspecific symptoms due to local tumor growth, such as abdominal or flank pain, abdominal fullness, or early satiety.

    “Another third present with nonspecific symptoms due to local tumor growth, such as abdominal or flank pain, abdominal fullness, or early satiety (9, 10).”

    From Adrenocortical carcinoma (2014) · cites this paper as [10]

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Cites

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3 of the 67 references couldn’t be matched to a known paper.

Research works printed but not linked: 23

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Authors
Luton, J.-P., Cerdas, S., Billaud, L., Thomas, G., Guilhaume, B., Bertagna, X., Laudat, M.-H., Louvel, A., Chapuis, Y., Blondeau, P., Bonnin, A., & Bricaire, H.
Affiliations
Hôpital Cochin (FR)
Venue
New England Journal of Medicine
Publisher
Massachusetts Medical Society
Published
1990-04-26
Volume
322
Issue
17
Pages
1195-1201
ISSN
  • 0028-4793 (ISSN-L)
  • 1533-4406
DOI
10.1056/nejm199004263221705
OpenAlex
W2336089418
Cited by
717
References
67
Citations by year
2026: 8 · 2025: 8 · 2024: 15 · 2023: 26 · 2022: 22 · 2021: 31 · 2020: 20 · 2019: 17 · 2018: 23 · 2017: 16 · 2016: 19 · 2015: 27 · 2014: 25 · 2013: 19 · 2012: 31
FWCI
12.17
Type
journal article
Language
en
Open access
Locations
  • New England Journal of Medicine (publishedVersion)
  • PubMed (publishedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
  • Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
Keywords
adrenocortical carcinoma, mitotane, prognostic factors, adrenal surgery, metastasis, survival, endocrine symptoms, tumor regression, adrenocortical function, age over 40
MeSH terms
Adrenal Cortex Hormones, Adrenal Cortex Neoplasms, Combined Modality Therapy, Female, Follow-Up Studies, Humans, Male, Middle Aged, Mitotane, Neoplasm Metastasis, Prognosis, Retrospective Studies, Survival Rate

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