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Clinical Features of Adrenocortical Carcinoma, Prognostic Factors, and the Effect of Mitotane Therapy
Open accessAbstract
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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In the citing paper
“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 the citing paper
“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]
In the citing paper
“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]
In the citing paper
“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]
In the citing paper
“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]
In the citing paper
“Patients with ACC only rarely present with classical tumor symptoms, such as cachexia or night sweats (3, 10).”
From Adrenocortical carcinoma (2014) · cites this paper as [10]
In the citing paper
“Biochemically or clinically apparent adrenocortical hormone production is evident in up to 45% to 70% (9, 10, 85).”
From Adrenocortical carcinoma (2014) · cites this paper as [10]
In the citing paper
“Hypercortisolism is the most common presentation of patients presenting with hormone excess (50%–80% of hormone-secreting ACCs), causing classic symptoms including plethora, diabetes mellitus, muscle weakness/atrophy, and osteoporosis.”
From Adrenocortical carcinoma (2014) · cites this paper as [10]
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Metadata
- 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
- 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
- bronze
- doi.org
- 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 › Oncology › 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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