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Cited by 446Adrenal and Paraganglionic TumorsAdrenal Hormones and DisordersNeuroendocrine Tumor Research Advances

Clinical and biological features in the prognosis of adrenocortical cancer: poor outcome of cortisol-secreting tumors in a series of 202 consecutive patients

Abiven, Gwenaelle, Coste, Joel, Groussin, Lionel, Anract, Philippe, Tissier, Frédérique, Legmann, Paul, Dousset, Bertrand, Bertagna, Xavier +1 more·2006·The Journal of Clinical Endocrinology & Metabolism
DOI 10.1210/jc.2005-2730
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Abstract

CONTEXT: Adrenocortical carcinomas (ACC) are rare tumors with a poor prognosis. Few reports concerning large and homogeneous series are available. OBJECTIVE: We aimed to determine the clinical characteristics and outcome of ACC and to identify prognostic factors. DESIGN AND SETTING: This study is a descriptive and multivariate analysis of a cohort from a single endocrinology center. PATIENTS: A total of 202 consecutive patients with ACC were studied. RESULTS: The sex ratio (female to male) was 2.7. Mean age at diagnosis was 44 +/- 16 yr (range, 11-88 yr). We found that 154 patients (76%) had hypersecreting tumors [mostly cortisol and androgens (47%), cortisol alone (27%), or androgens alone (6%)] and 43 patients (21%) had metastases at diagnosis. At initial staging or during follow-up, 85 patients (42%) had liver metastases, 79 patients (39%) had lung metastases, and 20 patients had bone metastases (10%). The survival rate was 37% at 5 yr. Multivariate analysis identified the following independent prognostic factors associated with shorter survival: older age at diagnosis [hazard ratio (HR), 1.03; P < 0.0001], initial MacFarlane extension stages 3 (HR, 4.42; P = 0.005) and 4 (HR, 7.93; P < 0.0001), and cortisol hypersecretion (HR, 3.90; P < 0.0001). Treatment with 1,1-dichlorodiphenildichloroethane (o,p'DDD) in the 3 months after surgery increased the survival rate of patients with cortisol-secreting tumors (HR, 0.40; P = 0.04). CONCLUSION: This study highlights the better prognosis of ACC diagnosed at a noninvasive local stage, the particularly poor prognosis of patients with cortisol-secreting tumors, and the beneficial effect of o,p'DDD therapy in this subgroup of patients.

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  • The impact of the functional status and clinical features of adrenocortical carcinomas on survival is unclear.

    “The impact of the functional status and clinical features of ACCs on survival is unclear (132,133).”

    From Adrenal incidentaloma (2020) · cites this paper as [133]

  • Biochemically or clinically apparent adrenocortical hormone production is evident in up to 45% to 70% of ACC patients.

    “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 [85]

  • Autonomous aldosterone secretion is rare in ACC.

    “Autonomous aldosterone secretion (which classically leads to hypertension and hypokalemia) is rare in ACC (85, 86).”

    From Adrenocortical carcinoma (2014) · cites this paper as [85]

  • At the time of presentation, ACCs are generally large tumors, measuring on average 10 to 13 cm.

    “At the time of presentation, ACCs are generally large tumors, measuring on average 10 to 13 cm (9, 85, 89).”

    From Adrenocortical carcinoma (2014) · cites this paper as [85]

  • Hypercortisolism is the most common presentation of patients presenting with hormone excess, accounting for 50%-80% of hormone-secreting ACCs.

    “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 [85]

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Authors
Abiven, G., Coste, J., Groussin, L., Anract, P., Tissier, F., Legmann, P., Dousset, B., Bertagna, X., & Bertherat, J.
Affiliations
  • Hôpital Cochin (FR)
  • Centre National de la Recherche Scientifique (FR)
ORCID
Venue
The Journal of Clinical Endocrinology & Metabolism
Publisher
The Endocrine Society
Published
2006-07
Volume
91
Issue
7
Pages
2650-2655
ISSN
  • 0021-972X (ISSN-L)
  • 1945-7197
DOI
10.1210/jc.2005-2730
OpenAlex
W2140991656
Cited by
435
References
34
Citations by year
2026: 12 · 2025: 12 · 2024: 14 · 2023: 23 · 2022: 20 · 2021: 30 · 2020: 25 · 2019: 23 · 2018: 33 · 2017: 17 · 2016: 23 · 2015: 32 · 2014: 31 · 2013: 21 · 2012: 18
FWCI
15.59
Type
journal article
Language
en
Open access
closed
Locations
  • The Journal of Clinical Endocrinology & Metabolism (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, prognosis, prognostic factors, survival, liver metastasis, lung metastasis, age at diagnosis, mitotane, adjuvant chemotherapy, androgen-secreting tumor
MeSH terms
Adrenal Cortex Neoplasms, Adult, Analysis of Variance, Androgens, Bone Neoplasms, Female, Humans, Hydrocortisone, Liver Neoplasms, Lung Neoplasms, Male, Middle Aged, Mitotane, Neoplasm Staging, Prognosis, Survival Rate, Chemotherapy, Adjuvant, Antineoplastic Agents, Hormonal

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