Adrenocortical carcinomas: surgical trends and results of a 253-patient series from the French Association of Endocrine Surgeons study group
Abstract
Because of the rarity of adrenocortical carcinoma, survival rates and the prognosis for patients who have undergone operation are not well known. The purpose of the French Association of Endocrine Surgery was to evaluate these factors over an 18-year period. A trend study was associated to assess changes in the clinical and biochemical presentations as well as the surgical evolution. A total of 253 patients (158 women, 95 men) with a mean age of 47 years were included. Cushing syndrome was the main clinical presentation (30%), and hormonal studies revealed secreting tumors in 66% of the cases. Altogether, 72% (n = 182) of patients underwent resection for cure, and 41.5% (n = 105) of them had an extensive resection because of metastatic cancer. A lymphadenectomy was performed in 32.5% (n = 89) of the cases. The operative mortality was 5.5% (n = 14). Patients were given mitotane as adjuvant therapy in 53.8% of the cases (n = 135). The results of staging were stage I in 16 patients (6.3%), stage II (local disease) in 126 patients (49.8%), stage III (locoregional disease) in 57 patients (22.5%), and stage IV (metastases) in 54 patients (21.3%). Neither tumor staging nor the rate of curative surgery changed during the study period. More subcostal incisions were performed, and the use of mitotane increased significantly. The 5-year actuarial survival rates were 38% overall, 50% in the curative group, 66% for stage I, 58% for stage II, 24% for stage III, and 0% for stage IV. Multivariate analysis showed that mitotane benefited only the group of patients not operated on for cure. A better prognosis was found in patients operated on after 1988 (p = 0.04), in those with precursor-secreting tumors (p = 0.005), and in those at local stages of the disease (p = 0.0003). Thus mitotane benefited only patients not operated on for cure. Curative resection, precursor secretion, recent diagnosis, and local stage were favorably associated with survival.
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Between 40% and 60% of adrenocortical carcinomas are functional and may present with symptoms of hormonal hypersecretion.
“Between 40% and 60% of ACCs are functional and may present with symptoms of hormonal hypersecretion (126,127,128,129), most commonly with Cushing’s syndrome (estimated 45%).”
From Adrenal incidentaloma (2020) · cites this paper as [129]
Cushing's syndrome is the most common presentation of hormonal hypersecretion in functional adrenocortical carcinoma, estimated at 45%.
“most commonly with Cushing’s syndrome (estimated 45%).”
From Adrenal incidentaloma (2020) · cites this paper as [129]
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 [19]
These results closely correspond with surgical outcomes from other solid malignancies, in which a survival benefit is often seen after complete resection with negative margins but not in those with positive margins.
“These results closely correspond with the surgical outcomes from a variety of other solid malignancies, in which a survival benefit is often seen in patients after a complete resection with negative margins but not in those with positive margins (19, 91, 244, 267, 273–275).”
From Adrenocortical carcinoma (2014) · cites this paper as [19]
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Self-citation
- Authors
- Icard, P., Goudet, P., Charpenay, C., Andreassian, B., Carnaille, B., Chapuis, Y., Cougard, P., Henry, J., & Proye, C.
- Affiliations
- Centre Hospitalier Universitaire de Caen Normandie (FR)
- CHU Dijon Bourgogne (FR)
- Service de Chirurgie Thoracique Hôpital Bichat‐Beaujon 46 Rue Henri‐Huchard 75018 Paris France
- Hôpital Claude Huriez (FR)
- Hôpital Cochin (FR)
- Hôpital de la Timone (FR)
- Corresponding author
- Goudet, Pierre
- Venue
- World Journal of Surgery
- Publisher
- Wiley
- Published
- 2001-07
- Volume
- 25
- Issue
- 7
- Pages
- 891-897
- ISSN
- 0364-2313 (ISSN-L)
- 1432-2323
- OpenAlex
- W1991690093
- Cited by
- 543
- References
- 27
- Citations by year
- 2026: 7 · 2025: 11 · 2024: 14 · 2023: 11 · 2022: 18 · 2021: 29 · 2020: 28 · 2019: 19 · 2018: 32 · 2017: 27 · 2016: 25 · 2015: 37 · 2014: 38 · 2013: 23 · 2012: 32
- FWCI
- 7.26
- Type
- journal article
- Language
- en
- Open access
- closed
- Locations
- World Journal of Surgery (publishedVersion)
- PubMed (publishedVersion)
- Topics
- Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
- Health Sciences › Medicine › Epidemiology › Neuroendocrine Tumor Research Advances
- Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
- Keywords
- adrenocortical carcinoma, adrenalectomy, mitotane, adjuvant therapy, Cushing's syndrome, tumor staging, surgical resection, lymph node dissection, survival, prognosis, metastatic disease, surgical trends, subcostal incision, postoperative mortality, multivariate analysis, endocrine surgery
- MeSH terms
- Adolescent, Adrenal Cortex Neoplasms, Adult, Aged, Aged, 80 and over, Child, Child, Preschool, Diagnostic Techniques, Endocrine, Female, France, Humans, Male, Middle Aged, Mitotane, Neoplasm Staging, Predictive Value of Tests, Prognosis, Registries, Retrospective Studies, Endocrine Surgical Procedures, Survival Rate, Treatment Outcome, Adrenocortical Carcinoma, Antineoplastic Agents, Hormonal
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