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Cited by 247Adrenal and Paraganglionic TumorsRenal cell carcinoma treatmentAdrenal Hormones and Disorders

The Italian registry for adrenal cortical carcinoma: Analysis of a multiinstitutional series of 129 patients

Crucitti, Francesco, Bellantone, Rocco, Ferrante, Angela, Boscherini, Mauro, Crucitti, Pierfilippo·1996·Surgery
DOI 10.1016/s0039-6060(96)80164-4
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Abstract

Background Adrenal cortical carcinoma is an uncommon tumor with a poor prognosis. The low incidence of this tumor makes it difficult to achieve reliable data on clinical manifestations, natural history, and the impact of therapies. The purpose of this study was to evaluate such aspects in a large series.Methods A retrospective series of 129 cases (55 men and 74 women, mean age of 49 years) was collected from 18 surgical institutions. At the time of diagnosis 45.7% of patients had endocrine symptoms. One hundred twenty-four patients underwent surgery, which was considered curative in 91 cases and palliative in 33. Sixty-three patients had local disease, 48 had regional disease, and 43 had distant metastases.Results This study confirmed a higher incidence in the 40- to 50-year-old population with a female prevalence; hormonal hyperincretion was more common in women, but it was not caused by advanced disease. The overall 5-year survival rate was 35%. Tumor stage and curative resection affected prognosis significantly. The influence of gender, side, age, and hormonal function has not been confirmed. Adjuvant therapies were ineffective in prolonging survival. Reoperated patients experienced better survival (mean, 41.5 months) than nonreoperated cases (mean, 15.6 months).Conclusions The poor prognosis of adrenal cortical carcinoma may be improved by early diagnosis and complete resection. Radical surgery is the sole effective therapy, particularly in early stages. Surgical treatment of recurrence seems to improve survival and should be attempted systematically. Adjuvant therapies obtained contrasting results, and their role should be evaluated in prospective multicentric trials.

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Crossref · 10.1016/s0039-6060(96)80164-4

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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 [128]

  • 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 [128]

  • 1.3% of all childhood cancers are ACCs, as opposed to 0.02% to 0.2% of adult cancers, confirming a higher relative incidence early in life.

    “The fact that 1.3% of all childhood cancers are ACCs as opposed to 0.02% to 0.2% of adult cancers confirms a higher relative incidence early in life (12, 15–17).”

    From Adrenocortical carcinoma (2014) · cites this paper as [16]

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Authors
Crucitti, F., Bellantone, R., Ferrante, A., Boscherini, M., & Crucitti, P.
Affiliations
Università Cattolica del Sacro Cuore (IT)
Corresponding author
Ferrante, Angela
ORCID
Venue
Surgery
Publisher
Elsevier BV
Published
1996-02
Volume
119
Issue
2
Pages
161-170
ISSN
0039-6060 (ISSN-L)
DOI
10.1016/s0039-6060(96)80164-4
OpenAlex
W2095310757
Cited by
247
References
66
Citations by year
2026: 1 · 2025: 6 · 2024: 7 · 2023: 8 · 2022: 6 · 2021: 12 · 2020: 5 · 2019: 10 · 2018: 4 · 2017: 8 · 2016: 5 · 2015: 19 · 2014: 14 · 2013: 7 · 2012: 17
FWCI
3.74
Type
journal article
Language
en
Open access
closed
Licence
www.elsevier.com/tdm/userlicense/1.0/ (tdm)
Locations
  • Surgery (publishedVersion)
  • PubMed (publishedVersion)
  • CINECA IRIS Institutial Research Information System (University of Genoa) (submittedVersion)
Topics
  • Health Sciences › Medicine › Surgery › Adrenal and Paraganglionic Tumors
  • Health Sciences › Medicine › Pulmonary and Respiratory Medicine › Renal cell carcinoma treatment
  • Health Sciences › Medicine › Endocrinology, Diabetes and Metabolism › Adrenal Hormones and Disorders
Keywords
adrenocortical carcinoma, prognosis, survival, adrenalectomy, curative resection, tumor stage, metastasis, adjuvant therapy, reoperation, endocrine symptoms, natural history (disease)
MeSH terms
Actuarial Analysis, Adolescent, Adrenal Cortex Neoplasms, Adrenalectomy, Adult, Age Factors, Aged, Aged, 80 and over, Child, Female, Humans, Italy, Male, Middle Aged, Neoplasm Staging, Palliative Care, Prognosis, Radiotherapy, Radiotherapy Dosage, Recurrence, Registries, Time Factors, Incidence, Survival Rate, Chemotherapy, Adjuvant

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