Evidence map›Paper›PMID 40014786›Full record

ArticleRadiology and oncology2025

Management of adrenocortical carcinoma in Slovenia: a real-life analysis of histopathologic markers, treatment patterns, prognostic factors, and survival.

Urska Bokal, Jera Jeruc, Tomaz Kocjan, Metka Volavsek, Janja Jerebic, Matej Rakusa, Marina Mencinger

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Article in Radiology and oncology, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Not yet cited in PubMed.

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1 · What the graph read from it

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2 · The registry

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3 · Its place in the literature

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4 · The record

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5 · Who and what money

Authors and funding

7 authors.

Urska Bokal1Department of Medical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia.
Jera Jeruc2Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Tomaz Kocjan3Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Metka Volavsek2Institute of Pathology, Faculty of Medicine, University of Ljubljana, Ljubljana, Slovenia.
Janja Jerebic5Department of Methodology, Faculty of Organizational Sciences, University of Maribor, Kranj, Slovenia.
Matej Rakusa3Department of Endocrinology, Diabetes and Metabolic Diseases, University Medical Centre Ljubljana, Ljubljana, Slovenia.
Marina Mencinger1Department of Medical Oncology, Institute of Oncology Ljubljana, Ljubljana, Slovenia.

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

backgroundAdrenocortical carcinoma (ACC) is a rare cancer that presents significant diagnostic and therapeutic challenges. We analyzed the management and estimated survival of ACC patients in Slovenia over a 17-year period. PATIENTS AND

methodsPatients registered in the National Cancer Registry and treated from 2000 to 2017 were included. The survival and prognostic factors were assessed using the Kaplan-Meier method and Cox regression, respectively.

resultsForty-eight patients were included in our analysis. At the time of diagnosis, 6%, 42%, 25% and 27% had stage according European Network for the Study of Adrenal Tumors (ENSAT) I, II, III and IV, respectively. Adjuvant treatment with mitotane was assigned to 18 of 34 potentially eligible patients. High-risk patients treated with adjuvant mitotane showed a reduced probability of death, although the difference was not statistically significant. Relapses had numerically higher rate of R1 resection and higher Ki67. Eleven patients underwent first-line therapy with etoposide, doxorubicin, cisplatin and mitotane (EDP-M). Their median progression-free survival was 4.4 months. The median overall survival of entire cohort was 28.9 and the median disease-specific survival (DSS) was 36.2 months. The 5-year DSS rate of ENSAT I, II, III and IV were 100%, 56%, 50% and 0%, respectively. The prognostic value of ENSAT stage and Helsinki score regarding overall survival was confirmed with the multivariate analysis.

conclusionsThe 5-year DSS of our ENSAT II patients was worse than reported in contemporary cohorts. Suboptimal surgery and inconsistent adjuvant therapy with mitotane might have contributed to this outcome. Better outcomes of this rare disease might be accomplished with dedicated teams including various specialties, working towards optimal staging, diagnostic and therapeutic measures.

Indexed as

Adrenal Cortex NeoplasmsAdrenocortical CarcinomaAdultAgedAntineoplastic Agents, HormonalAntineoplastic Combined Chemotherapy ProtocolsChemotherapy, AdjuvantFemaleHumansKaplan-Meier EstimateMaleMiddle AgedMitotanePrognosisRegistriesSloveniaAntineoplastic Agents, HormonalMitotaneadrenocortical carcinomaENSAT stageHelsinki scoreprognostic factorssurvivalsystemic treatment

Identifiers

PMID40014786
PMCPMC11867571

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Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the Socratic graph.