Raras
Buscar doenças, sintomas, genes...
Carcinoma adrenocortical
ORPHA:1501CID-10 · C74.0CID-11 · 2D11.ZDOENÇA RARA

Tumor epitelial maligno raro, geralmente grande (maior que 5 cm), que surge das células corticais adrenais. Os sintomas geralmente estão relacionados à produção excessiva de hormônios e incluem a síndrome de Cushing e o virilismo nas mulheres. Os locais comuns de metástase incluem fígado, pulmão, ossos e linfonodos retroperitoneais. Procedimentos radiológicos avançados permitiram a detecção de pequenos tumores, resultando na melhoria da sobrevida em 5 anos.

Mantido por Agente Raras·Colaborar como especialista →

Introdução

O que você precisa saber de cara

📋

Tumor epitelial maligno raro, geralmente grande (maior que 5 cm), que surge das células corticais adrenais. Os sintomas geralmente estão relacionados à produção excessiva de hormônios e incluem a síndrome de Cushing e o virilismo nas mulheres. Os locais comuns de metástase incluem fígado, pulmão, ossos e linfonodos retroperitoneais. Procedimentos radiológicos avançados permitiram a detecção de pequenos tumores, resultando na melhoria da sobrevida em 5 anos.

Pesquisas ativas
24 ensaios
111 total registrados no ClinicalTrials.gov
Publicações científicas
4.023 artigos
Último publicado: 2026

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-9 / 1 000 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.75
Europe
Início
Childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C74.0
Você se identifica com essa condição?
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

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Sinais e sintomas

O que aparece no corpo e com que frequência cada sintoma acontece

Partes do corpo afetadas

📏
Crescimento
3 sintomas
🧠
Neurológico
2 sintomas
🫘
Rins
2 sintomas
💪
Músculos
1 sintomas
🫃
Digestivo
1 sintomas
🫁
Pulmão
1 sintomas

+ 20 sintomas em outras categorias

Características mais comuns

100%prev.
Carcinoma adrenocortical
55%prev.
Diabetes mellitus
Frequente (79-30%)
55%prev.
Hipertensão
Frequente (79-30%)
55%prev.
Irritabilidade
Frequente (79-30%)
55%prev.
Fraqueza muscular
Frequente (79-30%)
55%prev.
Palpitações
Frequente (79-30%)
30sintomas
Muito frequente (1)
Frequente (27)
Ocasional (1)
Sem dados (1)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 30 características clínicas mais associadas, ordenadas por frequência.

Carcinoma adrenocorticalAdrenocortical carcinoma
Muito frequente100%
Diabetes mellitus
Frequente (79-30%)55%
HipertensãoHypertension
Frequente (79-30%)55%
IrritabilidadeIrritability
Frequente (79-30%)55%
Fraqueza muscularMuscle weakness
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico4.023PubMed
Últimos 10 anos200publicações
Pico2025122 papers
Linha do tempo
2026Hoje · 2026🧪 1993Primeiro ensaio clínico📈 2025Ano de pico
Publicações por ano (últimos 10 anos)

Encontrou um erro ou informação desatualizada? Sugira uma correção →

Genética e causas

O que está alterado no DNA e como passa nas famílias

Genes associados

6 genes identificados com associação a esta condição. Padrão de herança: Not applicable.

PRKAR1AcAMP-dependent protein kinase type I-alpha regulatory subunitDisease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

Regulatory subunit of the cAMP-dependent protein kinases involved in cAMP signaling in cells

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (10)
PKA activationGPER1 signalingCREB1 phosphorylation through the activation of Adenylate CyclaseDARPP-32 eventsHigh laminar flow shear stress activates signaling by PIEZO1 and PECAM1:CDH5:KDR in endothelial cells
MECANISMO DE DOENÇA

Carney complex 1

CNC is a multiple neoplasia syndrome characterized by spotty skin pigmentation, cardiac and other myxomas, endocrine tumors, and psammomatous melanotic schwannomas.

EXPRESSÃO TECIDUAL(Ubíquo)
Artéria tibial
152.7 TPM
Ovário
151.5 TPM
Aorta
138.6 TPM
Útero
138.3 TPM
Tecido adiposo
129.4 TPM
OUTRAS DOENÇAS (9)
familial atrial myxomapigmented nodular adrenocortical disease, primary, 1Acrodysostosis 1 with or without hormone resistanceCarney complex, type 1
HGNC:9388UniProt:P10644
ZNRF3E3 ubiquitin-protein ligase ZNRF3Disease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

E3 ubiquitin-protein ligase that acts as a negative regulator of the Wnt signaling pathway by mediating the ubiquitination and subsequent degradation of Wnt receptor complex components Frizzled and LRP6. Acts on both canonical and non-canonical Wnt signaling pathway. Acts as a tumor suppressor in the intestinal stem cell zone by inhibiting the Wnt signaling pathway, thereby restricting the size of the intestinal stem cell zone (PubMed:22575959). Along with RSPO2 and RNF43, constitutes a master s

LOCALIZAÇÃO

Cell membrane

VIAS BIOLÓGICAS (1)
Regulation of FZD by ubiquitination
EXPRESSÃO TECIDUAL(Ubíquo)
Cérebro - Hemisfério cerebelar
15.1 TPM
Cerebelo
15.1 TPM
Aorta
13.1 TPM
Artéria tibial
12.4 TPM
Córtex cerebral
11.3 TPM
OUTRAS DOENÇAS (1)
adrenal cortex carcinoma
HGNC:18126UniProt:Q9ULT6
CDKN2ACyclin-dependent kinase inhibitor 2ADisease-causing somatic mutation(s) inRestrito
FUNÇÃO

Acts as a negative regulator of the proliferation of normal cells by interacting strongly with CDK4 and CDK6. This inhibits their ability to interact with cyclins D and to phosphorylate the retinoblastoma protein

LOCALIZAÇÃO

CytoplasmNucleus

VIAS BIOLÓGICAS (8)
Regulation of TP53 DegradationOncogene Induced SenescenceOxidative Stress Induced SenescenceStabilization of p53Regulation of RUNX3 expression and activity
OUTRAS DOENÇAS (10)
B-lymphoblastic leukemia/lymphoma with t(9;22)(q34.1;q11.2)familial melanomaLi-Fraumeni syndromefamilial atypical multiple mole melanoma syndrome
HGNC:1787UniProt:P42771
CTNNB1Catenin beta-1Disease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

Key downstream component of the canonical Wnt signaling pathway (PubMed:17524503, PubMed:18077326, PubMed:18086858, PubMed:18957423, PubMed:21262353, PubMed:22155184, PubMed:22647378, PubMed:22699938). In the absence of Wnt, forms a complex with AXIN1, AXIN2, APC, CSNK1A1 and GSK3B that promotes phosphorylation on N-terminal Ser and Thr residues and ubiquitination of CTNNB1 via BTRC and its subsequent degradation by the proteasome (PubMed:17524503, PubMed:18077326, PubMed:18086858, PubMed:189574

LOCALIZAÇÃO

CytoplasmNucleusCytoplasm, cytoskeletonCell junction, adherens junctionCell junctionCell membraneCytoplasm, cytoskeleton, microtubule organizing center, centrosomeCytoplasm, cytoskeleton, spindle poleSynapseCytoplasm, cytoskeleton, cilium basal body

VIAS BIOLÓGICAS (10)
Formation of the nephric ductSpecification of the neural plate borderSynthesis, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1)TCF dependent signaling in response to WNTTranscriptional Regulation by VENTX
MECANISMO DE DOENÇA

Colorectal cancer

A complex disease characterized by malignant lesions arising from the inner wall of the large intestine (the colon) and the rectum. Genetic alterations are often associated with progression from premalignant lesion (adenoma) to invasive adenocarcinoma. Risk factors for cancer of the colon and rectum include colon polyps, long-standing ulcerative colitis, and genetic family history.

EXPRESSÃO TECIDUAL(Ubíquo)
Cervix Endocervix
297.5 TPM
Cervix Ectocervix
257.8 TPM
Artéria tibial
233.5 TPM
Ovário
201.9 TPM
Cérebro - Hemisfério cerebelar
201.3 TPM
OUTRAS DOENÇAS (17)
hepatocellular carcinomasevere intellectual disability-progressive spastic diplegia syndromeovarian cancerpilomatrixoma
HGNC:2514UniProt:P35222
TP53Cellular tumor antigen p53Disease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

Multifunctional transcription factor that induces cell cycle arrest, DNA repair or apoptosis upon binding to its target DNA sequence (PubMed:11025664, PubMed:12524540, PubMed:12810724, PubMed:15186775, PubMed:15340061, PubMed:17317671, PubMed:17349958, PubMed:19556538, PubMed:20673990, PubMed:20959462, PubMed:22726440, PubMed:24051492, PubMed:24652652, PubMed:35618207, PubMed:36634798, PubMed:38653238, PubMed:9840937). Acts as a tumor suppressor in many tumor types; induces growth arrest or apop

LOCALIZAÇÃO

CytoplasmNucleusNucleus, PML bodyEndoplasmic reticulumMitochondrion matrixCytoplasm, cytoskeleton, microtubule organizing center, centrosome

VIAS BIOLÓGICAS (10)
TP53 Regulates Metabolic GenesRegulation of TP53 ExpressionRegulation of TP53 DegradationOncogene Induced SenescenceOxidative Stress Induced Senescence
EXPRESSÃO TECIDUAL(Ubíquo)
Linfócitos
73.0 TPM
Skin Sun Exposed Lower leg
37.0 TPM
Skin Not Sun Exposed Suprapubic
35.2 TPM
Fibroblastos
32.9 TPM
Ovário
32.4 TPM
OUTRAS DOENÇAS (29)
Li-Fraumeni syndromenasopharyngeal carcinoma, susceptibility to, 1hepatocellular carcinomafamilial pancreatic carcinoma
HGNC:11998UniProt:P04637
TERTTelomerase reverse transcriptaseDisease-causing somatic mutation(s) inRestrito
FUNÇÃO

Telomerase is a ribonucleoprotein enzyme essential for the replication of chromosome termini in most eukaryotes. Active in progenitor and cancer cells. Inactive, or very low activity, in normal somatic cells. Catalytic component of the teleromerase holoenzyme complex whose main activity is the elongation of telomeres by acting as a reverse transcriptase that adds simple sequence repeats to chromosome ends by copying a template sequence within the RNA component of the enzyme. Catalyzes the RNA-de

LOCALIZAÇÃO

Nucleus, nucleolusNucleus, nucleoplasmNucleusChromosome, telomereCytoplasmNucleus, PML body

VIAS BIOLÓGICAS (3)
Telomere Extension By TelomeraseFormation of the beta-catenin:TCF transactivating complexRegulation of MITF-M-dependent genes involved in DNA replication, damage repair and senescence
EXPRESSÃO TECIDUAL(Baixa expressão)
Testículo
2.7 TPM
Intestino delgado
0.7 TPM
Brain Caudate basal ganglia
0.6 TPM
Cólon transverso
0.5 TPM
Brain Nucleus accumbens basal ganglia
0.5 TPM
OUTRAS DOENÇAS (13)
dyskeratosis congenita, autosomal dominant 2pulmonary fibrosis and/or bone marrow failure, Telomere-related, 1adrenal cortex carcinomaclear cell sarcoma of kidney
HGNC:11730UniProt:O14746

Medicamentos aprovados (FDA)

1 medicamento encontrado nos registros da FDA americana.

💊 Lysodren (MITOTANE)
Ver no DailyMed/FDA

Variantes genéticas (ClinVar)

2,622 variantes patogênicas registradas no ClinVar.

🧬 TERT: GRCh38/hg38 5p15.33-15.32(chr5:113462-5578808)x1 ()
🧬 TERT: NM_198253.3(TERT):c.3221G>A (p.Cys1074Tyr) ()
🧬 TERT: NM_198253.3(TERT):c.262del (p.Leu88fs) ()
🧬 TERT: NM_198253.3(TERT):c.198_207dup (p.Ser70fs) ()
🧬 TERT: NM_198253.3(TERT):c.11_27dup (p.Val10fs) ()
Ver todas no ClinVar

Vias biológicas (Reactome)

84 vias biológicas associadas aos genes desta condição.

PKA activation PKA activation in glucagon signalling DARPP-32 events Glucagon-like Peptide-1 (GLP1) regulates insulin secretion Vasopressin regulates renal water homeostasis via Aquaporins CREB1 phosphorylation through the activation of Adenylate Cyclase Hedgehog 'off' state GPER1 signaling ADORA2B mediated anti-inflammatory cytokines production FCGR3A-mediated IL10 synthesis ALK mutants bind TKIs Signaling by ALK fusions and activated point mutants Factors involved in megakaryocyte development and platelet production High laminar flow shear stress activates signaling by PIEZO1 and PECAM1:CDH5:KDR in endothelial cells Regulation of FZD by ubiquitination Oxidative Stress Induced Senescence Senescence-Associated Secretory Phenotype (SASP) Oncogene Induced Senescence Cyclin D associated events in G1 Transcriptional Regulation by VENTX Evasion of Oncogene Induced Senescence Due to Defective p16INK4A binding to CDK4 Evasion of Oncogene Induced Senescence Due to Defective p16INK4A binding to CDK4 and CDK6 Evasion of Oxidative Stress Induced Senescence Due to Defective p16INK4A binding to CDK4 Evasion of Oxidative Stress Induced Senescence Due to Defective p16INK4A binding to CDK4 and CDK6 Regulation of MITF-M-dependent genes involved in cell cycle and proliferation Degradation of beta-catenin by the destruction complex Beta-catenin phosphorylation cascade TCF dependent signaling in response to WNT Formation of the beta-catenin:TCF transactivating complex LRR FLII-interacting protein 1 (LRRFIP1) activates type I IFN production Apoptotic cleavage of cell adhesion proteins Deactivation of the beta-catenin transactivating complex Synthesis, secretion, and inactivation of Glucagon-like Peptide-1 (GLP-1) Ca2+ pathway Adherens junctions interactions Binding of TCF/LEF:CTNNB1 to target gene promoters Disassembly of the destruction complex and recruitment of AXIN to the membrane VEGFR2 mediated vascular permeability Myogenesis Signaling by GSK3beta mutants CTNNB1 S33 mutants aren't phosphorylated CTNNB1 S37 mutants aren't phosphorylated CTNNB1 S45 mutants aren't phosphorylated CTNNB1 T41 mutants aren't phosphorylated RHO GTPases activate IQGAPs InlA-mediated entry of Listeria monocytogenes into host cells RUNX3 regulates WNT signaling Cardiogenesis Germ layer formation at gastrulation Regulation of CDH11 function Regulation of CDH19 Expression and Function Regulation of CDH1 Function Degradation of CDH1 Regulation of CDH1 posttranslational processing and trafficking to plasma membrane Activation of NOXA and translocation to mitochondria Activation of PUMA and translocation to mitochondria Pre-NOTCH Transcription and Translation Formation of Senescence-Associated Heterochromatin Foci (SAHF) DNA Damage/Telomere Stress Induced Senescence SUMOylation of transcription factors Autodegradation of the E3 ubiquitin ligase COP1 Association of TriC/CCT with target proteins during biosynthesis Pyroptosis TP53 Regulates Metabolic Genes Ub-specific processing proteases Ovarian tumor domain proteases Recruitment and ATM-mediated phosphorylation of repair and signaling proteins at DNA double strand breaks Interleukin-4 and Interleukin-13 signaling TP53 Regulates Transcription of DNA Repair Genes TP53 Regulates Transcription of Genes Involved in Cytochrome C Release TP53 regulates transcription of several additional cell death genes whose specific roles in p53-dependent apoptosis remain uncertain TP53 Regulates Transcription of Caspase Activators and Caspases TP53 Regulates Transcription of Death Receptors and Ligands TP53 Regulates Transcription of Genes Involved in G2 Cell Cycle Arrest TP53 regulates transcription of additional cell cycle genes whose exact role in the p53 pathway remain uncertain TP53 Regulates Transcription of Genes Involved in G1 Cell Cycle Arrest Regulation of TP53 Expression Regulation of TP53 Activity through Phosphorylation Regulation of TP53 Degradation Regulation of TP53 Activity through Acetylation Regulation of TP53 Activity through Association with Co-factors Regulation of TP53 Activity through Methylation Telomere Extension By Telomerase Regulation of MITF-M-dependent genes involved in DNA replication, damage repair and senescence

Diagnóstico

Os sinais que médicos procuram e os exames que confirmam

Carregando...

Tratamento e manejo

Remédios, cuidados de apoio e o que precisa acompanhar

Pipeline de tratamentos
Pipeline regulatório — de medicamentos já aprovados a drogas em pesquisa exploratória.
2Fase 210
1Fase 15
·Pré-clínico5
Medicamentos catalogadosEnsaios clínicos· 0 medicamentos · 20 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

Hospitais de referência no Brasil e o protocolo oficial do SUS (PCDT)

🇧🇷 Atendimento SUS — Carcinoma adrenocortical

🗺️

Selecione um estado ou use sua localização para ver resultados.

Dados de DATASUS/CNES, SBGM, ABNeuro e Ministério da Saúde. Sempre confirme a disponibilidade diretamente com o estabelecimento.

Pesquisa ativa

Ensaios clínicos abertos e novidades científicas recentes

🟢 Recrutando agora

17 pesquisas recrutando participantes. Converse com seu médico sobre a possibilidade de participar.

NCT06487481 · Study of Preoperative Radiation Therapy in Participants With…Recrutando
PHASE1
NCT06066333 · Study of Radiotherapy and Pembrolizumab in People With Adren…Recrutando
PHASE2
NCT04897321 · B7-H3-Specific Chimeric Antigen Receptor Autologous T-Cell T…Recrutando
PHASE1
NCT06041516 · A First-in-Human Phase I Trial With Antibody Drug Conjugate …Recrutando
PHASE1
NCT04119024 · Gene Modified Immune Cells After Conditioning Regimen for th…Recrutando
PHASE1
NCT05286814 · PDS01ADC in Combination With Hepatic Artery Infusion Pump (H…Recrutando
PHASE2
NCT07189403 · A Study of DS9051b in Participants With Advanced or Metastat…Recrutando
PHASE1
NCT03050268 · Familial Investigations of Childhood Cancer PredispositionRecrutando
NCT06333314 · Dostarlimab for Locally Advanced or Metastatic Cancer (Non-c…Recrutando
PHASE2
NCT03127774 · Surgery and Heated Intraperitoneal Chemotherapy for Adrenoco…Recrutando
PHASE2
NCT00453674 · German Adrenocortical Carcinoma RegistryRecrutando
NCT00669266 · Adrenal Tumors - Pathogenesis and TherapyRecrutando
NCT06587802 · Phase II Study of PD-1 Antibody Combined With Radiotherapy i…Recrutando
PHASE2
NCT05563467 · Pembrolizumab in the Treatment of Advanced, Progressive Adre…Recrutando
PHASE2
NCT06279442 · Adrenal Project: Clinical and Epidemiological Characterizati…Recrutando
NCT05913427 · Evaluation of the Efficacy of Addition of Progesterone to St…Recrutando
PHASE2
NCT04318730 · Phase II Study for Combination of Camrelizumab and Apatinib …Recrutando
PHASE2

Outros ensaios clínicos

111 ensaios clínicos encontrados, 24 ativos.

Distribuição por fase
Ver todos no ClinicalTrials.gov
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Publicações mais relevantes

📖Melhor nível de evidência: Revisão
Timeline de publicações
2.326 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 2.326

#1

Factors Associated With Rare Pediatric Cancer Trial Enrollment: A Report From the Children's Oncology Group Rare Tumors Committee.

Pediatric blood &amp; cancer2026 Feb

Over 90% of US children with cancer are treated at Children's Oncology Group (COG) centers, which seek to maximize enrollment in therapeutic and biobanking studies. Rare cancers have demonstrated lower than expected COG enrollment. We evaluated trends in COG rare cancer enrollment compared to US incidence from Surveillance, Epidemiology, and End Results (SEER) registries, examining the impact of COG therapeutic trials and Project:EveryChild, a cancer biobank/registry. COG and SEER data from 2002 to 2020 were queried for US patients younger than 18 years old with adrenocortical carcinoma (ACC), nasopharyngeal carcinoma (NPC), retinoblastoma (RB), thyroid carcinoma, and melanoma. We compared demographic data between COG and SEER, extrapolating incidence for each cancer to analyze trends in COG enrollment. Patient characteristics, including age, sex, and race, were similar between COG (n = 2184) and SEER (n = 5514). COG enrollment for rare cancers remained low (11%). Initiating Project:EveryChild did not increase enrollment (12% pre- vs. 8% post-Project:EveryChild; p < 0.01). For cancers with available therapeutic trials (ACC, NPC, and RB), COG enrollment was higher during trial accrual (40%) than when no trial was open (12%; p < 0.01). Patient geography and income did not appear as barriers to COG enrollment. Although children with rare cancers enrolled in COG studies reflect the US population, enrollment in the COG registry/biospecimen repository continues to be limited in the absence of therapeutic trials, impacting data and biospecimens available to inform therapeutic trial development. Expansion of therapeutic trials or free molecular testing through the Molecular Characterization Initiative may increase data and biospecimens for these rare cancers.

#2

A Case of Pediatric Adrenocortical Carcinoma With McCune-Albright Syndrome Masquerading as Spontaneously Regressing Neuroblastoma.

Pediatric blood &amp; cancer2026 Mar 15
#3

Quality of life questionnaire development and validation for adrenocortical carcinoma patients.

European journal of endocrinology2026 Mar 24

ACC patients' quality of life may be impacted by symptoms related to cancer or treatments, as well as emotional, spiritual, existential, social, economic, and sexual aspects. The aim was the development and psychometric validation of a new instrument (QoLACC) for measuring Patient Reported Outcome in patients with ACC. The study was conducted from October 2024 to January 2025 at the Oncology Unit of Brescia's ASST. Fifteen patients and 15 physicians graded the comprehensibility/difficulty and relevance of the items of QoLACC whereas 37 consecutive patients assessed psychometric validity. Comprehensibility, difficulty and clinical relevance of the items to include in the QoLACC questionnaire were assessed by selected ACC patients and clinicians. Validated scales were co-administered to the developed QoLACC questionnaire to assess physical and psychological symptoms, and the test-retest reliability and construct validity psychometric validation were performed. The QoLACC items showed excellent content validity [0.93-1.00] and acceptable to excellent comprehensibility [0.80-1.00] and difficulty [0.80-1.00], with minor exceptions. Item analysis showed good discriminant capability and response variability. The total questionnaire has good reliability (the intraclass correlation coefficient (ICC) was 0.870 [95% CI: 0.734-0.937]) and construct validity proved by the moderate to strong correlations [0.39-0.62] between the QoLACC and the EORTC item 29 and item 30, the QoLACC financial section and the COST (0.47), and the QoLACC spiritual section and the JSWBS (0.60). We defined and validated the Italian questionnaire aiming to explore ACC patients' quality of life, to help the Health Care Professionals to manage and support these patients.

#4

Adrenocortical Carcinoma: From genomics to patients.

Annales d'endocrinologie2026 Mar 20

Adrenocortical carcinoma (ACC) is a rare malignancy with limited therapeutic options and poor prognosis in advanced stages. Clinico-pathological markers, such as Ki67 and ENSAT stage remain insufficient to fully predict recurrence or treatment response, highlighting the need for molecular characterization.Molecular classification: Omic approaches, including transcriptomics, DNA methylation and chromosomal alteration profiling have consistently identified two distinct ACC subtypes "C1A" and "C1B". C1A tumors, overexpressing proliferation genes, are associated to poorer outcomes. C1B tumors however, are characterized by their immune signature and are of better prognosis. Single-cell analyses have helped understanding the mechanisms underlying these differences, hinting at a central role of intratumoral steroid differentiation in shaping the immune microenvironment.Clinical and therapeutic perspectives: Transcriptome sequencing from paraffin-embedded samples are bringing molecular classification closer to routine care, with ongoing prospective trials evaluating its feasability. Genomic classification represents a major step in ACC management and understanding, offering improved prognostic stratification and orienting future therapeutic strategies.

#5

ACR Appropriateness Criteria® Staging and Follow-Up of Adrenal Cancer.

Journal of the American College of Radiology : JACR2026 Mar 21

Primary adrenal gland malignancies include adrenocortical carcinoma (ACC) and pheochromocytoma. For initial staging and restaging or surveillance of known or suspected ACC, MRI of the abdomen and pelvis without and with contrast, CT of the abdomen and pelvis with contrast, CT of the chest with contrast, and fluorine-18-2-fluoro-2-deoxy-D-glucose (FDG)-PET/CT, or FDG-PET/MRI are recommended to assess for local extent and distant metastases or to evaluate for recurrence. For initial staging and restaging or surveillance of known or suspected pheochromocytoma, MRI of the abdomen and pelvis without and with contrast, CT of the abdomen and pelvis with contrast, CT of the chest with contrast, DOTATATE PET/CT, or DOTATATE PET/MRI are recommended to assess local extent and distant metastases or to evaluate for recurrence. Meta-iodobenzylguanidine (MIBG) scan whole body with single-photon emission CT (SPECT) or SPECT/CT area of interest is an acceptable alternative for initial staging of known or suspected pheochromocytoma, in particular to determine eligibility for I-131 MIBG therapy. The American College of Radiology Appropriateness Criteria are evidence-based guidelines for specific clinical conditions that are reviewed annually by a multidisciplinary expert panel. The guideline development and revision process support the systematic analysis of the medical literature from peer reviewed journals. Established methodology principles such as Grading of Recommendations Assessment, Development, and Evaluation or GRADE are adapted to evaluate the evidence. The RAND/UCLA Appropriateness Method User Manual provides the methodology to determine the appropriateness of imaging and treatment procedures for specific clinical scenarios. In those instances where peer reviewed literature is lacking or equivocal, experts may be the primary evidentiary source available to formulate a recommendation.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC2.452 artigos no totalmostrando 199

2026

Quality of life questionnaire development and validation for adrenocortical carcinoma patients.

European journal of endocrinology
2026

Adrenocortical Carcinoma: From genomics to patients.

Annales d'endocrinologie
2026

ACR Appropriateness Criteria® Staging and Follow-Up of Adrenal Cancer.

Journal of the American College of Radiology : JACR
2026

Oncocytic adrenocortical carcinoma: A case report.

Urology case reports
2026

Adrenocortical Carcinoma as a Manifestation of Birt-Hogg Dubé Syndrome.

Endocrine-related cancer
2026

Surgical Resection of Abdominal Solid Organ Tumors With Inferior Vena Cava Extension: A Single-Center Experience With Long-Term Follow-Up.

Cureus
2026

A Case of Pediatric Adrenocortical Carcinoma With McCune-Albright Syndrome Masquerading as Spontaneously Regressing Neuroblastoma.

Pediatric blood &amp; cancer
2026

International Real-world study on osilodrostat efficacy and safety in adrenal Cushing syndrome.

The Journal of clinical endocrinology and metabolism
2026

Hepatic embolization for Cushing syndrome from metastatic tumors: a single-center case series.

Journal of the Endocrine Society
2026

In-depth Genetic and Molecular Characterization of Unilateral Coexisting Adrenal Cortical Adenoma and Carcinoma in the Context of MEN1 Syndrome.

Endocrine pathology
2026

Rare Association Between Neurofibromatosis Type 1 and Adrenocortical Carcinoma.

Clinical case reports
2025

Adrenocortical carcinoma presenting with extensive striae: A case report and literature review.

Indian journal of cancer
2025

Adult-type epithelial neoplasms in children and adolescents-A retrospective analysis on a subgroup of very rare tumors.

Indian journal of cancer
2026

Clinical predictors of outcome in advanced adrenocortical carcinoma: a multicenter international ENSAT study.

European journal of endocrinology
2026

Prognostic, chemotherapy and immunotherapy roles of GPR37/GPR37L1 in pan-cancer.

Medicine
2026

Natural history of large adrenal tumors.

Frontiers in endocrinology
2026

Prognostic role of pretreatment [18F]FDG PET/CT in patients affected by adrenocortical carcinoma and treated with chemotherapy.

European journal of nuclear medicine and molecular imaging
2026

Time trends in incidence, treatment and survival of patients with adrenocortical carcinoma, a nationwide study.

The Journal of clinical endocrinology and metabolism
2026

Prevalence of venous extension in malignant adrenal neoplasia: beyond primary tumors and identification of a novel imaging sign.

European radiology
2026

Expression of YME1 Like 1 ATPase Increases With the Stage of Adrenocortical Carcinoma Tissue and Is Associated With Poor Patient Prognosis.

Anticancer research
2026

Targeting ER stress in adrenocortical carcinoma: Celastrol as a novel therapeutic candidate.

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
2026

Exceptional Response to a Single Dose of Pembrolizumab as Salvage Therapy for Metastatic Adrenocortical Carcinoma.

JCEM case reports
2026

Histopathological and Molecular Characteristics of Pediatric Adrenocortical Tumors: where Do we Stand?

Endocrine pathology
2026

Digital vs conventional glycemic monitoring in rare endocrine cancers: comparison of effectiveness during chemotherapy.

Endocrine connections
2026

A case of immune thrombocytopenia aggravated by therapy for hypercortisolism in adrenocortical carcinoma.

Therapeutic advances in endocrinology and metabolism
2026

Clinical Application of Steroid Profiles and Their Interpretation in Adrenal Disorders.

Diagnostics (Basel, Switzerland)
2026

Three cases of pediatric adrenocortical carcinoma with intermediate malignant potential: a case report with literature review.

AME case reports
2026

Preclinical evaluation of progesterone combined with EDP-M scheme in 2D and 3D models of adrenocortical carcinoma.

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
2026

Endocrine Hypertension: The Role of Imaging in Diagnosis and Management.

The British journal of radiology
2026

Mismatch repair deficiency and microsatellite instability in adrenocortical carcinoma.

ESMO open
2026

Lipid synthesis seems to drive proliferation in Men1 mouse adrenals and human adrenocortical cell lines.

Endocrine-related cancer
2026

Reliability of CT attenuation value for adrenal masses.

Endocrine connections
2026

[Sarcomatoid variant of adrenocortical carcinoma].

Medicina
2026

Massive non-functioning Adrenocortical carcinoma presenting as acute abdomen without rupture: A case report.

Urology case reports
2026

Hepcidin concentration and effect on iron homeostasis in patients with adrenocortical carcinoma and benign adrenal lesions - a pilot study.

Endocrine
2026

[Adrenocortical carcinoma (ACC): current surgical treatment strategies].

Chirurgie (Heidelberg, Germany)
2025

Characterization of Unilateral Adrenal Incidentalomas: Hormonal Analysis, Computed Tomography, and Magnetic Resonance Imaging Correlation.

Cureus
2026

Over-expression of Anillin Actin Binding Protein in Adrenocortical Carcinoma Tissues Is Associated With Poorer Prognosis of Patients.

Anticancer research
2026

Pan-Cancer Landscape of CDK1 Uncovers Its Potential Prognostic Significance and Therapeutic Targeting in Adrenocortical Carcinoma.

Anticancer research
2026

A nonfunctional adrenal incidentaloma revealed by intestinal obstruction: metastatic adrenocortical carcinoma diagnosed by surgery.

Endocrinology, diabetes &amp; metabolism case reports
2026

Assessment of Adrenal Cortical Neoplasms for the GU Pathologist.

Advances in anatomic pathology
2026

Death receptor 3: A paradoxical biomarker and therapeutic target in pan-cancer.

Critical reviews in oncology/hematology
2026

Is adrenal adenoma to carcinoma transformation possible? - Illustrative cases and literature review.

The Journal of clinical endocrinology and metabolism
2026

Machine Learning Approach for Differentiation of Pheochromocytoma from Adrenocortical Cancer and Non-Functioning Adrenal Adenomas.

Life (Basel, Switzerland)
2025

Perspectives on Adrenal Tumor Surgery.

Medicina (Kaunas, Lithuania)
2026

Steroid Phenotype Stratification Reveals Distinct HLA Expression Signatures in Adrenocortical Carcinoma.

Cancers
2026

Adrenocortical Carcinoma With Right Atrial Extension in a Three-Year-Old Child: A Case Report.

Cureus
2026

Androgen production in adrenocortical H295R cells is regulated by thyroid hormone T3 without reciprocal thyroid axis modulation in pediatric CAH.

The Journal of steroid biochemistry and molecular biology
2026

Serum steroid profiling by LC-MS/MS in distinguishing adrenocortical carcinoma from other indeterminate adrenal masses.

The Journal of steroid biochemistry and molecular biology
2026

Targeting matrix metalloproteinase-14 disrupts DNA repair and reduces viability in adrenocortical carcinoma.

bioRxiv : the preprint server for biology
2026

Establishment of a patient-derived adrenocortical carcinoma 3D tumor construct platform for evaluation of therapeutic strategies.

bioRxiv : the preprint server for biology
2026

Machine Learning-Driven Identification and In Vitro Validation of the APOBEC3B-ANLN Regulatory Axis in Adrenocortical Carcinoma.

Endocrine
2026

Minimally Invasive Tubular Transpedicular Partial Corpectomy and Percutaneous Segmental Posterior Fixation With a Combined Titanium and Carbon Fiber Construct: 2-Dimensional Operative Video.

Operative neurosurgery (Hagerstown, Md.)
2026

Adrenal tumors in the elderly.

Endocrine-related cancer
2026

Chemo-immunotherapy with cisplatin and nivolumab as second-line approach in metastatic adrenocortical carcinoma.

European journal of endocrinology
2026

A novel 2D and 3D model for primary adrenocortical carcinoma of advanced and metastasized stage co-secreting cortisol, aldosterone, testosterone, 18-oxocortisol and 18-hydroxycortisol.

Endocrine-related cancer
2026

Prevalence and clinical characteristics of Lynch syndrome-associated adrenocortical carcinoma.

Endocrine-related cancer
2025

Neutrophil-to-lymphocyte ratio as a prognostic predictor in adrenocortical carcinoma: a retrospective cohort study stratified by cortisol secretion status.

Translational cancer research
2026

Psychosocial burden in adrenocortical carcinoma patients.

European journal of endocrinology
2026

Mitotane dosage, plasma levels, and anthropometric measurements in pediatric adrenocortical carcinoma.

Endocrine oncology (Bristol, England)
2026

Systemic immune-inflammation index as an independent predictor of malignancy in 4-6 cm adrenal incidentalomas.

Actas urologicas espanolas
2026

Interaction between ENPP1 and homologous recombination deficiency defines distinct pan-cancer signatures: A retrospective observational study.

Medicine
2026

The role of PCMT1 in prognosis tumor immune microenvironment and therapeutic responses across cancers.

Discover oncology
2026

Bioinformatics Analysis Reveals that Minichromosome Maintenance 10 Replication Initiation Factor Is a Possible Prognostic Marker for Patients With Adrenocortical Carcinoma.

Cancer diagnosis &amp; prognosis
2025

Adrenocortical Carcinoma and Inferior Vena Cava Thrombosis: Diagnosis and Perioperative Management.

Cureus
2026

Adrenocortical Mitochondria-Associated Membranes: Isolation, Characterization, and Lipidoproteomic Response to Mitotane.

Journal of the Endocrine Society
2025

Long-Term Transcriptomic Reprogramming in Peripheral Blood Mononuclear Cells of Severe COVID-19 Survivors Reveals Pro-Oncogenic Signatures and Cancer-Associated Hub Genes.

Viruses
2025

The Intricate Puzzle of Adrenocortical Tumors: Revisitation of Two Old Cases of Virilizing Adrenocortical Neoplasia with Contradictory Diagnostic and Histopathological Findings and Opposite Conclusions.

Life (Basel, Switzerland)
2025

Metabolic and Cardiac Complications in a Case of Adrenal Carcinoma.

EJIFCC
2026

Factors Associated With Rare Pediatric Cancer Trial Enrollment: A Report From the Children's Oncology Group Rare Tumors Committee.

Pediatric blood &amp; cancer
2025

Description of six cases of melanoma in 512 patients with germline pathogenic variants in the TP53 gene.

Familial cancer
2025

Mitotane-Induced Hypothyroidism and Dyslipidemia in Adrenocortical Carcinoma: Sex Differences and Novel Evidence from a Thyroid Cell Model.

Current oncology (Toronto, Ont.)
2025

Precision Care for Hereditary Urologic Cancers: Genetic Testing, Counseling, Surveillance, and Therapeutic Implications.

Current oncology (Toronto, Ont.)
2026

ASO Visual Abstract: Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study.

Annals of surgical oncology
2025

A genome-wide RNAi screen for novel CIN genes using human artificial chromosome.

PNAS nexus
2025

New Diagnosis of Adrenocortical Carcinoma with Stroke Workup.

Case reports in neurology
2026

Outcome and Toxicity Profile of Stereotactic Body Radiation Therapy for Adrenal Tumours in Dogs.

Veterinary and comparative oncology
2025

VLDL: The key factors influencing the distribution of mitotane in patients with adrenocortical carcinoma.

Journal of pharmaceutical analysis
2026

Impact of Adjuvant Radiotherapy and Mitotane on Survival in Localized Adrenocortical Carcinoma: A Retrospective Cohort Study.

International journal of urology : official journal of the Japanese Urological Association
2025

Exploring the causal relationship between matrix metalloproteinases and adrenocortical carcinoma: A Mendelian randomization study.

Medicine
2026

Rising Incidence of Neuroendocrine Neoplasms in Northern Switzerland-Data From the Cancer Registry.

Journal of the Endocrine Society
2025

Bilateral adrenal tumors presenting with Cushing syndrome in the setting of horseshoe kidney: surgical and urological considerations (case report).

The Pan African medical journal
2025

Characteristic of Aldosterone-Producing adrenocortical carcinoma: review of the literature.

Medical oncology (Northwood, London, England)
2025

Intratumoral Microbiota Correlates with AP-2 Expression: A Pan-Cancer Map with Cohort-Specific Prognostic and Molecular Footprints.

International journal of molecular sciences
2025

Adrenal-adipose tissue crosstalk in health and disease.

European journal of endocrinology
2025

Diagnostic accuracy of neutrophil-to-lymphocyte ratio and platelet-to-lymphocyte ratio in the differential diagnosis of adrenocortical carcinoma: a retrospective study.

Translational andrology and urology
2026

A quantitative, broad-panel LC-MS/MS method for the analysis of intact steroid conjugates: A novel approach to steroid profiling for biomarker research in corticoid-dependent diseases.

Journal of pharmaceutical and biomedical analysis
2025

Characterization of p53 p.T253I as a pathogenic mutation underlying Li-Fraumeni Syndrome.

PloS one
2025

Pediatric Adrenocortical Carcinoma Presenting With Virilization: A Case of a Low-Grade Tumor in a Young Child.

Cureus
2025

Prolonged Mitotane Administration in Metastatic Adrenocortical Carcinoma With Over a Decade of Survival: A Case Series.

Case reports in endocrinology
2025

Unveiling PAK4 as a Key Biomarker in Adrenocortical Carcinoma: Insights from Bioinformatics and Experimental Evidence.

Archivos espanoles de urologia
2026

Mitigating subtherapeutic cabozantinib exposure after prior mitotane therapy in adrenocortical carcinoma: Pharmacological boosting with cobicistat.

British journal of clinical pharmacology
2025

Postoperative Hemodynamic Instability Following Adrenalectomy for Presumed Adrenocortical Carcinoma: Anesthetic Implications of a Rare Cavernous Hemangioma.

Cureus
2025

Rare but not forgotten: Therapeutic advancements for rare childhood cancers.

Molecular therapy. Oncology
2025

Posterior retroperitoneoscopic adrenalectomy (PRA) in adrenocortical carcinoma (ACC).

Langenbeck's archives of surgery
2025

(Non)canonical Wnt signaling, cytoarchitecture and stemness: new insights from primary nonmetastatic, primary metastatic, regional and distant metastatic models of adrenocortical carcinoma.

Experimental &amp; molecular medicine
2025

A comprehensive pan-cancer analysis of the prognostic role and immunotherapy efficacy evaluation of Glyoxalase 1.

Discover oncology
2025

The Utility of Cabozantinib in the Therapy of Endocrine Tumours.

Endocrine pathology
2025

The RAB27A effector SYTL5 regulates mitophagy and mitochondrial metabolism.

eLife
2026

Oligometastatic adrenocortical carcinoma: definition and treatment.

Current opinion in oncology
2025

Oncocytic Adrenocortical Carcinoma in an Infant: Clinical Findings and Imaging Features.

Clinical nuclear medicine
2025

Synergistic Inhibition of PI3K and HSP90 Enhanced Antitumorigenic Efficacy in Adrenocortical Carcinoma.

Research square
2025

Corrigendum to "FGF/FGFR inhibitors downmodulate c-Myc oncoprotein and hamper the growth of adrenocortical carcinoma" [Biomed. Pharmacother. 192 (2025) 118677].

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
2025

Primary cholangiocarcinoma of the adrenal gland: a surgical and diagnostic challenge - a case report.

BMC urology
2026

Molecular landscape and biomarker discovery in adrenocortical carcinoma: An integrative review of bioinformatics and translational insights.

Pathology, research and practice
2025

Comparative Proteomic Profiling of Adrenocortical Neoplasia Using Mass Spectrometry.

Journal of proteome research
2026

Surgical Management and Outcomes in Pediatric Adrenocortical Carcinoma: A Pediatric Surgical Oncology Research Collaborative Study.

Annals of surgical oncology
2025

Real-world osilodrostat effectiveness and safety in non-pituitary Cushing syndrome.

The Journal of clinical endocrinology and metabolism
2025

Prevalence of Malignancy Among Incidental Indeterminate Adrenal Nodules on Contrast-Enhanced CT in Patients Without Known Cancer: A Multiinstitutional Study.

AJR. American journal of roentgenology
2025

Glutamine antagonism suppresses tumor growth in adrenocortical carcinoma through inhibition of de novo nucleotide biosynthesis.

bioRxiv : the preprint server for biology
2025

Innovative molecular targets for combatting metastasis in adrenocortical carcinoma.

Frontiers in endocrinology
2026

Potential endogenous lipid ligands for the nuclear receptor transcription factor Steroidogenic Factor-1.

Advances in biological regulation
2025

The Cytoskeleton in Adrenal Physiology and Tumours: Functional Roles and Emerging Molecular Targets.

International journal of molecular sciences
2026

Contemporary Approaches to Adrenocortical Carcinoma.

Hematology/oncology clinics of North America
2025

The role of 18F-FDG PET/CT in adrenocortical carcinoma: a systematic review.

Endocrine-related cancer
2025

Publisher Correction: Adrenocortical carcinoma survival gene HMMR was identified as being targeted by fluorouracil and epirubicin using a gene coexpression network-based drug repositioning strategy.

Scientific reports
2025

A comprehensive approach to the CT detection and evaluation of large adrenal masses part 2: malignant adrenal lesions and future directions.

Abdominal radiology (New York)
2025

[Advances in the diagnosis and treatment of adrenal cortex surgical diseases].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2025

Adrenal hemorrhage: diagnostics, management, and treatment. Review and clinical update.

Wideochirurgia i inne techniki maloinwazyjne = Videosurgery and other miniinvasive techniques
2025

Identification of EFNA3 as candidate prognosis marker and potential therapeutic target for adrenocortical carcinoma.

Oncology letters
2025

Comprehensive Analysis Identifies Tumor Mutation Burden-associated Genes ASPM and KIF11 as Novel Biomarkers for Adrenocortical Carcinoma.

Current cancer drug targets
2025

Agouti-related peptide: a promising prognostic biomarker in adrenocortical carcinoma. Results from a large single-centre cross-sectional analysis.

European journal of endocrinology
2025

Synergistic dual oncogenic role of CKS2 and ACAT2: Enhanced regulatory coherence and biomarker potential in adrenocortical carcinoma.

Cancer genetics
2025

Multivisceral Resection for Suspected Adrenocortical Carcinoma.

Journal of clinical medicine
2025

Phase 2 Study of Monotherapy with Pembrolizumab for Advanced Adrenocortical Carcinoma.

Journal of immunotherapy and precision oncology
2025

Bilateral and recurrent adrenocortical carcinoma in MEN1: a case report and review of the literature.

Endocrine oncology (Bristol, England)
2025

Determination of phytonutrients, antioxidant properties and in vitro effect of the microgreen Trigonella foenum-graecum L. on H295R carcinoma cells.

3 Biotech
2026

A novel therapeutic approach to adrenocortical carcinoma repurposing fingolimod to target sphingolipid metabolism in metastatic disease.

Surgery
2025

Feminizing Adrenocortical Carcinoma in Men: A Rare Cause of Persistent Gynecomastia and a Contemporary Literature Review.

Epidemiologia (Basel, Switzerland)
2025

Role of Palliative Bronchial Stenting in Endobronchial Metastasis: An Atypical Case of Metastatic Adrenocortical Carcinoma in a Young Patient.

Clinical case reports
2025

FGF/FGFR inhibitors downmodulates c-Myc oncoprotein and hampers the growth of adrenocortical carcinoma.

Biomedicine &amp; pharmacotherapy = Biomedecine &amp; pharmacotherapie
2025

Case Report: Adrenal angiomatoid fibrous histiocytoma misdiagnosed as pheochromocytoma.

Frontiers in oncology
2025

SorCS3 suppresses adrenocortical carcinoma progression by enhancing IGF2R-mediated endocytic trafficking and signaling attenuation.

Journal of translational medicine
2025

Germline targeted next-generation sequencing in patients with adrenal incidentalomas.

Frontiers in endocrinology
2025

Primary adrenal lymphoma and its mimics: clinico-radiological differential diagnosis.

Frontiers in endocrinology
2025

Preclinical evaluation of the antitumoral efficacy of Wee1 inhibitor AZD1775 in adrenocortical carcinoma.

Pharmacological research
2025

Demographics, Clinical Profiles, and Outcomes of Patients With Adrenal Disorders in a Tertiary Care Center: A Retrospective Study.

Cureus
2025

Automated Microfluidic Platform for Single Spheroid Culture and Extracellular Vesicle Isolation: Application to Spheroid Transcriptomic Profiling.

Small (Weinheim an der Bergstrasse, Germany)
2025

Investigation of DNA Damage Response Genes Validates the Role of DNA Repair in Pediatric Cancer Risk and Identifies SMARCAL1 as a Novel Osteosarcoma Predisposition Gene.

Journal of clinical oncology : official journal of the American Society of Clinical Oncology
2025

The choice of adjuvant radiotherapy in adrenocortical carcinoma patients after radical adrenalectomy: a population-based analysis.

Discover oncology
2026

Diagnosis and grading of adrenal cortical carcinoma.

Virchows Archiv : an international journal of pathology
2025

Impact of steroid differentiation on tumor microenvironment revealed by single-nucleus atlas of adrenal tumors.

Nature communications
2025

Rapidly Progressive Metastatic Adrenocortical Carcinoma With Oncocytic Features in a Young Male: A Case Report.

Cureus
2025

Robotic adrenalectomy: a comprehensive review of perioperative outcomes, comparative efficacy, and technological advancements.

Journal of robotic surgery
2025

Multi-omics-based molecular classification of adrenocortical carcinoma predicts response to immunotherapy and targeted treatments.

Discover oncology
2025

Incidence and risk factors of venous thromboembolism in an adrenocortical carcinoma patient cohort.

Endocrine connections
2026

Surgical management of metastatic adrenocortical carcinoma: Is there a role for multivisceral resection?

Surgery
2025

Case Report: Trilostane therapy in a dog with recurrent adrenocortical carcinoma producing an array of steroid hormones.

Frontiers in veterinary science
2026

Abdominal distention and lethargy in a 6-year-old spayed female ferret.

Journal of the American Veterinary Medical Association
2025

Oncocytic Carcinoma: A Rare Hormone-Producing Tumor.

Cureus
2025

Multi-modal characterization of metabolic and immune gene clusters in adrenocortical carcinoma treatment.

NPJ precision oncology
2025

Educational Point-of-Care Ultrasound Discovers Adrenal Cortical Carcinoma, a Case Report: Clinical Implications and Review of the Literature.

HCA healthcare journal of medicine
2025

Cabozantinib plus Atezolizumab in Advanced, Progressive Endocrine Malignancies: A Multicohort, Basket, Phase II Trial (CABATEN/GETNE-T1914).

Clinical cancer research : an official journal of the American Association for Cancer Research
2025

Efficacy of systemic therapy in metastatic adrenocortical carcinoma: a meta-analysis of prospective clinical trials.

Endocrine oncology (Bristol, England)
2025

SLC16A3 (MCT4) expression in tumor immunity and Metabolism: Insights from pan-cancer analysis.

Biochemistry and biophysics reports
2025

Iron Oxide Nanoparticle Uptake, Toxicity, and Steroidogenesis in Adrenocortical Carcinoma Cells Using a Multicellular in vitro Model.

International journal of nanomedicine
2025

Giant non-functioning adrenocortical carcinoma: a case report and literature review.

Chinese clinical oncology
2025

Non-functional oncocytic adrenocortical carcinoma: Case report with clinicopathologic and surgical correlation.

Urology case reports
2025

Frontiers in Systemic Therapy for Unresectable or Metastatic Adrenocortical Carcinoma: Harnessing Novel Therapeutic Approaches.

Clinical Medicine Insights. Oncology
2025

Mitotane-Induced Endocrine Alterations in Children with Adrenocortical Carcinoma: Clinical Implications from a 20-Year Retrospective Study.

Children (Basel, Switzerland)
2026

Tailored surgery for large adrenal tumors: the minimally invasive to open (hybrid) approach.

Updates in surgery
2025

The UALCAN and GEPIA Analyses of the TCGA Database Show a Strong Association Between Increased Expression of Stathmin 1 in Adrenocortical Carcinoma Tissues and Patient Survival.

Anticancer research
2025

Exploring DNA topoisomerase II alpha in adrenocortical carcinoma through multi-omics analysis: a potential biomarker and therapeutic target.

Acta biochimica et biophysica Sinica
2025

The value of CT texture analysis in predicting mitotic activity and morphological variants of adrenocortical carcinoma.

Frontiers in radiology
2025

Clinicopathological spectrum of adrenal tumors: a retrospective study from a Romanian tertiary referral center.

Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie
2025

Clinical spectrum, imaging characteristics, and treatment outcomes of pediatric adrenocortical tumors: a 24-year experience from Western India.

Journal of pediatric endocrinology &amp; metabolism : JPEM
2025

A standardized surgical approach to multifocal locoregionally recurrent left-sided adrenocortical carcinoma.

European journal of surgical oncology : the journal of the European Society of Surgical Oncology and the British Association of Surgical Oncology
2025

In-frame germline TP53 variant impairs p53 oligomerization and predisposes to cancer.

Scientific reports
2025

A Case of Myxoid Adrenocortical Carcinoma With 1p Deletion Identified by Whole Exome Sequencing.

Pathology international
2025

Evaluating the diagnostic utility of [⁶⁸Ga]Ga-Pentixafor in solid tumors: a systematic review.

Annals of nuclear medicine
2025

A nationwide study evaluating indications and outcomes for adrenalectomy in children in the Netherlands.

Surgery
2025

A rare pediatric case of Li-Fraumeni syndrome presenting with virilization symptoms and dual primary malignancies on magnetic resonance imaging and [18F]-fluorodeoxyglucose positron emission tomography/computed tomography.

Pediatric radiology
2025

Transcriptomic validation and clinical translation of CCDC78 as a prognostic biomarker in colorectal cancer.

American journal of cancer research
2025

External validation of the S-GRAS score for predicting recurrence in patients with adrenocortical carcinoma: implications for adjuvant mitotane therapy.

European journal of endocrinology
2025

Efficacy and Safety of Radiotherapy and Systemic Treatments in Adrenocortical Carcinoma: Systematic Review and Meta-Analysis.

The Journal of clinical endocrinology and metabolism
2025

Adverse events related to mitotane during treatment of adrenocortical carcinoma.

Medicina
2025

Adrenal Incidentaloma Masquerading as Malignancy: A Rare Case of Ganglioneuroma with Nodal Metastasis.

Case reports in oncology
2025

Metastatic Pheochromocytoma in a Patient With Li-Fraumeni Syndrome.

JCEM case reports
2025

Differentiating between adrenocortical carcinoma and pheochromocytoma by a CT-based radiomics model: a multicenter retrospective study.

BMC medical imaging
2025

Extraordinary Response to Mitotane Monotherapy in Advanced Aggressive Adrenocortical Carcinoma.

JCEM case reports
2025

Hyperandrogenism-induced Athletic Excellence in a Young Patient with Adrenocortical Carcinoma: 10 Years of Remission.

JCEM case reports
2025

Management of cushing's syndrome in patients with adrenocortical cancer: state of the art and future perspectives.

Reviews in endocrine &amp; metabolic disorders
2025

Machine learning-based classification of adrenal tumors using clinical, hormonal, and body composition data.

European journal of endocrinology
2025

Prognostic Factors Among Patients with Non-metastatic Adrenocortical Carcinoma.

International journal of endocrinology and metabolism
2025

Prognostic factors and treatment outcomes in patients with adrenal cortical carcinoma: A registry-based study.

Medicine
2025

Ki-67 as a Predictor of Metastasis in Adrenocortical Carcinoma: Artificial Intelligence Insights from Retrospective Imaging Data.

Journal of clinical medicine
2025

Comprehensive analysis of an ATF/CREB family-based signature with regard to prognosis and immune feature in adrenocortical carcinoma: a cohort study.

International journal of surgery (London, England)
2025

β-catenin functions as a molecular adapter for disordered cBAF interactions.

Molecular cell
2025

High expression of EFNA3 in adrenocortical carcinoma and its association with prognosis.

International journal of clinical pharmacology and therapeutics
2025

Cell division cycle 20 promotes tumor progression and predicts poor clinical outcome in childhood and adult adrenocortical carcinoma.

Journal of clinical &amp; translational endocrinology
2025

Report of a rare case of childhood adrenocortical carcinoma and literature review.

Urology case reports
2025

Adrenocortical carcinoma survival gene HMMR was identified as being targeted by fluorouracil and epirubicin using a gene coexpression network-based drug repositioning strategy.

Scientific reports
2025

Emerging treatment options for adrenocortical carcinoma.

Current opinion in endocrinology, diabetes, and obesity
2025

Defactinib in Combination with Mitotane Can Be an Effective Treatment in Human Adrenocortical Carcinoma.

International journal of molecular sciences
2025

Bimodal Genomic Approach Predicting Semaphorin 7A (SEMA7A) as Prognostic Biomarker in Adrenocortical Carcinoma.

Cancers
2025

Adrenocortical organoids: A promising tool for modelling human physiology and translational research.

Presse medicale (Paris, France : 1983)
2025

The management of adrenocortical carcinoma in the era of precision medicine.

Critical reviews in oncology/hematology
2025

Therapeutic frontiers in adrenocortical carcinoma: from standards to innovation.

Medical oncology (Northwood, London, England)
2025

An Excellent Clinical and Radiological Response Pattern to Pembrolizumab in a Patient With Metastatic Adrenocortical Carcinoma and Lynch Syndrome.

IJU case reports
Ver todos os 2.452 no EuropePMC

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Doenças relacionadas

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Referências e fontes

Bases de dados externas citadas neste artigo

Publicações científicas

Artigos indexados no PubMed ligados a esta doença no grafo RarasNet — título, periódico e PMID direto da fonte, sem intermediação de IA.

  1. Factors Associated With Rare Pediatric Cancer Trial Enrollment: A Report From the Children's Oncology Group Rare Tumors Committee.
    Pediatric blood &amp; cancer· 2026· PMID 41451685mais citado
  2. A Case of Pediatric Adrenocortical Carcinoma With McCune-Albright Syndrome Masquerading as Spontaneously Regressing Neuroblastoma.
    Pediatric blood &amp; cancer· 2026· PMID 41834309mais citado
  3. Quality of life questionnaire development and validation for adrenocortical carcinoma patients.
    European journal of endocrinology· 2026· PMID 41875393mais citado
  4. Adrenocortical Carcinoma: From genomics to patients.
    Annales d'endocrinologie· 2026· PMID 41866070mais citado
  5. ACR Appropriateness Criteria&#xae; Staging and Follow-Up of Adrenal Cancer.
    Journal of the American College of Radiology : JACR· 2026· PMID 41863523mais citado
  6. Case Report: Peritoneal disease from adrenal cortical carcinoma with hepatic metastases managed with cytoreductive surgery and multiple HIPEC sessions, resulting in survival beyond 13 years.
    Front Surg· 2026· PMID 41993797recente
  7. Clinicopathologic Determinants of Overall Survival in Adrenocortical Carcinoma: A SEER-Based Population Study.
    Cancers (Basel)· 2026· PMID 41976326recente
  8. Pan-cancer analysis reveals FOLH1 as a prognostic biomarker and immune modulator in human tumors.
    Transl Cancer Res· 2026· PMID 41969500recente
  9. Management of Adrenal Incidentalomas: A Survey of Physicians from the Middle East and Africa.
    Tunis Med· 2025· PMID 41949969recente
  10. Normalization of Potassium Despite 11-Deoxycorticosterone Rise During Etomidate Therapy in Adrenocortical Carcinoma.
    JCEM Case Rep· 2026· PMID 41940234recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:1501(Orphanet)
  2. MONDO:0006639(MONDO)
  3. GARD:558(GARD (NIH))
  4. Variantes catalogadas(ClinVar)
  5. Busca completa no PubMed(PubMed)
  6. Q918285(Wikidata)

Dados compilados pelo RarasNet a partir de fontes abertas (Orphanet, OMIM, MONDO, PubMed/EuropePMC, ClinicalTrials.gov, DATASUS, PCDT/MS). Este conteúdo é informativo e não substitui avaliação médica.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Carcinoma adrenocortical
Compêndio · Raras BR

Carcinoma adrenocortical

ORPHA:1501 · MONDO:0006639
Prevalência
1-9 / 1 000 000
Herança
Not applicable
CID-10
C74.0 · Neoplasia maligna do córtex da supra-renal
CID-11
Ensaios
24 ativos
Início
Childhood
Prevalência
0.75 (Europe)
MedGen
UMLS
C0206686
EuropePMC
Wikidata
Papers 10a
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