Raras
Buscar doenças, sintomas, genes...
Tumor gastrointestinal do estroma
ORPHA:44890CID-10 · C26.9CID-11 · 2B5BOMIM 606764DOENÇA RARA

O tumor estromal gastrointestinal (GIST) é o tipo mais comum de câncer que surge nas células de suporte do trato gastrointestinal (TGI). Geralmente aparece em adultos com mais de 40 anos (a média de idade é 63 anos) e é raro em crianças. Pode surgir em várias partes do TGI, sendo mais comum no estômago ou intestino delgado, mas também pode ocorrer, com menos frequência, no esôfago, apêndice, reto e cólon. Os GISTs podem não apresentar sintomas ou manifestar vários sinais inespecíficos, dependendo da localização e do tamanho do tumor, como perda de apetite, anemia, perda de peso, cansaço, desconforto ou sensação de inchaço/plenitude no abdômen, náuseas e vômitos. Também podem causar uma massa (caroço) no abdômen, sangue nas fezes e obstrução intestinal. Os GISTs também podem estar associados a síndromes familiares, como a tríade de Carney e a neurofibromatose tipo 1.

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Introdução

O que você precisa saber de cara

📋

O tumor estromal gastrointestinal (GIST) é o tipo mais comum de câncer que surge nas células de suporte do trato gastrointestinal (TGI). Geralmente aparece em adultos com mais de 40 anos (a média de idade é 63 anos) e é raro em crianças. Pode surgir em várias partes do TGI, sendo mais comum no estômago ou intestino delgado, mas também pode ocorrer, com menos frequência, no esôfago, apêndice, reto e cólon. Os GISTs podem não apresentar sintomas ou manifestar vários sinais inespecíficos, dependendo da localização e do tamanho do tumor, como perda de apetite, anemia, perda de peso, cansaço, desconforto ou sensação de inchaço/plenitude no abdômen, náuseas e vômitos. Também podem causar uma massa (caroço) no abdômen, sangue nas fezes e obstrução intestinal. Os GISTs também podem estar associados a síndromes familiares, como a tríade de Carney e a neurofibromatose tipo 1.

Pesquisas ativas
31 ensaios
373 total registrados no ClinicalTrials.gov
Publicações científicas
6.457 artigos
Último publicado: 2026 Jan-Dec
Medicamentos
5 registrados
SUNITINIB MALATE, IMATINIB MESYLATE, AVAPRITINIB

Tem tratamento?

5 medicamentos registrados
Ver detalhes, fases e interações →
SUNITINIB MALATEIMATINIB MESYLATEAVAPRITINIBRIPRETINIBSUNITINIB

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
1-5 / 10 000
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
13.0
Europe
Início
Adolescent
+ adult, childhood
🏥
SUS: Sem cobertura SUSScore: 0%
CID-10: C26.9
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Sinais e sintomas

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

Partes do corpo afetadas

🫃
Digestivo
12 sintomas
🧬
Pele e cabelo
3 sintomas
🩸
Sangue
1 sintomas
🧠
Neurológico
1 sintomas

+ 7 sintomas em outras categorias

Características mais comuns

90%prev.
Sarcoma
Muito frequente (99-80%)
90%prev.
Tumor estromal gastrointestinal
Muito frequente (99-80%)
90%prev.
Neoplasia do estômago
Muito frequente (99-80%)
55%prev.
Disfagia
Frequente (79-30%)
55%prev.
Obstrução intestinal
Frequente (79-30%)
55%prev.
Hemorragia gastrointestinal
Frequente (79-30%)
24sintomas
Muito frequente (3)
Frequente (6)
Ocasional (9)
Sem dados (6)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 24 características clínicas mais associadas, ordenadas por frequência.

Sarcoma
Muito frequente (99-80%)90%
Tumor estromal gastrointestinalGastrointestinal stroma tumor
Muito frequente (99-80%)90%
Neoplasia do estômagoNeoplasm of the stomach
Muito frequente (99-80%)90%
DisfagiaDysphagia
Frequente (79-30%)55%
Obstrução intestinalIntestinal obstruction
Frequente (79-30%)55%

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2026
Total histórico6.457PubMed
Últimos 10 anos200publicações
Pico2025115 papers
Linha do tempo
2026Hoje · 2026🧪 1990Primeiro 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

5 genes identificados com associação a esta condição. Padrão de herança: Autosomal dominant, Not applicable.

SDHCSuccinate dehydrogenase cytochrome b560 subunit, mitochondrialCandidate gene tested inTolerante
FUNÇÃO

Membrane-anchoring subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:9533030). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (3)
Maturation of TCA enzymes and regulation of TCA cycleCitric acid cycle (TCA cycle)Respiratory electron transport
MECANISMO DE DOENÇA

Pheochromocytoma/paraganglioma syndrome 3

A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL3 inheritance is autosomal dominant.

EXPRESSÃO TECIDUAL(Ubíquo)
Fibroblastos
21.2 TPM
Glândula adrenal
19.6 TPM
Músculo esquelético
18.4 TPM
Cérebro - Hemisfério cerebelar
16.9 TPM
Linfócitos
16.5 TPM
OUTRAS DOENÇAS (6)
gastrointestinal stromal tumorCarney-Stratakis syndromepheochromocytoma/paraganglioma syndrome 3hereditary pheochromocytoma-paraganglioma
HGNC:10682UniProt:Q99643
KITMast/stem cell growth factor receptor KitDisease-causing germline mutation(s) (gain of function) inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for the cytokine KITLG/SCF and plays an essential role in the regulation of cell survival and proliferation, hematopoiesis, stem cell maintenance, gametogenesis, mast cell development, migration and function, and in melanogenesis. In response to KITLG/SCF binding, KIT can activate several signaling pathways. Phosphorylates PIK3R1, PLCG1, SH2B2/APS and CBL. Activates the AKT1 signaling pathway by phosphorylation of PIK3R1, the regulator

LOCALIZAÇÃO

Cell membraneCytoplasm

VIAS BIOLÓGICAS (5)
Developmental Lineage of Mammary Gland Luminal Epithelial CellsDevelopmental Lineage of Mammary Gland Alveolar CellsTFAP2 (AP-2) family regulates transcription of growth factors and their receptorsSignaling by SCF-KITRegulation of KIT signaling
MECANISMO DE DOENÇA

Piebald trait

Autosomal dominant genetic developmental abnormality of pigmentation characterized by congenital patches of white skin and hair that lack melanocytes.

EXPRESSÃO TECIDUAL(Ubíquo)
Esôfago - Muscular
44.5 TPM
Ovário
36.4 TPM
Esôfago - Junção
35.3 TPM
Cérebro - Hemisfério cerebelar
29.8 TPM
Tireoide
28.1 TPM
OUTRAS DOENÇAS (21)
gastrointestinal stromal tumorcutaneous mastocytosisacute myeloid leukemiapiebaldism
HGNC:6342UniProt:P10721
PDGFRAPlatelet-derived growth factor receptor alphaDisease-causing somatic mutation(s) inAltamente restrito
FUNÇÃO

Tyrosine-protein kinase that acts as a cell-surface receptor for PDGFA, PDGFB and PDGFC and plays an essential role in the regulation of embryonic development, cell proliferation, survival and chemotaxis. Depending on the context, promotes or inhibits cell proliferation and cell migration. Plays an important role in the differentiation of bone marrow-derived mesenchymal stem cells. Required for normal skeleton development and cephalic closure during embryonic development. Required for normal dev

LOCALIZAÇÃO

Cell membraneCell projection, ciliumGolgi apparatus

VIAS BIOLÓGICAS (1)
Signaling by PDGF
EXPRESSÃO TECIDUAL(Ubíquo)
Ovário
217.5 TPM
Fibroblastos
164.2 TPM
Cervix Endocervix
100.0 TPM
Fallopian Tube
89.1 TPM
Útero
77.5 TPM
OUTRAS DOENÇAS (8)
polyps, multiple and recurrent inflammatory fibroid, gastrointestinalidiopathic hypereosinophilic syndromeprimary hypereosinophilic syndromegastrointestinal stromal tumor
HGNC:8803UniProt:P16234
SDHBSuccinate dehydrogenase [ubiquinone] iron-sulfur subunit, mitochondrialCandidate gene tested inTolerante
FUNÇÃO

Iron-sulfur protein (IP) subunit of the succinate dehydrogenase complex (mitochondrial respiratory chain complex II), responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:26925370, PubMed:27604842). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate (By similarity)

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (1)
Maturation of TCA enzymes and regulation of TCA cycle
MECANISMO DE DOENÇA

Pheochromocytoma/paraganglioma syndrome 4

A form of pheochromocytoma/paraganglioma syndrome, a tumor predisposition syndrome characterized by the development of neuroendocrine tumors, usually in adulthood. Pheochromocytomas are catecholamine-producing tumors that arise from chromaffin cells in the adrenal medulla. Paragangliomas develop from sympathetic paraganglia in the thorax, abdomen, and pelvis, as well as from parasympathetic paraganglia in the head and neck. PPGL4 inheritance is autosomal dominant.

EXPRESSÃO TECIDUAL(Ubíquo)
Músculo esquelético
177.6 TPM
Coração - Ventrículo esquerdo
168.2 TPM
Linfócitos
144.5 TPM
Fígado
124.9 TPM
Coração - Átrio
122.8 TPM
OUTRAS DOENÇAS (9)
Carney-Stratakis syndromepheochromocytoma/paraganglioma syndrome 4gastrointestinal stromal tumormitochondrial complex 2 deficiency, nuclear type 4
HGNC:10681UniProt:P21912
SDHASuccinate dehydrogenase [ubiquinone] flavoprotein subunit, mitochondrialDisease-causing germline mutation(s) (loss of function) inTolerante
FUNÇÃO

Flavoprotein (FP) subunit of succinate dehydrogenase (SDH) that is involved in complex II of the mitochondrial electron transport chain and is responsible for transferring electrons from succinate to ubiquinone (coenzyme Q) (PubMed:10746566, PubMed:24781757). SDH also oxidizes malate to the non-canonical enol form of oxaloacetate, enol-oxaloacetate (By similarity). Enol-oxaloacetate, which is a potent inhibitor of the succinate dehydrogenase activity, is further isomerized into keto-oxaloacetate

LOCALIZAÇÃO

Mitochondrion inner membrane

VIAS BIOLÓGICAS (1)
Maturation of TCA enzymes and regulation of TCA cycle
MECANISMO DE DOENÇA

Mitochondrial complex II deficiency, nuclear type 1

A disorder of the mitochondrial respiratory chain with heterogeneous clinical manifestations. Clinical features include psychomotor regression in infants, poor growth with lack of speech development, severe spastic quadriplegia, dystonia, progressive leukoencephalopathy, muscle weakness, exercise intolerance, cardiomyopathy. Some patients manifest Leigh syndrome or Kearns-Sayre syndrome. MC2DN1 inheritance is autosomal recessive.

EXPRESSÃO TECIDUAL(Ubíquo)
Coração - Ventrículo esquerdo
302.0 TPM
Músculo esquelético
237.6 TPM
Coração - Átrio
206.8 TPM
Glândula adrenal
172.2 TPM
Linfócitos
168.3 TPM
OUTRAS DOENÇAS (9)
mitochondrial complex II deficiency, nuclear type 1pheochromocytoma/paraganglioma syndrome 5neurodegeneration with ataxia and late-onset optic atrophydilated cardiomyopathy 1GG
HGNC:10680UniProt:P31040

Medicamentos e terapias

SUNITINIB MALATEPhase 4

Mecanismo: Vascular endothelial growth factor receptor inhibitor

IMATINIB MESYLATEPhase 4

Mecanismo: Tyrosine-protein kinase ABL inhibitor

AVAPRITINIBPhase 4

Mecanismo: Platelet-derived growth factor receptor alpha inhibitor

RIPRETINIBPhase 4

Mecanismo: Platelet-derived growth factor receptor alpha inhibitor

SUNITINIBPhase 4

Mecanismo: Vascular endothelial growth factor receptor inhibitor

Ver mais no OpenTargets

Variantes genéticas (ClinVar)

1,385 variantes patogênicas registradas no ClinVar.

🧬 SDHA: GRCh38/hg38 5p15.33-15.32(chr5:113462-5578808)x1 ()
🧬 SDHA: NM_004168.4(SDHA):c.1517dup (p.Arg507fs) ()
🧬 SDHA: NM_004168.4(SDHA):c.896-1G>A ()
🧬 SDHA: NM_004168.4(SDHA):c.1648A>T (p.Lys550Ter) ()
🧬 SDHA: NM_004168.4(SDHA):c.567C>A (p.Cys189Ter) ()
Ver todas no ClinVar

Classificação de variantes (ClinVar)

Distribuição de 8,055 variantes classificadas pelo ClinVar.

5236
2819
VUS (65.0%)
Benigna (35.0%)
VARIANTES MAIS SIGNIFICATIVAS
PDGFRA: NM_006206.6(PDGFRA):c.1925T>C (p.Met642Thr) [Uncertain significance]
PDGFRA: NM_006206.6(PDGFRA):c.2883T>G (p.Ser961Arg) [Uncertain significance]
KIT: NM_000222.3(KIT):c.2914G>C (p.Val972Leu) [Uncertain significance]
PDGFRA: NM_006206.6(PDGFRA):c.2270T>C (p.Ile757Thr) [Uncertain significance]
PDGFRA: NM_006206.6(PDGFRA):c.211A>G (p.Ile71Val) [Uncertain significance]

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.
Aprovado5
2Fase 210
1Fase 13
·Pré-clínico7
Medicamentos catalogadosEnsaios clínicos· 5 medicamentos · 20 ensaios
✓ Aprovados — podem ser usados hoje
SUNITINIB MALATEIMATINIB MESYLATEAVAPRITINIBRIPRETINIBSUNITINIB
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Tumor gastrointestinal do estroma

🗺️

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

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

NCT07434843 · PH 2 Pemigatinib in SDH-deficient GISTRecrutando
PHASE2
NCT07406633 · KQB198 in Combination With Imatinib in Participants With Adv…Recrutando
PHASE2
NCT06748690 · MCB vs EUS-FNA for Preoperative Pathological Evaluation of G…Recrutando
NA
NCT04557969 · Surgery in Gastrointestinal Stromal Tumors (GISTs) for Treat…Recrutando
NCT04924075 · Belzutifan/MK-6482 for the Treatment of Advanced Pheochromoc…Recrutando
PHASE2
NCT07522762 · Long-term Survivorship Challenges of Advanced/Metastatic GIS…Recrutando
NCT03594422 · A Study of HQP1351 in Patients With GIST or Other Solid Tumo…Recrutando
PHASE1
NCT06326346 · GIST Oral Paclitaxel(Liporaxel)Recrutando
PHASE2
NCT05905887 · Rivoceranib Plus Paclitaxel in Patients With Gastrointestina…Recrutando
PHASE2
NCT05661643 · The Efficacy and Safety of Temozolomide in SDH-deficient GIS…Recrutando
PHASE2
NCT07124000 · DESTINY-PANTUMOUR04Recrutando
NCT07171203 · Neoadjuvant Imatinib and Fampridine in KIT Mutant Gastrointe…Recrutando
PHASE1
NCT05500391 · Assessment of Compliance With Monitoring Conducted by a Phys…Recrutando
PHASE2
NCT05493215 · Imatinib TDM in GISTRecrutando
PHASE2
NCT07411118 · RCT of EFTR Versus STER for GIST TreatmentRecrutando
NA
NCT07405125 · Safety of Robotic Surgery for GISTs at Special Anatomic Site…Recrutando
NA
NCT06630234 · A Master Protocol to Evaluate DCC-3009 in Gastrointestinal S…Recrutando
PHASE1, PHASE2
NCT06655246 · A Study of Ziftomenib in Combination With Imatinib in Patien…Recrutando
PHASE1
NCT02012699 · Integrated Cancer Repository for Cancer ResearchRecrutando
NCT05152472 · A Prospective, Randomized, Multicenter, Comparative Study of…Recrutando
PHASE2

Outros ensaios clínicos

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Publicações mais relevantes

Timeline de publicações
3.854 papers (10 anos)

Mostrando amostra de 200 publicações de um total de 3.854

#1

Endoscopic submucosal dissection and submucosal tunneling endoscopic resection of gastric subepithelial lesions originating from the muscle layer: a multicenter retrospective study.

Annals of gastroenterology2026

Gastric subepithelial lesions (SELs) from the muscularis propria are usually managed surgically or with surveillance. Advances in endoscopic techniques now permit safe en bloc resection, even for lesions with extraluminal growth, but data from outside East Asia are scarce. We performed a multicenter retrospective study across 18 centers in Europe, the Middle East, South America and South Asia, including patients with gastric SELs from the muscularis propria resected by endoscopic submucosal dissection (ESD) or submucosal tunneling endoscopic resection (STER) between 2017 and 2024. Outcomes included en bloc resection, safety, complications, and recurrence. Eighty-two patients (62.2% women; median age 59 years) were included. Median lesion size was 2cm (range 0.6-8). Lesions were located in the cardia (18.3%), fundus (18.3%), body (40.2%), and antrum (23.2%). Histology showed gastrointestinal stromal tumors in 78.0%, leiomyomas in 19.5%, and schwannomas in 2.5%. Most were endoluminal (80.5%), 19.5% were extraluminal, and 7.3% extended beyond the serosa. ESD was performed in 65.9% and STER in 34.1%. Median procedure time was 120 min. Deliberate perforation was performed in 31.7%, while 92.3% were managed endoscopically. En bloc resection was achieved in 96.3%, with defect closure in 90.2%. Complications occurred in 7%, with no mortality. Local relapse occurred in 2.5% after a median 9-month follow up. One-third of procedures took place in non-academic centers. ESD and STER are safe and effective alternatives to surgery or surveillance for gastric SELs from the muscularis propria, including lesions with extraluminal growth. Long-term, comparative studies alongside laparoscopic surgery are warranted.

#2

Systematic Screening via WGCNA, Machine Learning, and Validation Identifies C3 and CFD as Prognostically Significant Regulators in GIST Progression.

Molecular and cellular biology2026 Feb 24

Gastrointestinal stromal tumor (GIST), the most common gastrointestinal mesenchymal neoplasm, remains poorly understood at the molecular level, limiting precise diagnosis and targeted therapy. This study aimed to systematically identify key GIST-associated genes through multiomic integration and experimental validation. We analyzed three GIST transcriptomic datasets from GEO, corrected batch effects via surrogate variable analysis (SVA), and identified 61 differentially expressed genes (DEGs) using limma. Weighted gene co-expression network analysis (WGCNA) highlighted progression-related modules, which were refined using random forests and LASSO regression to prioritize C3 and complement factor D (CFD), both of which showed robust diagnostic performance (AUC: 0.928 for C3; 0.955 for CFD). Experimental validation confirmed C3/CFD downregulation in GIST tissues, correlating with advanced stage and poor survival. Functional assays demonstrated their tumor-suppressive roles, inhibiting GIST cell proliferation, colony formation, and migration. CIBERSORT analysis linked C3/CFD to altered immune infiltration, while ssGSEA/GSEA implicated their involvement in lipid metabolism and oxidative phosphorylation. These findings establish C3 and CFD as critical tumor-suppressive biomarkers that modulate the immune response and reprogram metabolism, offering new avenues for GIST diagnosis and therapy.

#3

Golgi Retention and Oncogenic Signaling of KIT Tyrosine Kinase in Gastrointestinal Stromal Tumor.

Sub-cellular biochemistry2026

KIT, a receptor protein tyrosine kinase (RTK), is localized in the plasma membrane and binds stem cell factor. A cancer-causing permanently active mutant of KIT (KITmut) accumulates aberrantly in intracellular compartments. Oncogenic RTK mutants other than KITmut also exhibit characteristic organelle localization, unlike their wild-type counterparts. However, the precise subcellular localization of RTKmut and its biological significance in oncogenic signaling remain unclear. Recent studies have shown that in gastrointestinal stromal tumors (GISTs), KITmut is inappropriately retained in the Golgi/trans-Golgi network (TGN) area in a manner dependent on its tyrosine kinase activity. It is trapped in the biosynthetic secretory pathway in which normal RTK trafficking occurs. Importantly, KITmut is activated and exerts oncogenic signaling predominantly in the Golgi/TGN of GIST cells. Recently, key players in the Golgi retention of KITmut have been reported, but they are unlikely to be involved in the intracellular retention of other RTKs, suggesting that the retention of individual RTKs is regulated by different molecular mechanisms. Here, we review the present knowledge on KIT retention in the Golgi/TGN region in GISTs, molecular mechanisms underlying this retention, and the aberrant localization of other RTK mutants. We also discuss strategies for the medical development of RTK inhibition by blocking intracellular trafficking.

#4

Gastric bronchogenic cyst pooled case analysis: a case report and systematic review.

BMC gastroenterology2026 Feb 13

Gastric bronchogenic cyst constitutes a rare form of ectopic bronchogenic cyst, with an estimated incidence of less than 1 in 68,000 to 1 in 42,000. This study reported a case who was preoperatively misdiagnosed as gastrointestinal stromal tumor and reviewed the literature on gastric bronchogenic cyst to summarize its clinical features, diagnosis, treatment, pathological manifestations, and prognosis. A systematic search of the literature in the PubMed, Embase, Cochrane Library, Web of Science and Scopus databases was conducted by two independent reviewers focusing on cases of gastric bronchogenic cyst. Firstly, we report a rare case of bronchogenic cyst in a middle-aged female, with the lesion located near the gastric fundus. After literature review, 48 cases of gastric bronchogenic cyst (male-to-female ratio of 1:1.67, mean age was 47.54 ± 15.83 years) derived from 46 studies were enrolled. Abdominal pain (45.8%) was the most prevalent symptom, whereas 15 patients (31.3%) remained asymptomatic. Gastric bronchogenic cysts predominantly affected the gastric fundus (33.3%) and cardia (27.1%). Gastrointestinal stromal tumor (37.5%) was the most frequent misdiagnosis, while 9 patients (18.8%) were misdiagnosed as cysts originating from other abdominal organs. Laparoscopic surgery was the primary treatment (50.0%), followed by laparotomy (35.4%). Median follow-up was 10 months, no postoperative recurrence or mortality was reported in the pooled cases. Gastric bronchogenic cyst is a rare and diagnostically challenging congenital abnormality with limited evidence to guide preoperative diagnosis. It should be incorporated into the differential diagnosis of gastrointestinal neoplasms, surgical resection is regarded as the effective approach for definitive diagnosis treatment.

#5

Prediction of Mutations and Outcome in Gastrointestinal Stromal Tumors with Deep Learning: A Multicenter, Multinational Study.

medRxiv : the preprint server for health sciences2026 Feb 03

Gastrointestinal stromal tumor (GIST) is the most common gastrointestinal mesenchymal tumor, driven by tyrosine-protein kinase KIT and platelet-derived growth factor receptor A (PDGFRA) mutations. Specific variants, such as KIT exon 11 deletions, carry prognostic and therapeutic implications, whereas wild-type (WT) variants derive limited benefit from tyrosine kinase inhibitors (TKIs). Given the limited reproducibility of established clinicopathological risk models, deep learning (DL) applied to whole-slide images (WSIs) emerged as a promising tool for molecular classification and prognostic assessment. We analyzed 8398 GIST cases from 21 centers in 7 countries, including 7238 with molecular data and 2638 with clinical follow-up. DL models were trained on WSIs to predict mutations, treatment sensitivity, and recurrence-free survival (RFS). DL predicted mutational status in GIST from WSIs, with area under the curve (AUC) of 0.87 for KIT, 0.96 for PDGFRA. High performance was observed for subtypes, including KIT exon 11 del-inss 557-558 (0.67) and PDGFRA exon 18 D842V (0.93). For therapeutic categories, performance reached 0.84 for avapritinib sensitivity, 0.81 for imatinib sensitivity. DL models predicted RFS, with hazard-ratios (HR) of 8.44 (95%CI 6.14-11.61) in the overall cohort and 4.74 (95%CI 3.34-6.74) in patients receiving adjuvant therapy. Prognostic performance was comparable to pathology-based scores, with highest discrimination in the overall cohort and in patients without adjuvant therapy (9.44, 95%CI (5.87-15.20)). DL applied to WSIs enables prediction of molecular alterations, treatment sensitivity, and RFS in GIST, performing comparably to established risk scores across international cohorts, providing a baseline for future multimodal predictors.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC3.563 artigos no totalmostrando 198

2026

CARMN::NOTCH2 Fusion-Positive Malignant Glomus Tumor of the Liver: A Rare Tumor With Review of Literature.

International journal of surgical pathology
2026

Gastric Neuroendocrine Tumor: A Report of a Rare Case.

Cureus
2026

Endoscopic submucosal dissection and submucosal tunneling endoscopic resection of gastric subepithelial lesions originating from the muscle layer: a multicenter retrospective study.

Annals of gastroenterology
2026

A giant gastrointestinal stromal tumour mimicking gastric diverticulum: a case report.

Journal of surgical case reports
2026

[Full-course perioperative management of gastrointestinal stromal tumors: the evolution of modern surgical practice from scalpel to systems engineering].

Zhonghua wei chang wai ke za zhi = Chinese journal of gastrointestinal surgery
2026

Laparoscopic partial gastrectomy for gastric calcifying fibrous tumor: a case report and literature review.

International journal of surgery case reports
2026

Benign Keratoses With Inverted Follicular Keratosis-Like Features in a Patient Receiving Ripretinib: A Case Report.

Journal of cutaneous pathology
2026

Ruptured jejunal gastrointestinal stromal tumor (GIST) presenting with acute hemoperitoneum in a young adult with autosomal dominant polycystic kidney disease (ADPKD).

Journal of surgical case reports
2026

Concomitant occurrence of primary gastric sarcomatoid carcinoma and giant gastrointestinal stromal tumor: a case report and literature review.

Frontiers in oncology
2026

Unmasking the Mimic: Clinicopathologic Insights from Gastrointestinal Leiomyosarcoma.

International journal of surgical pathology
2026

Large Pancreatic Gastrointestinal Stromal Tumor: A Diagnostic Quandary.

Cureus
2026

Massive gastrointestinal stromal tumor with exophytic growth mimicking pelvic leiomyosarcoma: a case report and review of the literature.

International journal of surgery case reports
2026

Beyond Laparotomy: Multimodal Strategies and Emerging Evidence in Minimally Invasive Management of Gastric Gastrointestinal Stromal Tumors.

BioMed research international
2026

Impact of neoadjuvant imatinib duration and tumor response on recurrence-free survival in locally advanced gastrointestinal stromal tumors: a retrospective multicenter cohort study.

World journal of surgical oncology
2026

Design and Synthesis of BLU-654, a Potent and Selective Mutant KIT V654A Inhibitor for the Treatment of Imatinib-Resistant GIST.

Journal of medicinal chemistry
2026

Divergent manifestations and management of GISTs of the small intestine: a case series.

Journal of surgical case reports
2026

Surgical management of gastrointestinal stromal tumor (GIST)-Induced sarcomatosis (GISTOSIS).

Surgical oncology
2026

Quality-of-life outcomes with pimitespib in patients with advanced gastrointestinal stromal tumor: results from the randomized, double-blind, placebo-controlled, phase III, CHAPTER-GIST-301 study.

ESMO open
2026

Comparative Evaluation of Endoscopic Ultrasonography and Multi-Slice Spiral CT in Diagnosing Gastrointestinal Stromal Tumors: A Retrospective Study.

International journal of general medicine
2026

Ripretinib-Induced Focal Segmental Glomerulosclerosis in a Patient With Gastrointestinal Stromal Tumor (GIST).

Cureus
2026

Multifocal small bowel gastrointestinal stromal tumor causing perforation peritonitis: A rare cause of acute abdomen.

Radiology case reports
2026

Prediction model of malignant risk of gastrointestinal stromal tumor based on endoscopic ultrasonography.

Digestive diseases (Basel, Switzerland)
2026

Navigating the complex landscape of duodenal gastrointestinal stromal tumor: Innovations in diagnosis and treatment.

Critical reviews in oncology/hematology
2026

Delayed Rupture of Subcapsular Hepatic Gastrointestinal Stromal Tumor Metastasis Following Percutaneous Biopsy.

Journal of vascular and interventional radiology : JVIR
2026

Duodenal ampullary neuroendocrine tumor, high risk gastrointestinal stromal tumor, and gastric leiomyoma in a patient with neurofibromatosis type 1: a rare case report and literature review.

Frontiers in oncology
2026

Use of the Alexis® Wound Protector for Exposure and Sterility in Perianal Oncologic Surgery: A Technical Report.

Cureus
2026

Systematic Screening via WGCNA, Machine Learning, and Validation Identifies C3 and CFD as Prognostically Significant Regulators in GIST Progression.

Molecular and cellular biology
2026

Pulmonary Arterial Hypertension and Cancer: Unveiling Parallels in Epidemiology, Clinical Pathways, and Therapeutic Strategies.

Journal of market access &amp; health policy
2026

Golgi Retention and Oncogenic Signaling of KIT Tyrosine Kinase in Gastrointestinal Stromal Tumor.

Sub-cellular biochemistry
2026

Endoscopic resection of a gastrointestinal stromal tumor using a new mucosal dissection knife in combination with far-view endoscopic submucosal dissection.

Endoscopy
2026

BRAF V600E Expression in c-Kit+ Interstitial Cells of Cajal Drives Gastrointestinal Stromal Tumor Formation in Mice.

Cancer research communications
2026

Endoscopic vs laparoscopic resection for gastric gastrointestinal stromal tumors: Oncological outcomes.

World journal of gastrointestinal oncology
2026

Unicentric Castleman Disease of the Mesentery Mimicking a Gastrointestinal Stromal Tumor: A Case Report.

Cureus
2026

Peritoneal Sarcomatosis Secondary to a Gastrointestinal Stromal Tumor Treated With a Multimodal Approach: A Case Report.

Cureus
2026

Incidental finding of gastrointestinal stromal tumor in appendix.

Journal of surgical case reports
2026

Intratumoral and Peritumoral Radiomics Based on Contrast-Enhanced CT Images: Biomarkers to Predict the Risk-Grade of Gastrointestinal Stromal Tumors.

Current medical imaging
2026

Gastric bronchogenic cyst pooled case analysis: a case report and systematic review.

BMC gastroenterology
2026

Survival analysis and prognostic factors for gastrointestinal and extragastrointestinal stromal tumors: a SEER-based study.

Annals of medicine and surgery (2012)
2026

Prediction of Mutations and Outcome in Gastrointestinal Stromal Tumors with Deep Learning: A Multicenter, Multinational Study.

medRxiv : the preprint server for health sciences
2026

Gastrointestinal Stromal Tumors: Histopathological Spectrum, Molecular Subtypes, and Implications for Targeted Therapy.

Cureus
2026

Augmenting CT evaluation of primary small bowel malignancies: The role of cinematic rendering.

Emergency radiology
2026

Novel therapeutic ultrathin endoscope facilitates endoscopic intermuscular dissection for gastrointestinal stromal tumor adjacent to the anus.

Endoscopy
2026

The Successful Treatment of Primary and Recurrent Ruptured Gastric Gastrointestinal Stromal Tumor (GIST) Through Coordinated Surgical Intervention and c-Kit Inhibitor Therapy.

Cureus
2026

Laparoscopic resection for high-risk gastric gastrointestinal stromal tumors: safety and oncological outcome.

Surgical endoscopy
2026

Pelvic Gastrointestinal Stromal Tumor (GIST) Mimicking Ovarian Cancer in a Kidney-Liver Transplant Recipient Under Chronic Immunosuppression.

Case reports in oncological medicine
2025

Submucosal tunneling endoscopic resection with bidirectional full-thickness resection of gastrointestinal stromal tumor: a case report.

iGIE : innovation, investigation and insights
2026

Laparoscopic resection for gastric gastrointestinal stromal tumor at the esophagogastric junction: feasibility and long-term results.

Updates in surgery
2026

Two in one: paraganglioma and gastrointestinal stromal tumor presentation of Carney-Stratakis syndrome.

Polish archives of internal medicine
2026

Laparoscopic Resection of a Large Jejunal Diverticulum-Like Gastrointestinal Stromal Tumor: A Case Report.

Surgical case reports
2025

Intra-abdominal Desmoid Tumors Mimicking Gastrointestinal Stromal Tumor (GIST) Recurrence: A Case Report.

Cureus
2026

Rare co-occurrence of gastrointestinal stromal tumors and leiomyomas: A case report and review of the literature.

Experimental and therapeutic medicine
2026

Submucosal colonic fecalith mimicking a submucosal malignant tumor at the anastomotic site after colon cancer: A case report.

Experimental and therapeutic medicine
2026

DOG1 Immunohistochemical Expression in Normal and Neoplastic Canine Tissues: Is It Only a Marker for GISTs?

Animals : an open access journal from MDPI
2026

Clinicopathological features of PDGFRA D842Y-mutant gastrointestinal stromal tumors: insights from four cases.

International cancer conference journal
2025

Enhancing gastrointestinal stromal tumor risk stratification: A novel deep learning approach applied to EUS imaging.

Endoscopic ultrasound
2025

A new classification of gastric submucosal tumors under EUS based on blood flow signals.

Endoscopic ultrasound
2025

Primary Gastrointestinal Stromal Tumor of the Pancreatic Head: A Diagnostic Challenge Mimicking a Neuroendocrine Tumor.

Cureus
2026

[Cytoreductive surgery in the management of recurrent or metastatic gastrointestinal stromal tumors: a retrospective analysis from a single-center experience].

Zhonghua wai ke za zhi [Chinese journal of surgery]
2026

Gastroduodenal Intussusception With Gastric Outlet Obstruction and Periampullary Compression Due to a Gastrointestinal Stromal Tumor in the Stomach.

The American journal of case reports
2026

AOC3 Loss Promotes Imatinib Resistance in GIST by Stabilizing HK2 and Enhancing H3K18la-Driven Myc Transcription.

Molecular carcinogenesis
2026

Synchronous Rectal Adenocarcinoma and Sigmoid Gastrointestinal Stromal Tumor: A Case Report.

Case reports in oncology
2026

Analysis of Risk Factors for Postoperative Delayed Perforation Following Endoscopic Submucosal Dissection of Gastrointestinal Stromal Tumors.

Surgical laparoscopy, endoscopy &amp; percutaneous techniques
2026

Clinicopathologic and molecular spectrum of gastrointestinal stromal tumor (GIST) with NTRK fusion and marked response to larotrectinib in GIST with NTRK fusion: a case report.

Cellular oncology (Dordrecht, Netherlands)
2026

Unexpected metachronous multiple primary cancers: pilonidal sinus‑related squamous cell carcinoma and incidentally detected gastric gastrointestinal stromal tumour.

BJR case reports
2026

Gastric Heterotopic Pancreas Mimicking A Submucosal Tumor: A Case Report.

Clinical case reports
2025

[A Case of Gastric GIST Concomitant with Early Gastric Cancer and Leiomyoma].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2025

[A Case of Intraperitoneal Gastrointestinal Stromal Tumor with Pedicle Torsion Requiring Emergency Surgery].

Gan to kagaku ryoho. Cancer &amp; chemotherapy
2025

A case report of jejunal myeloid sarcoma presented with intestinal intussusception in a 19-year-old man: An uncommon presentation of a rare disease.

International journal of surgery case reports
2026

Sequential Endoscopy-Contrast-Dnhanced CT Protocol for Rapid Localization of Bleeding Small-Bowel Gastrointestinal Stromal Tumor.

Digestive diseases and sciences
2026

Rectal PEComa Mimicking Gastrointestinal Stromal Tumor on Endoscopic Ultrasound.

ACG case reports journal
2026

Diagnostic Value of Endoscopic Ultrasound-Guided Tissue Acquisition With Rapid On-Site Evaluation in Small Gastric Subepithelial Lesions.

DEN open
2025

The clinical efficacy and pathological assessment of neoadjuvant imatinib treatment in patients with primary gastrointestinal stromal tumors.

Journal of gastrointestinal oncology
2026

Endoscopic Diagnosis of Gastric Subepithelial Lesions < 20 mm: Current Strategies and Emerging Solutions.

Digestive endoscopy : official journal of the Japan Gastroenterological Endoscopy Society
2026

The Evolving Role of Second- and Third-Generation Tyrosine Kinase Inhibitors in Gastrointestinal Malignancies: Advances in Targeted Therapy with Sunitinib, Regorafenib, and Avapritinib.

Journal of clinical medicine
2026

Contrast-Enhanced Harmonic Endoscopic Ultrasonography for Diagnosing Gastric Subepithelial Tumors.

Diagnostics (Basel, Switzerland)
2025

A Rare Case of Gastric Glomangioma Mimicking a Gastrointestinal Stromal Tumor (GIST).

Cureus
2026

An incidental finding of duodenal gastrointestinal stromal tumor (dGIST): a case report.

Annals of medicine and surgery (2012)
2026

An intercostal artery aneurysm rupture in a patient with neurofibromatosis type 1-associated gastrointestinal stromal tumor during sunitinib therapy.

Clinical journal of gastroenterology
2025

A case of Meckel's diverticulum misdiagnosed as gastrointestinal stromal tumor: case report and literature review.

Frontiers in medicine
2026

Successful preoperative diagnosis and laparoscopic endoscopic cooperative surgery for gastric plexiform fibromyxoma: a case report.

Clinical journal of gastroenterology
2026

A Giant Lower Rectal Gastrointestinal Stromal Tumor Treated with Modified Endoscopic Submucosal Dissection.

Digestive diseases and sciences
2025

Hypercalcemia episodes caused by ectopic parathyroid adenoma and subsequent gastrointestinal stromal tumor: A case report and literature review.

Frontiers in oncology
2025

Laparoscopic Wedge Resection of a 6 cm Gastrointestinal Stromal Tumor (GIST) Arising From the Lesser Curvature: A Case Report From Jordan.

Cureus
2025

A rare entity of esophageal gastrointestinal stromal tumor with literature review.

Journal of cancer research and therapeutics
2026

Five Cases of Hepatic Mesenchymal Tumors Diagnosed by Endoscopic Ultrasound-Guided Tissue Acquisition.

DEN open
2025

Ki-67 and Tumor Size in Small Bowel Tumors: Findings from an Exploratory Immunohistochemical Analysis.

Life (Basel, Switzerland)
2025

Recurrent gastrointestinal stromal tumor with c-KIT double exon mutations: A rare case report.

CytoJournal
2026

Gastrointestinal stromal tumor liver metastasis rupture following yttrium-90 radioembolization: A previously unreported complication.

Radiology case reports
2026

Subtype classification of gastric spindle cell tumors in whole slide images.

Computers in biology and medicine
2026

Gastrointestinal stromal tumor of the rectum mimicking a prostatic mass: case report.

Radiology case reports
2025

Sporadic Multiple Gastrointestinal Stromal Tumors With Distinct KIT and PDGFRA Mutations in Two Separate Tumors: A Case Report and Literature Review.

International journal of surgical pathology
2025

Intraperitoneal tuberculous abscess mimicking a para-gastric mass: A case report and literature review.

Medicine
2026

The efficacy of immunotherapy in the treatment of tyrosine kinase inhibitor-refractory gastrointestinal stromal tumors: a systematic review and meta-analysis.

Journal of gastrointestinal surgery : official journal of the Society for Surgery of the Alimentary Tract
2025

Laparoscopic and Endoscopic Cooperative Surgery Outcomes for Gastric Submucosal Tumors and Our Approach Using the Positional Change Technique.

Indian journal of surgical oncology
2025

Three-dimensional bioprinting of patient-derived Gastrointestinal stromal tumor: a novel platform for precision oncology and drug response profiling.

Journal of nanobiotechnology
2025

Performance and clinical utility of two targeted multigene panels for GIST molecular characterization.

Scientific reports
2025

Transanal minimally invasive surgery for rectal gastrointestinal stromal tumor using articulated forceps after neoadjuvant chemotherapy with imatinib mesylate.

International cancer conference journal
2025

Single-Stage Non-Exposed Endoscopic Wall-Inversion Surgery and Laparoscopic Pylorus-Preserving Gastrectomy for a Gastric Leiomyoma in the Cardia and Early Gastric Cancer in the Middle Stomach: A Case Report.

Asian journal of endoscopic surgery
2025

Robotic Approach to Multivisceral Resection for Gastrointestinal Stromal Tumor With En Bloc Resection of the Stomach, Distal Pancreas, and Spleen: A Case Report.

Cureus
2025

Small bowel GISTs: clinicopathological patterns and the role of inflammatory markers.

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

Primary extra-gastrointestinal stromal tumor of the gallbladder: A case report.

World journal of gastroenterology
2025

Chronic Disseminated Intravascular Coagulation in Patient With Metastatic Gastrointestinal Stromal Tumor: Treating Bleeding With Anticoagulation.

Journal of medical cases
2025

Merged gastrointestinal stromal tumor, intestinal obstruction, and perforation in Meckel's diverticulum: Case report.

Medicine
2025

Monitoring the plasma concentration of ripretinib and its metabolites in Chinese patients with gastrointestinal stromal tumors in a real-life setting.

Cancer
2025

Long-term outcomes of ripretinib versus sunitinib in Chinese patients with advanced gastrointestinal stromal tumor: An updated analysis of a phase 2 randomized clinical trial.

Cancer
2025

Development and validation of a machine-learning-based pathomics nomogram for predicting recurrence of localized primary gastrointestinal stromal tumors after complete surgical resection: A retrospective multicenter study.

Cancer
2025

Disparities in management guidelines for gastrointestinal stromal tumors: A comparison of recommendations from the Chinese Society of Clinical Oncology, National Comprehensive Cancer Network, and European Society for Medical Oncology.

Cancer
2025

Chinese expert consensus on diagnosis and treatment of small gastrointestinal stromal tumors (2025 edition).

Cancer
2026

Germ cell immunoprofile and KIT exon 17 mutation guide rectification of seminoma misclassified as epithelioid gastrointestinal stromal tumor: A case report.

Biomedical reports
2025

[Esophageal gastrointestinal stromal tumor with MKRN1::BRAF gene fusion: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2025

The Rare Upper Thoracic and Cervical Esophageal GIST Resected Through Thoracoscopic Esophageal Mobilization and a Cervical Approach: A Case Report.

Cancer reports (Hoboken, N.J.)
2025

Phenotypic Carney-Stratakis syndrome with DIS3L2 variant: a case challenging the current genetic paradigm.

Proceedings (Baylor University. Medical Center)
2025

A Prostatic Extra-Gastrointestinal Stromal Tumor Treated by Surgery: A Case Report with 7-Year Follow-Up and Review of Literature.

Archivos espanoles de urologia
2025

A Rare Case of Presumed Insulin-Like Growth Factor 2 (IGF-2)-Mediated Hypoglycemia.

Cureus
2025

Comparative efficacy of neoadjuvant imatinib therapy in gastric versus rectal gastrointestinal stromal tumors: a two-center retrospective cohort study.

BMC gastroenterology
2026

Retroperitoneal cavernous hemangioma: A case report with literature review.

Radiology case reports
2025

Robotic Full-Thickness Resection of a Type IV Gastric Gastrointestinal Stromal Tumor (GIST) Through Organoaxial Gastric Rotation: Technical Description With Video and Operative Management Insights.

Cureus
2025

Pre-emptive purse-string suture for full-thickness resection of a gastrointestinal stromal tumor in the duodenal bulb: a case report.

Endoscopy
2025

Endoscopic Resection of an Esophageal Gastrointestinal Stromal Tumor After Neoadjuvant Treatment: A New Paradigm for Minimally Invasive Therapy?

ACG case reports journal
2025

Age-dependent diminution of female prognostic advantage in gastrointestinal stromal tumors: a retrospective cohort analysis.

Frontiers in immunology
2025

NF1 Mutated Gastrointestinal Stromal Tumors With Coamplification of MDM2 and MYC.

Genes, chromosomes &amp; cancer
2025

Appraising Treatment for Gastrointestinal Stromal Tumors of the Wild-Type Mutation in the Setting of NF1: A Case Report.

International journal of hematology-oncology and stem cell research
2025

FGFR2 fusions as novel oncogenic drivers in gastrointestinal stromal tumors: Two case reports and review of literature.

World journal of gastrointestinal oncology
2025

Systematic elucidation and pharmacologic targeting on non-oncogene dependencies in imatinib-resistant gastrointestinal stromal tumor.

bioRxiv : the preprint server for biology
2025

Succinate dehydrogenase deficient GIST: Case series and review of literature from a tertiary care center in India.

Indian journal of cancer
2025

Single Incision Laparoscopic Local Resection for Gastric Gastrointestinal Stromal Tumors Compared to Multiport Laparoscopic Local Resection: A Single-Center Retrospective Study.

Asian journal of endoscopic surgery
2026

Incidental appendiceal neoplasms in a multicenter registry of appendicitis management.

Surgery
2026

Efficacy and safety of pimitespib in gastrointestinal tumors.

Surgery today
2025

A CT-based decision tree model for differentiating sub-3 cm gastric ectopic pancreas from gastrointestinal stromal tumors.

BMC gastroenterology
2025

Ultrasonographic Diagnosis of Sigmoid Colon Schwannoma: Report of One Case and Literature Review.

Zhongguo yi xue ke xue yuan xue bao. Acta Academiae Medicinae Sinicae
2025

Primary hemangioblastoma of rectum: a rare case report and review of literature.

Journal of cancer research and clinical oncology
2025

Local Excision of a 5-cm Rectal Gastrointestinal Stromal Tumor Using Transanal Minimally Invasive Surgery (TAMIS): A Case Report.

Asian journal of endoscopic surgery
2025

A Large Gastrointestinal Tumor With Cystic Degeneration Initially Mistaken for Gastric Splenosis: A Case Report.

Cureus
2025

Endoscopic full-thickness resection of a gastrointestinal stromal tumor using a double-endoscope snare technique.

Endoscopy international open
2025

Gastric Leiomyosarcoma: A Case Report and Literature Review.

Cureus
2025

Ampullary Gastrointestinal Stromal Tumor (GIST) - A Rare Culprit for Transient Biliary Obstruction.

Digestive diseases and sciences
2025

Iatrogenic diaphragmatic hernia - a serious delayed complication: Three cases report and literature review.

Medicine
2025

Enhanced resection of exophytic gastric gastrointestinal stromal tumor using traction-assisted subserosal dissection and endoscopic suturing.

Endoscopy
2025

Regression of Extensive Keloids During Imatinib Therapy for Gastrointestinal Stromal Tumor.

JAMA dermatology
2025

Radiomics models with baseline MRI and clinical data to predict target therapy response and high-risk mortality in metastatic GIST.

Scientific reports
2025

Development and validation of CT-based nomograms for predicting recurrence and prognosis in gastrointestinal stromal tumor.

Abdominal radiology (New York)
2026

Innovative development and validation of a scoring system for predicting electrocoagulation syndrome post-endoscopic submucosal dissection of gastric gastrointestinal stromal tumors.

Surgical endoscopy
2025

[Primary extra-gastrointestinal stromal tumor presenting as an isolated mediastinum mass: report of a case].

Zhonghua bing li xue za zhi = Chinese journal of pathology
2026

PEComas of the colon and rectum: a case report of a 15-year-old girl and a review of the pertinent literature.

Clinical journal of gastroenterology
2025

A Retrospective Analysis of the Efficacy and Safety of Imatinib for Advanced Gastrointestinal Stromal Tumor in Elderly Patients.

Cancer medicine
2025

Evaluation of the Ki-67 Labeling Index as a Prognostic Stratification Factor in Non-High-Risk Gastrointestinal Stromal Tumors.

Cureus
2025

Optimal Sunitinib Dosing Based on Platelet Count in an Older Patient With Gastrointestinal Stromal Tumor.

In vivo (Athens, Greece)
2025

Ruptured Gastrointestinal Stromal Tumor in a 12-Year-Old Child: A Case Report.

Cureus
2025

Gastric Adenocarcinoma Resembling Submucosal Tumor: A Case Report.

The American journal of case reports
2025

Molecular Mechanisms and Clinical Implications of Fibroblast Growth Factor Receptor 2 Signaling in Gastrointestinal Stromal Tumors.

Current issues in molecular biology
2025

Nuclear Pleomorphism in Gastrointestinal Stromal Tumors: Insights Beyond Risk Stratification.

Cureus
2025

Quantitative evaluation of upper gastrointestinal subepithelial lesions using endoscopic ultrasound-guided shear wave elastography.

Nagoya journal of medical science
2025

Gastrointestinal stromal tumor presenting in the pancreas.

Clinics and research in hepatology and gastroenterology
2025

Primary Localized Peritoneal Malignant Mesothelioma Involving the Stomach and Transverse Colon: A Case Report.

The Korean journal of gastroenterology = Taehan Sohwagi Hakhoe chi
2026

Comparative Analysis of a Dual DNA-RNA Panel and a DNA-Only Panel for Sarcoma: Real-World Data From a Nationwide Genomic Database.

Cancer science
2026

Cross-sectional imaging features of duodenal obstruction: A case series highlighting etiologic diversity.

Radiology case reports
2025

Delayed Relapse of Gastric Gastrointestinal Stromal Tumor After 17 Years: A Case Report and Review of Surveillance Challenges.

Cureus
2025

Gastric granular cell tumor masquerading as gastrointestinal stromal tumor: a rare concept.

Gastroenterology and hepatology from bed to bench
2025

Small bowel ulcers and bleeding: An overlooked yet critical clinical challenge.

World journal of clinical cases
2025

Laparoscopic Endoscopic Cooperative Surgery for Multiple Gastrointestinal Stromal Tumors Associated with Incomplete Carney Triad: A Case Report.

Surgical case reports
2025

Colonic Lipoma Mimicking Gastrointestinal Stromal Tumor: A Case Report of a Diagnosis Pitfall.

Cureus
2025

Gastrointestinal stromal tumor causing an eletrocardiogram mimic of cardiomyopathy.

Revista portuguesa de cardiologia : orgao oficial da Sociedade Portuguesa de Cardiologia = Portuguese journal of cardiology : an official journal of the Portuguese Society of Cardiology
2025

Is lymph node metastasis an advanced event of gastrointestinal stromal tumor?

The oncologist
2025

Case Report: Renal metastasis of a gastric gastrointestinal stromal tumor mimicking a primary renal carcinoma.

Frontiers in oncology
2025

Perforated Jejunal Gastrointestinal Stromal Tumor in a Young Male Patient With Acute Peritonitis: A Case Report and Literature Review.

Cureus
2025

A 20-Year Real-World Study of Small Bowel Cancers: Histologic Subtypes, Clinical Features, and Survival Implications.

Journal of clinical medicine
2025

Gastric diverticulum misdiagnosed as gastrointestinal stromal tumor: a case description.

Quantitative imaging in medicine and surgery
2025

Neoadjuvant Chemotherapy Followed by Laparoscopic and Endoscopic Cooperative Surgery for Locally Advanced Duodenal Gastrointestinal Stromal Tumor: A Case Report.

Surgical case reports
2025

Chilaiditi Syndrome With Colonic Gastrointestinal Stromal Tumor (GIST): An Unusual Overlap.

Cureus
2025

Durable Response to Pazopanib (Tyrosine Kinase Inhibitor) in a Patient With EWSR1::CREM Gene Fusion Positive Intra-Abdominal Unclassified Epithelioid Sarcoma.

Genes, chromosomes &amp; cancer
2025

Influence of genetic variants of imatinib on pharmacokinetics and recurrence-free survival in postoperative patients with gastrointestinal stromal tumor.

European journal of clinical pharmacology
2025

[Small bowel gastrointestinal stromal tumor in a young woman - from onset of symptoms to recovery].

Orvosi hetilap
2025

Noninvasive Diagnostic Method for Gastric Subepithelial Tumors Based on Circularity: A Multicenter Prospective Study.

Digestion
2026

Plexiform Fibromyxoma of the Gastric Body: A Rare Benign Mesenchymal Tumor in an Unconventional Location.

International journal of surgical pathology
2025

The predictive value of the systemic inflammation-nutrition index for treatment response and prognosis in patients with locally advanced gastrointestinal stromal tumors receiving neoadjuvant imatinib therapy: a dual-center retrospective cohort study.

Therapeutic advances in medical oncology
2025

Gastric endophytic gastrointestinal stromal tumor (GIST) as a rare cause of gastroduodenal intussusception: case report and literature review.

Diagnostic pathology
2025

Yttrium-90 Selective Internal Radiotherapy for Gastrointestinal Stromal Tumor With Liver Metastasis: A Case Report and Review.

Cancer innovation
2025

Incidental Discovery of a Sigmoid Colon Gastrointestinal Stromal Tumor Mimicking Colitis: A Case Report.

Cureus
2025

SMARCA4-Deficient Undifferentiated Carcinoma: A Report of 2 Cases.

The American journal of case reports
2025

"Ewing's sarcoma of the duodenum: a rare gastrointestinal presentation": case report and review of literature.

World journal of surgical oncology
2025

Myxoid epithelioid smooth muscle tumor with a novel MEF2A::NCOA2 fusion arising in the rectum: case report and literature review.

Virchows Archiv : an international journal of pathology
2025

Rectal gastrointestinal stromal tumor with early liver metastasis misdiagnosed as vaginal wall tumor: a rare case report.

Frontiers in oncology
2025

Gastrointestinal stromal tumor of the rectum: A case report with emphasis on MRI-pathologic correlation.

Radiology case reports
2025

Utility of inflammatory markers as predictors of recurrence in gastrointestinal stromal tumors: Insights from a nomogram-based approach.

World journal of gastrointestinal oncology
2025

A novel prognostic system for locally advanced gastrointestinal stromal tumors after neoadjuvant imatinib therapy based on the metro-ticket paradigm: a retrospective dual-center study.

Surgical endoscopy
2025

Short-tunnel submucosal tunneling endoscopic resection for the removal of a rectal gastrointestinal stromal tumor above the dentate line.

Endoscopy
2025

Endoscopic subserosal dissection combined with the pocket-creation method for the treatment of exophytic gastric gastrointestinal stromal tumor.

Endoscopy
2025

Gastrointestinal Stromal Tumor of the Pancreas With Hepatic Metastasis Presenting as Obstructive Jaundice.

Cureus
2025

Aptamer-based drug delivery for targeted therapy of imatinib-resistant gastrointestinal stromal tumor.

Theranostics
2025

Outcomes of submucosal tunneling endoscopic resection for subepithelial tumors in the upper gastrointestinal tract: experience from the United States.

Gastrointestinal endoscopy
2025

Imatinib-Induced Myopathy in a Patient With Gastrointestinal Stromal Tumor: A Case Report.

Cureus
2025

Rectal Leiomyosarcoma Treated With Surgery and Adjuvant Radiotherapy: A Case Report With a Brief Review of Radiotherapy Techniques From a Radiation Oncologist's Perspective.

Cureus
2025

The feasibility and safety of laparoscopic limited resection for duodenal gastrointestinal stromal tumor: a propensity score-matched analysis.

Surgical endoscopy
2025

A Case of Neurofibromatosis Type 1 With Early Gastric Cancer and Multiple Small Bowel Gastrointestinal Stromal Tumors.

The Korean journal of helicobacter and upper gastrointestinal research
2025

Letter Re: Avelumab plus axitinib in patients with advanced gastrointestinal stromal tumor.

European journal of cancer (Oxford, England : 1990)
2025

Immunotherapy and Targeted Therapies in Sarcoma: Proposed Synergy with Combination Treatment.

Journal of immunotherapy and precision oncology
2025

Solitary Fibrous Tumor of the Stomach: Diagnostic Pitfalls and Clinical Implications.

Surgical case reports
2025

Targeting yes-associated protein to overcome imatinib resistance in gastrointestinal stromal tumor drug-tolerant persister cells.

Gastric cancer : official journal of the International Gastric Cancer Association and the Japanese Gastric Cancer Association
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Referências e fontes

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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. Endoscopic submucosal dissection and submucosal tunneling endoscopic resection of gastric subepithelial lesions originating from the muscle layer: a multicenter retrospective study.
    Annals of gastroenterology· 2026· PMID 41868877mais citado
  2. Systematic Screening via WGCNA, Machine Learning, and Validation Identifies C3 and CFD as Prognostically Significant Regulators in GIST Progression.
    Molecular and cellular biology· 2026· PMID 41736511mais citado
  3. Golgi Retention and Oncogenic Signaling of KIT Tyrosine Kinase in Gastrointestinal Stromal Tumor.
    Sub-cellular biochemistry· 2026· PMID 41718985mais citado
  4. Gastric bronchogenic cyst pooled case analysis: a case report and systematic review.
    BMC gastroenterology· 2026· PMID 41688934mais citado
  5. Prediction of Mutations and Outcome in Gastrointestinal Stromal Tumors with Deep Learning: A Multicenter, Multinational Study.
    medRxiv : the preprint server for health sciences· 2026· PMID 41674582mais citado
  6. Rectal Gastrointestinal Stromal Tumor Complicated by Tumor Lysis Syndrome after Imatinib Initiation: A Rare and Fatal Case.
    Case Rep Gastroenterol· 2026· PMID 41994196recente
  7. 15-year ultra-late presentation of bifocal hepatic metastasis from gastrointestinal stromal tumor: a case report.
    Front Surg· 2026· PMID 41993795recente
  8. Analysis of Clinical Factors Impacting Imatinib Trough Concentrations in Chinese Patients With Gastrointestinal Stromal Tumors: A Real-World Study.
    Asia Pac J Clin Oncol· 2026· PMID 41992306recente
  9. Expression Patterns of Succinate Dehydrogenase B: An Immunohistochemical Study of 57 Tumors With Known SDHx Germline Pathogenic Alterations.
    Int J Surg Pathol· 2026· PMID 41985045recente
  10. Endoscopic tunneling and subserosal dissection using a natural orifice transluminal endoscopic surgery approach for a gastrointestinal stromal tumor.
    VideoGIE· 2026· PMID 41982446recente

Bases de dados e fontes oficiais

Identificadores e referências canônicas usadas para montar este verbete.

  1. ORPHA:44890(Orphanet)
  2. OMIM OMIM:606764(OMIM)
  3. MONDO:0011719(MONDO)
  4. GARD:8598(GARD (NIH))
  5. Variantes catalogadas(ClinVar)
  6. Busca completa no PubMed(PubMed)
  7. Q1495661(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

Tumor gastrointestinal do estroma
Compêndio · Raras BR

Tumor gastrointestinal do estroma

ORPHA:44890 · MONDO:0011719
Prevalência
1-5 / 10 000
Herança
Autosomal dominant, Not applicable
CID-10
C26.9 · Neoplasia maligna de localizações mal definidas dentro do aparelho digestivo
CID-11
Ensaios
31 ativos
Medicamentos
5 registrados
Início
Adolescent, Adult, Childhood
Prevalência
13.0 (Europe)
MedGen
UMLS
C0238198
Repurposing
3 candidatos
imatinibBcr-Abl kinase inhibitor|KIT inhibitor|PDGFR tyrosine kinase receptor inhibitor
regorafenibFGFR inhibitor|KIT inhibitor|PDGFR tyrosine kinase receptor inhibitor|RAF inhibitor|RET tyrosine kinase inhibitor|VEGFR inhibitor
sunitinibFLT3 inhibitor|KIT inhibitor|PDGFR tyrosine kinase receptor inhibitor|RET tyrosine kinase inhibitor|VEGFR inhibitor
EuropePMC
Wikidata
Papers 10a
DiscussaoAtiva

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