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Tumor epitelial do timo
ORPHA:3398DOENÇA RARA
Esta doença tem causa genética. Recomendamos procurar um médico geneticista para diagnóstico, exames genéticos e aconselhamento familiar.
lungInício adultaHerança AR/Unknown
Também conhecida comoTEN

Um tumor epitelial que afeta a glândula timo. Essa categoria inclui timomas e carcinomas.

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Introdução

O que você precisa saber de cara

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EM LINGUAGEM SIMPLES

O tumor epitelial do timo é uma categoria de lesões que se formam na glândula timo, abrangendo tanto timomas quanto carcinomas tímicos. A pessoa pode notar problemas respiratórios como tosse e falta de ar, além de fadiga muscular e outras manifestações autoimunes associadas. O início dos sintomas ocorre tipicamente na idade adulta. No Brasil, a condição não conta com um protocolo clínico oficial de doença rara no Ministério da Saúde, sendo acompanhada pela rede pública geral.

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Informacoes curadas por IA — podem conter imprecisoes

Um tumor epitelial que afeta a glândula timo. Essa categoria inclui timomas e carcinomas.

Pesquisas ativas
17 ensaios
25 total registrados no ClinicalTrials.gov
Publicações científicas
44 artigos
Último publicado: 2025 Oct
Medicamentos
11 com registro
PACLITAXEL, TOXXEL, PACLIMEIZ

Tem tratamento?

✓ 11 medicamentos específicos desta doença (registro ANVISA, FDA ou SUS/CEAF)
Ver detalhes, fases e interações →
PACLITAXELTOXXELPACLIMEIZONTAXABRAXANETAXILANPOKLIEVOTAXEL
Mais 9 moléculas em estudo para esta doença.

Escala de raridade

CLASSIFICAÇÃO ORPHANET · BRASIL 2024
Unknown
Ultra-rara
<1/50k
Muito rara
1/20k
Rara
1/10k
Pouco freq.
1/5k
Incomum
1/2k
Prevalência
0.0
Europe
Início
Adult
🏥
SUS: Cobertura mínimaScore: 0%
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Entender a doença

Do básico ao detalhe, leia no seu ritmo

Preparando trilha educativa...

Sinais e sintomas

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

Partes do corpo afetadas

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Sangue
5 sintomas
🫁
Pulmão
4 sintomas
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Músculos
3 sintomas
📏
Crescimento
2 sintomas
🛡️
Imunológico
2 sintomas
🧠
Neurológico
1 sintomas

+ 29 sintomas em outras categorias

Características mais comuns

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Neoplasia
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Timoma
—
Insuficiência respiratória
—
Perda de peso
—
Lúpus eritematoso sistêmico
—
Neoplasia da glândula tireoide
48sintomas
Sem dados (48)

Os sintomas variam de pessoa para pessoa. Abaixo estão as 48 características clínicas mais associadas, ordenadas por frequência.

NeoplasiaNeoplasm
TimomaThymoma
Insuficiência respiratóriaRespiratory insufficiency
Perda de pesoWeight loss
Lúpus eritematoso sistêmicoSystemic lupus erythematosus

Linha do tempo da pesquisa

Publicações por ano — veja quando o interesse científico cresceu
Anos de pesquisa1desde 2025
Total histórico44PubMed
Últimos 10 anos24publicações
Pico20224 papers
Linha do tempo
2025Hoje · 2026🧪 2008Primeiro ensaio clínico📈 2022Ano de pico
Publicações por ano (últimos 10 anos)

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Genética e causas

O que está alterado no DNA e como passa nas famílias

🧬

Nenhum gene associado encontrado

Os dados genéticos desta condição ainda estão sendo catalogados.

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.
3Fase 31
2Fase 222
·Pré-clínico11
Medicamentos catalogadosEnsaios clínicos· 9 medicamentos · 25 ensaios
Carregando informações de tratamento...

Onde tratar no SUS

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

🇧🇷 Atendimento SUS — Tumor epitelial do timo

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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

Onde estão os ensaios

Com ensaio aberto em 5 países. O ponto verde marca onde há vaga agora.

🟢 Recrutando agora

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

Outros ensaios clínicos

25 ensaios clínicos encontrados, 17 ativos.

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

Timeline de publicações
24 papers (10 anos)
#1

Paraneoplastic minimal change disease in malignant thymoma: a rare association progressing to end-stage renal disease.

Oxford medical case reports2025 Oct

Thymoma, commonly seen in middle-aged and elderly individuals, is associated with autoimmune disorders. While renal involvement is rare, its occurrence remains poorly characterized. We report a 24-year-old female with a ten-year history of malignant thymoma who developed nephrotic syndrome. Renal biopsy revealed minimal change disease (MCD), a rare paraneoplastic manifestation of thymoma. Despite treatment with steroids and rituximab, her renal function deteriorated, leading to end-stage renal disease (ESRD). This case underscores the challenges of managing paraneoplastic glomerular disease and highlights the need for better understanding of such rare associations. Thymoma-related glomerular diseases, often linked to immune dysregulation, pose significant treatment challenges. Recognizing these associations may guide more effective management strategies, particularly for patients unresponsive to steroids.

#2

Histologic features, growth patterns and classification of atypical thymomas.

Mediastinum (Hong Kong, China)2025

Atypical thymomas are a rare form of primary thymic epithelial neoplasm that are characterized by conservation of most of the organotypical features of thymic differentiation, but show atypical cytological features. The spectrum of histologic features and growth patterns these tumors can exhibit have not been extensively documented or illustrated in the literature. The basic histologic growth patterns seen in atypical thymomas include the epithelioid or "Squamoid" subtype and the spindle cell subtype. The histologic picture may often be confused with thymic carcinomas or metastatic disease, in particular squamous cell carcinoma due to the overlap in histologic features. In addition, these subtypes may be seen in combination with each other or in combination with conventional types of thymoma including type A, type AB, type B1 and type B2. Cases of thymic carcinoma arising from atypical thymoma have also been documented in the literature. The biologic behavior of atypical thymomas is intermediate between conventional thymoma and thymic carcinoma. The tumors tend to present with aggressive behavior and an increased rate of metastasis and thus achieving the correct diagnosis is of utmost importance. We present a review of the various morphologic appearances of these tumors to emphasize the wide spectrum of histologic features that they can display, with a discussion of the current nomenclature and approach to these neoplasms.

#3

Histomorphological Spectrum and Diagnostic Challenges in Thymic Epithelial Neoplasms with their Prognostic Significance: A Case Series of 33 Cases at a Regional Cancer Center in Western India.

South Asian journal of cancer2024 Jul

Jahnavi Gandhi, DCPObjective  Pathological diagnosis of thymic epithelial neoplasms is challenging due to multiple subtypes, tumor heterogeneity, and variations in inter-observer reproducibility. Very few studies are available on their spectrum in the Indian subcontinent. In this study, we aimed to explore the morphological spectrum and diagnostic difficulties in the classification and subtyping of thymic epithelial neoplasms along with their prognostic significance in the Indian population. Material and Methods  Retrospectively, all surgically resected thymectomy specimens operated at our institute as well as outside review cases during the period were included. Histomorphology and immunohistochemistry (IHC) slides were reviewed and correlated with clinicopathological variables. Statistical Analysis  Microsoft Excel 2019 and SPSS version 20 were used for data analysis. Results  Among the 33 thymic epithelial neoplasms operated during the study period, the commonest subtype was thymoma B2 type followed by AB, B1, A, and B3 types. A single case each of micronodular thymoma, microscopic thymoma, and thymic carcinoma were identified. Six cases of thymomas with more than one pattern (other than 'A') were noted. The male:female ratio was 2:1. Stage I in Modified Masaoka staging and pT1a in TNM staging were most common. Seven cases had metastasis, four during initial presentation and three during subsequent follow-up. Discussion and Conclusion  Thymic epithelial neoplasms show morphological overlapping of features. Thorough sampling, morphology, and IHC for exact subtyping of thymoma and diligent search and documenting of lymphovascular invasion (LVI) are vital as both are separate risk factors for metastasis/recurrence and help the clinician in a better follow-up of patients.

#4

Artificial intelligence-powered spatial analysis of tumor-infiltrating lymphocytes as a biomarker in locally advanced unresectable thymic epithelial neoplasm: A single-center, retrospective, longitudinal cohort study.

Thoracic cancer2023 Oct

Thymic epithelial tumors (TET) are rare malignancies and lack well-defined biomarkers for neoadjuvant therapy. This study aimed to evaluate the clinical utility of artificial intelligence (AI)-powered tumor-infiltrating lymphocyte (TIL) analysis in TET. Patients initially diagnosed with unresectable thymoma or thymic carcinoma who underwent neoadjuvant therapy between January 2004 and December 2021 formed our study population. Hematoxylin and eosin-stained sections from the initial biopsy and surgery were analyzed using an AI-powered spatial TIL analyzer. Intratumoral TIL (iTIL) and stromal TIL (sTIL) were quantified and their immune phenotype (IP) was identified. Thirty-five patients were included in this study. The proportion of patients with partial response to neoadjuvant therapy was higher in the group with nondesert IP in preneoadjuvant biopsy (63.6% vs. 17.6%, p = 0.038). A significant increase in both iTIL (median 22.18/mm2 vs. 340.69/mm2 , p < 0.001) and sTIL (median 175.19/mm2 vs. 531.02/mm2 , p = 0.004) was observed after neoadjuvant therapy. Patients with higher iTIL (>147/mm2 ) exhibited longer disease-free survival (median, 29 months vs. 12 months, p = 0.009) and overall survival (OS) (median, 62 months vs. 45 months, p = 0.002). Patients with higher sTIL (>232.1/mm2 ) exhibited longer OS (median 62 months vs. 30 months, p = 0.021). Nondesert IP in initial biopsy was associated with a better response to neoadjuvant therapy. Increased infiltration of both iTIL and sTIL in surgical specimens were associated with longer OS in patients with TET who underwent resection followed by neoadjuvant therapy.

#5

Asymptomatic lipofibroadenoma in a 17-year-old male: a case report and literature review of a rare entity.

Mediastinum (Hong Kong, China)2023

The most common thymic tumours, thymomas, are derived from thymic epithelium and are generally low-grade neoplasm. Frankly malignant tumours, thymic carcinomas are rarer still. Exceedingly rare thymic tumours contain a mesenchymal tissue component such as fibrous connective tissue and/or mature fat. Lipofibroadenoma (LFA) is a very rare mixed epithelial-mesenchymal thymic tumour, included in the category of thymic epithelial tumors. LFA in addition to a mature adipocytic component, contains variable epithelial and mesenchymal tissue components. Owing to the presence of an epithelial component in LFA, this entity, in contrast to thymolipoma, is included in the World Health Organization (WHO) category of thymic epithelial neoplasm. Currently only 12 LFA cases have been described. The 12 previously reported cases all behaved in a benign fashion, although four cases were associated with a conventional type of thymoma. We here present a new, 13th, case of LFA. The LFA was discovered incidentally in a previously healthy 17-year-old male after investigations for suspected pneumonia. On imaging a mass was discovered in the anterior mediastinum which was subsequently surgically removed. The resected tumour was extensively investigated, including the first instance of full molecular analysis of this rare entity and all available literature on LFA was sourced to provide a comprehensive overview. The histology of this LFA was similar to previously described cases. No gene mutations or rearrangements were identified. The patient made an uneventful recovery and after 13 months of follow-up remained well. An additional, 13th case of LFA is presented. Based on the available literature it appears that LFA may be considered a benign composite thymic tumour, although the combination of an additional conventional thymoma component may warrant closer follow-up.

Publicações recentes

Ver todas no PubMed

📚 EuropePMC6 artigos no totalmostrando 24

2025

Paraneoplastic minimal change disease in malignant thymoma: a rare association progressing to end-stage renal disease.

Oxford medical case reports
2025

Histologic features, growth patterns and classification of atypical thymomas.

Mediastinum (Hong Kong, China)
2023

Artificial intelligence-powered spatial analysis of tumor-infiltrating lymphocytes as a biomarker in locally advanced unresectable thymic epithelial neoplasm: A single-center, retrospective, longitudinal cohort study.

Thoracic cancer
2023

Asymptomatic lipofibroadenoma in a 17-year-old male: a case report and literature review of a rare entity.

Mediastinum (Hong Kong, China)
2023

Prostate Adenocarcinoma Within a Thymoma: A Rare Case of Tumor-to-Tumor Metastasis.

Cureus
2024

Histomorphological Spectrum and Diagnostic Challenges in Thymic Epithelial Neoplasms with their Prognostic Significance: A Case Series of 33 Cases at a Regional Cancer Center in Western India.

South Asian journal of cancer
2023

B3 thymoma mimicking poorly differentiated thyroid carcinoma: Diagnostic pitfalls of anterior mediastinal mass fine needle aspiration.

Diagnostic cytopathology
2022

Diagnostic Challenges in the Cytology of Thymic Epithelial Neoplasms.

Cancers
2022

Thymic Epithelial Neoplasms: Focusing on the Epigenetic Alterations.

International journal of molecular sciences
2022

Atypical thymomas with squamoid and spindle cell features: clinicopathologic, immunohistochemical and molecular genetic study of 120 cases with long-term follow-up.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2022

Concurrent myasthenia gravis-related cervical thymoma in a patient with diffuse large B-cell lymphoma: a case report.

Journal of medical case reports
2021

Novel Tumor-Specific Antigens for Immunotherapy Identified From Multi-omics Profiling in Thymic Carcinomas.

Frontiers in immunology
2021

Difference in the distribution of tumor-infiltrating CD8+ T cells and FOXP3+ T cells between micronodular thymoma with lymphoid stroma and micronodular thymic carcinoma with lymphoid stroma.

Pathology international
2021

Imaging of the Anterior/Prevascular Mediastinum.

Radiologic clinics of North America
2019

The role of positron emission tomography/computed tomography in the evaluation of anterior mediastinal masses and differentiating between the histological subtypes of thymic epithelial neoplasms.

Turk gogus kalp damar cerrahisi dergisi
2020

Metaplastic thymoma: a distinctive thymic neoplasm characterized by YAP1-MAML2 gene fusions.

Modern pathology : an official journal of the United States and Canadian Academy of Pathology, Inc
2019

Selected Case From the Arkadi M. Rywlin International Pathology Slide Seminar: Atypical Thymoma With Rhabdomyomatous Differentiation.

Advances in anatomic pathology
2018

Poorly Differentiated Nonkeratinizing Squamous Cell Carcinoma of the Thymus: Clinicopathologic and Molecular Genetic Study of 25 Cases.

The American journal of surgical pathology
2017

Diagnostic Value of Dual-time-point F-18 FDG PET/CT and Chest CT for the Prediction of Thymic Epithelial Neoplasms.

Acta medica Okayama
2016

Ectopic micronodular thymoma with lymphoid stroma in the cervical region: a rare case associated with Langerhans cells proliferation.

OncoTargets and therapy
2016

Chemotherapy with gemcitabine plus cisplatin in patients with advanced thymic squamous cell carcinoma: Evaluation of efficacy and toxicity.

Thoracic cancer
2015

State of the Art: MR Imaging of Thymoma.

Magnetic resonance imaging clinics of North America
2015

Reproducibility of 3 histologic classifications and 3 staging systems for thymic epithelial neoplasms and its effect on prognosis.

The American journal of surgical pathology
2015

Modified Masaoka stage and size are independent prognostic predictors in thymoma and modified Masaoka stage is superior to histopathologic classifications.

Journal of thoracic oncology : official publication of the International Association for the Study of Lung Cancer

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Doença com base genética

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Conselhos e sociedades

Doenças relacionadas

Doenças com sintomas parecidos — ajudam quem ainda está buscando diagnóstico

Perguntas frequentes

O que as famílias mais perguntam sobre esta doença — cada resposta com a fonte de onde saiu

Respostas geradas por IA a partir das fontes citadas

Trata-se de uma neoplasia de origem epitelial localizada na glândula timo. Essa categoria abrange os timomas e os carcinomas tímicos.

Referências

Fontes citadas no texto, publicações do grafo e bases de dados usadas neste verbete

Citar este verbete

Raras. (s.d.). Tumor epitelial do timo. Em Raras — Enciclopédia de Doenças Raras do Brasil. https://raras.org/doenca/tumor-epitelial-do-timo

Formato APA. Conteúdo sob CC BY 4.0 — reuso livre com atribuição.

Conteúdo mantido por Agente Raras · Médicos e pesquisadores podem colaborar

Compêndio · Raras BR

Tumor epitelial do timo

ORPHA:3398 · MONDO:0018079
Prevalência
Unknown
Herança
Autosomal recessive, Not applicable
Ensaios
17 ativos
Medicamentos
9 registrados
Início
Adult
Prevalência
0.0 (Europe)
MedGen
UMLS
C1266101
EuropePMC
Wikidata
Papers 10a

📋 Origem dos dados

Esta página agrega dados de fontes públicas e oficiais. Dados sobre cobertura no SUS (PCDT, CEAF) são verificados ativamente por agente proativo (ver badge no infobox). Demais dados têm atribuição de fonte + data da última sincronização — clique para abrir o original.

Doença rara (ontologia)
fonte: Orphanet
Identificador unificado
fonte: MONDO
Indexação biomédica
fonte: MeSH (NLM)
Dado público estruturado
fonte: Wikidata
Moléculas estudadas na doença
fonte: OpenTargets
Rara