# Mesotelioma

> Página oficial: https://raras.org/doenca/mesotelioma
> Fonte: Raras.org — Banco de Dados de Doenças Raras em Português (CC BY-NC-SA 4.0)
> Última atualização: 2026-05-07

## Identificadores

- **ORPHA**: 50251 — https://www.orpha.net/en/disease/detail/50251
- **CID-10**: C45.0
- **OMIM**: OMIM:156240 — https://omim.org/entry/156240

## Descrição clínica

É um tipo de câncer que se desenvolve nas células de revestimento da pleura (a membrana que envolve os pulmões) ou do peritônio (a membrana que reveste o abdômen e seus órgãos). Ele está associado à exposição ao amianto.

## Epidemiologia e herança

- **Prevalência**: 1-9 / 100 000
- **Padrão de herança**: Not applicable

## Sinais e sintomas (28 fenótipos HPO)

- **Anormalidade da fisiologia do sistema cardiovascular** — HPO: HP:0011025 (Ocasional (29-5%))
- **Hepatomegalia** — HPO: HP:0002240 (Ocasional (29-5%))
- **Tosse** — HPO: HP:0012735 (Frequente (79-30%))
- **Sintoma constitucional** — HPO: HP:0025142 (Frequente (79-30%))
- **Morfologia anormal da pleura** — HPO: HP:0002103 (Frequente (79-30%))
- **Paralisia do quarto nervo craniano** — HPO: HP:0007011 (Ocasional (29-5%))
- **Morfologia anormal do tórax** — HPO: HP:0000765 (Frequente (79-30%))
- **Obstrução da veia cava superior** — HPO: HP:0031041 (Ocasional (29-5%))
- **Dispneia** — HPO: HP:0002094 (Frequente (79-30%))
- **Desconforto respiratório** — HPO: HP:0002098 (Frequente (79-30%))
- **Disfagia** — HPO: HP:0002015 (Ocasional (29-5%))
- **Fisiologia anormal do sistema respiratório** — HPO: HP:0002795 (Ocasional (29-5%))
- **Linfadenopatia** — HPO: HP:0002716 (Ocasional (29-5%))
- **Morfologia pulmonar anormal** — HPO: HP:0002088 (Ocasional (29-5%))
- **Dor torácica** — HPO: HP:0100749 (Frequente (79-30%))
- **Derrame pleural** — HPO: HP:0002202 (Muito frequente (99-80%))
- **Perda de peso** — HPO: HP:0001824 (Frequente (79-30%))
- **Mesotelioma maligno** — HPO: HP:0100001
- **Sibilos** — HPO: HP:0030828
- **Anormalidade da coagulação** — HPO: HP:0001928
- **Dor abdominal** — HPO: HP:0002027
- **Peritonite** — HPO: HP:0002586
- **Ascite** — HPO: HP:0001541
- **Íleo** — HPO: HP:0002595
- **Neoplasia** — HPO: HP:0002664
- **Distensão abdominal** — HPO: HP:0003270
- **Edema pedal** — HPO: HP:0010741
- **HP:0001442** — HPO: HP:0001442

## Genes associados (3)

- **BAP1** — Membrane-associated guanylate kinase, WW and PDZ domain-containing protein 1 [Disease-causing germline mutation(s) in]
  - Função: Plays a role in coupling actin fibers to cell junctions in endothelial cells, via its interaction with AMOTL2 and CDH5 (By similarity). May regulate acid-induced ASIC3 currents by modulating its expre
- **WT1** — Wilms tumor protein [Disease-causing germline mutation(s) in]
  - Função: Transcription factor that plays an important role in cellular development and cell survival (PubMed:7862533). Recognizes and binds to the DNA sequence 5'-GCG(T/G)GGGCG-3' (PubMed:17716689, PubMed:2525
- **BCL10** — B-cell lymphoma/leukemia 10 [Candidate gene tested in]
  - Função: Plays a key role in both adaptive and innate immune signaling by bridging CARD domain-containing proteins to immune activation (PubMed:10187770, PubMed:10364242, PubMed:10400625, PubMed:24074955, PubM

## Ensaios clínicos ativos (32)

- **NCT00242723** [RECRUITING]: Evaluation of Cell Changes in Blood and Tissue in Cancers of the Lung, Esophagus and Lung Lining — https://clinicaltrials.gov/study/NCT00242723
- **NCT07532902** [RECRUITING]: A Study of BMS-986504 With Standard-of-Care Therapy for People With Solid Tumor Cancer — https://clinicaltrials.gov/study/NCT07532902
- **NCT06362369** [RECRUITING]: A Study of Oral 7HP349 (Alintegimod) in Combination With Ipilimumab Followed by Nivolumab Monotherapy — https://clinicaltrials.gov/study/NCT06362369
- **NCT07510815** [RECRUITING]: Dual-Target MSLN/FAP CAR-NK Cells for Pleural and Peritoneal Mesothelioma — https://clinicaltrials.gov/study/NCT07510815
- **NCT07277413** [RECRUITING]: A Study of IDE892 as Monotherapy and Combination in MTAP-deleted Advanced Solid Tumors — https://clinicaltrials.gov/study/NCT07277413
- **NCT06097728** [RECRUITING]: MEDI5752 in Combination With Carboplatin Plus Pemetrexed in Unresectable Pleural Mesothelioma — https://clinicaltrials.gov/study/NCT06097728
- **NCT05655078** [RECRUITING]: Hemithoracic Irradiation With Proton Therapy in Malignant Pleural Mesothelioma — https://clinicaltrials.gov/study/NCT05655078
- **NCT06535607** [RECRUITING]: Study of Volrustomig as Monotherapy or in Combination With Anti- Cancer Agents in Participants With Advanced/Metastatic Solid Tumors — https://clinicaltrials.gov/study/NCT06535607
- **NCT06840834** [RECRUITING]: Evaluating Ivonescimab as a Potential Treatment for Pleural Mesothelioma Patients Whose Cancer Has Returned After Previous Immunotherapy and Chemotherapy — https://clinicaltrials.gov/study/NCT06840834
- **NCT07126509** [RECRUITING]: Partial Pleurectomy (Surgery) for Unresectable Pleural Mesothelioma — https://clinicaltrials.gov/study/NCT07126509

## Doenças relacionadas (por similaridade fenotípica)

- [Síndrome hipereosinofílico idiopático](https://raras.org/doenca/sindrome-hipereosinofilico-idiopatico) — ORPHA:3260 — 11 sintomas em comum
- [Tumor raro induzido por vírus](https://raras.org/doenca/tumor-raro-induzido-por-virus) — ORPHA:289635 — 11 sintomas em comum
- [Doença Castleman](https://raras.org/doenca/doenca-castleman) — ORPHA:160 — 11 sintomas em comum
- [Complicação após transplante de órgão](https://raras.org/doenca/complicacao-apos-transplante-de-orgao) — ORPHA:306644 — 10 sintomas em comum
- [Tumor relacionado com vírus Epstein-Barr](https://raras.org/doenca/tumor-relacionado-com-virus-epstein-barr) — ORPHA:289638 — 10 sintomas em comum
- [Síndromes hipereosinofílicos](https://raras.org/doenca/sindromes-hipereosinofilicos) — ORPHA:168956 — 10 sintomas em comum
- [Lúpus eritematoso sistêmico](https://raras.org/doenca/lupus-eritematoso-sistemico) — ORPHA:536 — 10 sintomas em comum
- [Hipertensão arterial pulmonar](https://raras.org/doenca/hipertensao-arterial-pulmonar) — ORPHA:182090 — 10 sintomas em comum
- [Hipertensão arterial pulmonar idiopática/hereditária](https://raras.org/doenca/hipertensao-arterial-pulmonar-idiopaticahereditaria) — ORPHA:422 — 10 sintomas em comum
- [Doença linfoproliferativa associada a vírus Epstein-Barr](https://raras.org/doenca/doenca-linfoproliferativa-associada-a-virus-epstein-barr) — ORPHA:289644 — 10 sintomas em comum

## Importante

O Raras **não diagnostica e não prescreve**. Esta página é educativa e informativa.
Pacientes devem consultar profissionais de saúde qualificados para decisões clínicas.

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**Citação sugerida**: Raras.org — Mesotelioma. Disponível em: https://raras.org/doenca/mesotelioma
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