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Mesothelioma, malignant(MESOM)

MedGen UID:
91062
Concept ID:
C0345967
Neoplastic Process
Synonyms: Malignant mesothelioma (disease); MESOM
SNOMED CT: Mesothelioma, malignant (62064005); Malignant mesothelioma (62064005); Mesothelioma (malignant, clinical disorder) (109378008); Malignant mesothelioma (109378008); Cancer, mesothelioma (109378008)
Modes of inheritance:
Not genetically inherited
MedGen UID:
988794
Concept ID:
CN307044
Finding
Source: Orphanet
clinical entity without genetic inheritance.
 
Genes (locations): BCL10 (1p22.3); WT1 (11p13)
 
HPO: HP:0100001
Monarch Initiative: MONDO:0006292
OMIM®: 156240
Orphanet: ORPHA50251

Definition

Malignant mesothelioma is an aggressive neoplasm of the serosal lining of the chest etiologically linked to asbestos. It is diagnosed in approximately 2,000 to 3,000 individuals annually in the United States, most of whom die within 2 years of diagnosis (summary by Bott et al., 2011). See also 614327 for a tumor predisposition syndrome that may contribute to the development of malignant mesothelioma upon asbestos exposure and is caused by germline mutation in the BAP1 gene (603089) on chromosome 3p21. [from OMIM]

Clinical features

From HPO
Mesothelioma, malignant
MedGen UID:
91062
Concept ID:
C0345967
Neoplastic Process
Malignant mesothelioma is an aggressive neoplasm of the serosal lining of the chest etiologically linked to asbestos. It is diagnosed in approximately 2,000 to 3,000 individuals annually in the United States, most of whom die within 2 years of diagnosis (summary by Bott et al., 2011). See also 614327 for a tumor predisposition syndrome that may contribute to the development of malignant mesothelioma upon asbestos exposure and is caused by germline mutation in the BAP1 gene (603089) on chromosome 3p21.

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVMesothelioma, malignant

Conditions with this feature

Mesothelioma, malignant
MedGen UID:
91062
Concept ID:
C0345967
Neoplastic Process
Malignant mesothelioma is an aggressive neoplasm of the serosal lining of the chest etiologically linked to asbestos. It is diagnosed in approximately 2,000 to 3,000 individuals annually in the United States, most of whom die within 2 years of diagnosis (summary by Bott et al., 2011). See also 614327 for a tumor predisposition syndrome that may contribute to the development of malignant mesothelioma upon asbestos exposure and is caused by germline mutation in the BAP1 gene (603089) on chromosome 3p21.
BAP1-related tumor predisposition syndrome
MedGen UID:
482122
Concept ID:
C3280492
Disease or Syndrome
BAP1 tumor predisposition syndrome (BAP1-TPDS) is associated with an increased risk for a specific skin lesion, BAP1-inactivated melanocytic tumors (BIMT; formerly called atypical Spitz tumors), and the following cancers, in descending order of frequency: uveal (eye) melanoma (UM), malignant mesothelioma (MMe), cutaneous melanoma (CM), renal cell carcinoma (RCC), and basal cell carcinoma (BCC). Hepatocellular carcinoma, cholangiocarcinoma, and meningioma may also be associated with BAP1-TPDS. Affected individuals can have more than one type of primary cancer. In general, the median age of onset of these tumors is younger than in the general population. UM tends to be a more aggressive class 2 tumor with higher risk for metastasis and reduced survival compared to UM occurring in the general population. Due to the limited number of families reported to date, the penetrance, natural history, and frequencies of BAP1-associated tumors are yet to be determined. Other suspected but unconfirmed tumors in BAP1-TPDS include (in alphabetic order): breast cancer, neuroendocrine carcinoma, non-small-cell lung adenocarcinoma, thyroid cancer, and urinary bladder cancer.

Professional guidelines

PubMed

Skok K, Hladnik G, Grm A, Crnjac A
Medicina (Kaunas) 2019 Aug 15;55(8) doi: 10.3390/medicina55080490. PMID: 31443309Free PMC Article
Husain AN, Colby TV, Ordóñez NG, Allen TC, Attanoos RL, Beasley MB, Butnor KJ, Chirieac LR, Churg AM, Dacic S, Galateau-Sallé F, Gibbs A, Gown AM, Krausz T, Litzky LA, Marchevsky A, Nicholson AG, Roggli VL, Sharma AK, Travis WD, Walts AE, Wick MR
Arch Pathol Lab Med 2018 Jan;142(1):89-108. Epub 2017 Jul 7 doi: 10.5858/arpa.2017-0124-RA. PMID: 28686500
Ruffié P, Lehmann M, Galateau-Sallé F, Lagrange JL, Pairon JC; French National Federation of Cancer (FNCLCC)
Br J Cancer 2001 May;84 Suppl 2(Suppl 2):49-50. doi: 10.1054/bjoc.2001.1763. PMID: 11355969Free PMC Article

Recent clinical studies

Etiology

Farinea G, Crespi V, Listì A, Righi L, Bironzo P, Merlini A, Malapelle U, Novello S, Scagliotti GV, Passiglia F
J Thorac Oncol 2023 Sep;18(9):1146-1164. Epub 2023 Jun 16 doi: 10.1016/j.jtho.2023.05.028. PMID: 37331604
Caceres JD, Venkata AN
Curr Opin Pulm Med 2023 Mar 1;29(2):76-82. Epub 2023 Jan 11 doi: 10.1097/MCP.0000000000000939. PMID: 36630203
Chun CP, Song LX, Zhang HP, Guo DD, Xu GX, Li Y, Xin X, Cao J, Li F
Am J Med Sci 2023 Jan;365(1):99-103. Epub 2022 Aug 6 doi: 10.1016/j.amjms.2022.07.008. PMID: 35940275
Dacic S
Mod Pathol 2022 Jan;35(Suppl 1):51-56. Epub 2021 Aug 31 doi: 10.1038/s41379-021-00895-7. PMID: 34465883
Ledda C, Caltabiano R, Vella F, Matera S, Marconi A, Loreto C, Rapisarda V
Biomark Med 2019 Jul;13(10):875-886. Epub 2019 Jun 25 doi: 10.2217/bmm-2018-0285. PMID: 31237454

Diagnosis

Ando K, Morohoshi T, Tsuura Y, Masuda M
Interact Cardiovasc Thorac Surg 2022 Oct 10;35(5) doi: 10.1093/icvts/ivac255. PMID: 36205712Free PMC Article
Dacic S
Mod Pathol 2022 Jan;35(Suppl 1):51-56. Epub 2021 Aug 31 doi: 10.1038/s41379-021-00895-7. PMID: 34465883
Janes SM, Alrifai D, Fennell DA
N Engl J Med 2021 Sep 23;385(13):1207-1218. doi: 10.1056/NEJMra1912719. PMID: 34551230
Mann S, Khawar S, Moran C, Kalhor N
Ann Diagn Pathol 2019 Apr;39:74-77. Epub 2019 Feb 19 doi: 10.1016/j.anndiagpath.2019.02.014. PMID: 30798074
Li CY, Alexander HR Jr
Surg Oncol Clin N Am 2018 Jul;27(3):539-549. doi: 10.1016/j.soc.2018.02.010. PMID: 29935688

Therapy

John A, O'Sullivan H, Popat S
Curr Treat Options Oncol 2023 Dec;24(12):1758-1789. Epub 2023 Nov 17 doi: 10.1007/s11864-023-01148-2. PMID: 37975977
Fennell DA, Dulloo S, Harber J
Nat Rev Clin Oncol 2022 Sep;19(9):573-584. Epub 2022 Jul 1 doi: 10.1038/s41571-022-00649-7. PMID: 35778611
Janes SM, Alrifai D, Fennell DA
N Engl J Med 2021 Sep 23;385(13):1207-1218. doi: 10.1056/NEJMra1912719. PMID: 34551230
Wadowski B, De Rienzo A, Bueno R
Thorac Surg Clin 2020 Nov;30(4):383-393. Epub 2020 Sep 12 doi: 10.1016/j.thorsurg.2020.08.005. PMID: 33012428Free PMC Article
Opitz I, Weder W
Semin Thorac Cardiovasc Surg 2015 Summer;27(2):240-9. Epub 2015 Jun 17 doi: 10.1053/j.semtcvs.2015.05.003. PMID: 26686455

Prognosis

Iczkowski KA
Adv Anat Pathol 2023 Jul 1;30(4):259-261. Epub 2022 Sep 21 doi: 10.1097/PAP.0000000000000371. PMID: 36129341
Lee GM, Stowell JT, Pope K, Carter BW, Walker CM
AJR Am J Roentgenol 2021 Mar;216(3):649-658. Epub 2020 Dec 30 doi: 10.2214/AJR.20.23523. PMID: 33377793
Euhus CJ, Ripley RT
Thorac Surg Clin 2020 Nov;30(4):425-433. Epub 2020 Sep 12 doi: 10.1016/j.thorsurg.2020.07.001. PMID: 33012430
Ledda C, Caltabiano R, Vella F, Matera S, Marconi A, Loreto C, Rapisarda V
Biomark Med 2019 Jul;13(10):875-886. Epub 2019 Jun 25 doi: 10.2217/bmm-2018-0285. PMID: 31237454
Mann S, Khawar S, Moran C, Kalhor N
Ann Diagn Pathol 2019 Apr;39:74-77. Epub 2019 Feb 19 doi: 10.1016/j.anndiagpath.2019.02.014. PMID: 30798074

Clinical prediction guides

Papavassiliou KA, Sofianidi AA, Papavassiliou AG
J Cell Mol Med 2024 Apr;28(8):e18330. doi: 10.1111/jcmm.18330. PMID: 38606782Free PMC Article
Chun CP, Song LX, Zhang HP, Guo DD, Xu GX, Li Y, Xin X, Cao J, Li F
Am J Med Sci 2023 Jan;365(1):99-103. Epub 2022 Aug 6 doi: 10.1016/j.amjms.2022.07.008. PMID: 35940275
Han YQ, Xu SC, Zheng WQ, Hu ZD
Thorac Cancer 2021 Jan;12(1):8-12. Epub 2020 Nov 22 doi: 10.1111/1759-7714.13746. PMID: 33225621Free PMC Article
Oehl K, Vrugt B, Opitz I, Meerang M
Int J Mol Sci 2018 May 30;19(6) doi: 10.3390/ijms19061603. PMID: 29848954Free PMC Article
Anevlavis S, Froudarakis ME
Clin Respir J 2018 Mar;12(3):839-847. Epub 2017 Jan 11 doi: 10.1111/crj.12597. PMID: 27997741

Recent systematic reviews

Zhuang W, Liu L, Sun B, Bai H, Wang Z, Duan J, Wan R, Ma Z, Zhong J, Wang J
Crit Rev Oncol Hematol 2024 Jun;198:104372. Epub 2024 Apr 26 doi: 10.1016/j.critrevonc.2024.104372. PMID: 38677356
Martinez-Zayas G, Molina S, Ost DE
Eur Respir Rev 2022 Dec 31;31(166) Epub 2022 Dec 21 doi: 10.1183/16000617.0053-2022. PMID: 36543349Free PMC Article
Scherpereel A, Opitz I, Berghmans T, Psallidas I, Glatzer M, Rigau D, Astoul P, Bölükbas S, Boyd J, Coolen J, De Bondt C, De Ruysscher D, Durieux V, Faivre-Finn C, Fennell D, Galateau-Salle F, Greillier L, Hoda MA, Klepetko W, Lacourt A, McElnay P, Maskell NA, Mutti L, Pairon JC, Van Schil P, van Meerbeeck JP, Waller D, Weder W, Cardillo G, Putora PM
Eur Respir J 2020 Jun;55(6) Epub 2020 Jun 11 doi: 10.1183/13993003.00953-2019. PMID: 32451346
Faccioli E, Bellini A, Mammana M, Monaci N, Schiavon M, Rea F
Expert Rev Respir Med 2020 Jan;14(1):67-79. Epub 2019 Nov 13 doi: 10.1080/17476348.2020.1688147. PMID: 31674841
Cao C, Tian D, Park J, Allan J, Pataky KA, Yan TD
Lung Cancer 2014 Feb;83(2):240-5. Epub 2013 Dec 6 doi: 10.1016/j.lungcan.2013.11.026. PMID: 24360321

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