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Lung PEComa

MedGen UID:
1639012
•Concept ID:
C4684821
•
Neoplastic Process
Synonyms: lung PEComa; Lung PEComatous Tumor; lung pecomatous tumor; lung pecomatous tumour
 
Monarch Initiative: MONDO:0020588

Definition

A lung tumor that arises from perivascular epithelioid cells (PECs). [from NCI]

Professional guidelines

PubMed

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Recent advances in the pathobiology and clinical management of lymphangioleiomyomatosis.
Harari S, Spagnolo P, Cocconcelli E, Luisi F, Cottin V
Curr Opin Pulm Med 2018 Sep;24(5):469-476. doi: 10.1097/MCP.0000000000000502. PMID: 29927757
Diagnosis and treatment of cystic lung disease.
Park S, Lee EJ
Korean J Intern Med 2017 Mar;32(2):229-238. Epub 2017 Feb 28 doi: 10.3904/kjim.2016.242. PMID: 28264540Free PMC Article

Recent clinical studies

Etiology

Cystic lung disease.
Cabeza Martínez B, Giménez Palleiro A, Mazzini Florindez SP
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:265-276. doi: 10.1016/j.rxeng.2022.09.005. PMID: 36737165
Ultra-rare cystic disease.
Elia D, Torre O, Cassandro R, Caminati A, Harari S
Eur Respir Rev 2020 Sep 30;29(157) Epub 2020 Sep 2 doi: 10.1183/16000617.0163-2019. PMID: 32878971Free PMC Article
Diffuse Cystic Lung Diseases.
Obaidat B, Yazdani D, Wikenheiser-Brokamp KA, Gupta N
Respir Care 2020 Jan;65(1):111-126. Epub 2019 Oct 15 doi: 10.4187/respcare.07117. PMID: 31615921
The 2015 World Health Organization Classification of Lung Tumors: Impact of Genetic, Clinical and Radiologic Advances Since the 2004 Classification.
Travis WD, Brambilla E, Nicholson AG, Yatabe Y, Austin JHM, Beasley MB, Chirieac LR, Dacic S, Duhig E, Flieder DB, Geisinger K, Hirsch FR, Ishikawa Y, Kerr KM, Noguchi M, Pelosi G, Powell CA, Tsao MS, Wistuba I; WHO Panel
J Thorac Oncol 2015 Sep;10(9):1243-1260. doi: 10.1097/JTO.0000000000000630. PMID: 26291008
Diffuse Cystic Lung Disease. Part I.
Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX
Am J Respir Crit Care Med 2015 Jun 15;191(12):1354-66. doi: 10.1164/rccm.201411-2094CI. PMID: 25906089Free PMC Article

Diagnosis

Cystic lung disease.
Cabeza Martínez B, Giménez Palleiro A, Mazzini Florindez SP
Radiologia (Engl Ed) 2022 Dec;64 Suppl 3:265-276. doi: 10.1016/j.rxeng.2022.09.005. PMID: 36737165
Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Single-Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell.
Guo M, Yu JJ, Perl AK, Wikenheiser-Brokamp KA, Riccetti M, Zhang EY, Sudha P, Adam M, Potter A, Kopras EJ, Giannikou K, Potter SS, Sherman S, Hammes SR, Kwiatkowski DJ, Whitsett JA, McCormack FX, Xu Y
Am J Respir Crit Care Med 2020 Nov 15;202(10):1373-1387. doi: 10.1164/rccm.201912-2445OC. PMID: 32603599Free PMC Article
Lymphangioleiomyomatosis.
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
A Stepwise Diagnostic Approach to Cystic Lung Diseases for Radiologists.
Lee KC, Kang EY, Yong HS, Kim C, Lee KY, Hwang SH, Oh YW
Korean J Radiol 2019 Sep;20(9):1368-1380. doi: 10.3348/kjr.2019.0057. PMID: 31464115Free PMC Article

Therapy

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Emerging biomarkers of lymphangioleiomyomatosis.
Nijmeh J, El-Chemaly S, Henske EP
Expert Rev Respir Med 2018 Feb;12(2):95-102. Epub 2017 Dec 1 doi: 10.1080/17476348.2018.1409622. PMID: 29171770
Lymphangioleiomyomatosis.
Johnson SR, Taveira-DaSilva AM, Moss J
Clin Chest Med 2016 Sep;37(3):389-403. doi: 10.1016/j.ccm.2016.04.002. PMID: 27514586
Tuberous sclerosis complex.
Henske EP, Jóźwiak S, Kingswood JC, Sampson JR, Thiele EA
Nat Rev Dis Primers 2016 May 26;2:16035. doi: 10.1038/nrdp.2016.35. PMID: 27226234
Diffuse Cystic Lung Disease. Part I.
Gupta N, Vassallo R, Wikenheiser-Brokamp KA, McCormack FX
Am J Respir Crit Care Med 2015 Jun 15;191(12):1354-66. doi: 10.1164/rccm.201411-2094CI. PMID: 25906089Free PMC Article

Prognosis

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Single-Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell.
Guo M, Yu JJ, Perl AK, Wikenheiser-Brokamp KA, Riccetti M, Zhang EY, Sudha P, Adam M, Potter A, Kopras EJ, Giannikou K, Potter SS, Sherman S, Hammes SR, Kwiatkowski DJ, Whitsett JA, McCormack FX, Xu Y
Am J Respir Crit Care Med 2020 Nov 15;202(10):1373-1387. doi: 10.1164/rccm.201912-2445OC. PMID: 32603599Free PMC Article
Lymphangioleiomyomatosis.
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
Pulmonary manifestations in tuberous sclerosis complex.
Gupta N, Henske EP
Am J Med Genet C Semin Med Genet 2018 Sep;178(3):326-337. Epub 2018 Jul 28 doi: 10.1002/ajmg.c.31638. PMID: 30055039Free PMC Article
Lymphangioleiomyomatosis.
Kitaichi M, Izumi T
Curr Opin Pulm Med 1995 Sep;1(5):417-24. PMID: 9363104

Clinical prediction guides

Lymphangioleiomyomatosis: pathogenesis, clinical features, diagnosis, and management.
McCarthy C, Gupta N, Johnson SR, Yu JJ, McCormack FX
Lancet Respir Med 2021 Nov;9(11):1313-1327. Epub 2021 Aug 27 doi: 10.1016/S2213-2600(21)00228-9. PMID: 34461049
Single-Cell Transcriptomic Analysis Identifies a Unique Pulmonary Lymphangioleiomyomatosis Cell.
Guo M, Yu JJ, Perl AK, Wikenheiser-Brokamp KA, Riccetti M, Zhang EY, Sudha P, Adam M, Potter A, Kopras EJ, Giannikou K, Potter SS, Sherman S, Hammes SR, Kwiatkowski DJ, Whitsett JA, McCormack FX, Xu Y
Am J Respir Crit Care Med 2020 Nov 15;202(10):1373-1387. doi: 10.1164/rccm.201912-2445OC. PMID: 32603599Free PMC Article
Lymphangioleiomyomatosis.
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
Emerging biomarkers of lymphangioleiomyomatosis.
Nijmeh J, El-Chemaly S, Henske EP
Expert Rev Respir Med 2018 Feb;12(2):95-102. Epub 2017 Dec 1 doi: 10.1080/17476348.2018.1409622. PMID: 29171770
Multiple cystic lung disease.
Ferreira Francisco FA, Soares Souza A Jr, Zanetti G, Marchiori E
Eur Respir Rev 2015 Dec;24(138):552-64. doi: 10.1183/16000617.0046-2015. PMID: 26621970Free PMC Article

Recent systematic reviews

Clinical features and outcomes of male patients with lymphangioleiomyomatosis: A review.
Zhang H, Hu Z, Wang S, Wu K, Yang Q, Song X
Medicine (Baltimore) 2022 Dec 30;101(52):e32492. doi: 10.1097/MD.0000000000032492. PMID: 36596036Free PMC Article
Clear cell sugar tumor of the lung; a systematic review for a rare entity.
Ntanasis-Stathopoulos I, Kyriazoglou A, Tsilimigras DI, Tzanninis IG, Liontos M, Dimopoulos MA, Gavriatopoulou M
J BUON 2021 Jan-Feb;26(1):17-32. PMID: 33721428
A Systematic Review of Lymphangioleiomyomatosis on Diagnosis and Molecular Mechanism.
Dong X, Jin L, Wang A, Wu L, Fan X, Hou Q, Li T, Zhao R, Zhang Y
Biomed Res Int 2021;2021:6612776. Epub 2021 Feb 10 doi: 10.1155/2021/6612776. PMID: 33628792Free PMC Article
The efficacy and safety of pharmacological treatments for lymphangioleiomyomatosis.
Wang Q, Luo M, Xiang B, Chen S, Ji Y
Respir Res 2020 Feb 14;21(1):55. doi: 10.1186/s12931-020-1316-3. PMID: 32059669Free PMC Article
The efficacy and adverse events of mTOR inhibitors in lymphangioleiomyomatosis: systematic review and meta-analysis.
Gao N, Zhang T, Ji J, Xu KF, Tian X
Orphanet J Rare Dis 2018 Aug 14;13(1):134. doi: 10.1186/s13023-018-0874-7. PMID: 30107845Free PMC Article

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