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Clear cell-sugar-tumor of the lung

MedGen UID:
232370
Concept ID:
C1333065
Neoplastic Process
Synonyms: CCSTL; Clear Cell-Sugar-Tumor of Lung; clear cell-Sugar-tumor of lung; Clear Cell-Sugar-Tumor of the Lung; clear cell-sugar-tumor of the lung; clear cell-Sugar-tumor of the lung; Lung Clear Cell Tumor; lung clear cell tumor; lung clear cell tumour; lung clear cell-sugar-tumor; Sugar Tumor; Sugar tumor; Sugar tumour
 
Monarch Initiative: MONDO:0003637

Definition

A rare benign lung tumor with perivascular epithelioid cell differentiation. It is composed of round or oval cells with abundant clear or eosinophilic cytoplasm and distinct cell borders. The vast majority of patients are asymptomatic and the tumors are discovered incidentally. Excision is curative. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVClear cell-sugar-tumor of the lung

Professional guidelines

PubMed

Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906
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
Rufino R
J Bras Pneumol 2015 Jul-Aug;41(4):295-6. doi: 10.1590/S1806-37132015000400001. PMID: 26398747Free PMC Article

Recent clinical studies

Etiology

Cottin V, Blanchard E, Kerjouan M, Lazor R, Reynaud-Gaubert M, Taille C, Uzunhan Y, Wemeau L, Andrejak C, Baud D, Bonniaud P, Brillet PY, Calender A, Chalabreysse L, Court-Fortune I, Desbaillets NP, Ferretti G, Guillemot A, Hardelin L, Kambouchner M, Leclerc V, Lederlin M, Malinge MC, Mancel A, Marchand-Adam S, Maury JM, Naccache JM, Nasser M, Nunes H, Pagnoux G, Prévot G, Rousset-Jablonski C, Rouviere O, Si-Mohamed S, Touraine R, Traclet J, Turquier S, Vagnarelli S, Ahmad K; OrphaLung network
Respir Med Res 2023 Jun;83:101010. Epub 2023 Mar 24 doi: 10.1016/j.resmer.2023.101010. PMID: 37087906
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
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
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
Weinstock M, Vaidya A, El-Chemaly S, Godleski J, Henske E
N Engl J Med 2018 Mar 29;378(13):e18. doi: 10.1056/NEJMimc1708586. PMID: 29590548

Diagnosis

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
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
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
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
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

Therapy

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
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
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
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
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

Prognosis

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
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
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
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
Kitaichi M, Izumi T
Curr Opin Pulm Med 1995 Sep;1(5):417-24. PMID: 9363104

Clinical prediction guides

Xu W, Yang C, Cheng C, Wang Y, Hu D, Huang J, He Y, Wang J, Chen K, Yang L, Zhou W, Zhang T, Liu S, Dai J, Meng S, Li X, Yang Y, Wang ST, Feng R, Zhang W, Zhang H, Wang L, Tian X, Xu KF
Chest 2023 Jul;164(1):137-148. Epub 2023 Feb 18 doi: 10.1016/j.chest.2023.02.026. PMID: 36801466
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
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
Doubková M, Štefániková M, Čan V, Merta Z, Svoboda M
Klin Onkol 2019 Fall;32(5):367-374. doi: 10.14735/amko2019367. PMID: 31610670
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

Recent systematic reviews

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
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
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
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
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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