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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

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
New guidelines for LAM diagnosis and treatment.
Lobo P
Lancet Respir Med 2016 Nov;4(11):862. Epub 2016 Oct 11 doi: 10.1016/S2213-2600(16)30311-3. PMID: 27742353
Treatment of lymphangioleiomyomatosis and Camões.
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

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
The Verge of Collapse.
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
Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.
Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW
Am J Surg Pathol 2005 Dec;29(12):1558-75. doi: 10.1097/01.pas.0000173232.22117.37. PMID: 16327428
NHLBI Workshop Summary. Report of workshop on lymphangioleiomyomatosis. National Heart, Lung, and Blood Institute.
Am J Respir Crit Care Med 1999 Feb;159(2):679-83. doi: 10.1164/ajrccm.159.2.9803107. PMID: 9927387

Diagnosis

Lymphangioleiomyomatosis and Other Cystic Lung Diseases.
Koslow M, Lynch DA, Cool CD, Groshong SD, Downey GP
Immunol Allergy Clin North Am 2023 May;43(2):359-377. doi: 10.1016/j.iac.2023.01.003. PMID: 37055093Free PMC Article
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
Lymphangioleiomyomatosis.
Chen BC, Liao CY, Hung YC, Chan SH, Li MF
QJM 2021 Oct 7;114(6):421-422. doi: 10.1093/qjmed/hcab036. PMID: 33599269
Lymphangioleiomyomatosis (LAM).
Ataya A, Brantly M, Riley L
Am J Respir Crit Care Med 2018 Aug 15;198(4):P7-P8. doi: 10.1164/rccm.1984P7. PMID: 30109949

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
Lymphangioleiomyomatosis (LAM).
Ataya A, Brantly M, Riley L
Am J Respir Crit Care Med 2018 Aug 15;198(4):P7-P8. doi: 10.1164/rccm.1984P7. PMID: 30109949
Lymphangioleiomyomatosis.
Sclafani A, VanderLaan P
N Engl J Med 2018 Jun 7;378(23):2224. doi: 10.1056/NEJMicm1712581. PMID: 29874537
The Verge of Collapse.
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
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

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
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
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
Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.
Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW
Am J Surg Pathol 2005 Dec;29(12):1558-75. doi: 10.1097/01.pas.0000173232.22117.37. PMID: 16327428

Clinical prediction guides

Determinants of Progression and Mortality in Lymphangioleiomyomatosis.
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
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
Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature.
Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW
Am J Surg Pathol 2005 Dec;29(12):1558-75. doi: 10.1097/01.pas.0000173232.22117.37. PMID: 16327428

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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