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Advanced Malignant PEComa

MedGen UID:
1717409
•Concept ID:
C5236089
•
Neoplastic Process

Definition

A malignant PEComa that has spread extensively to other anatomic sites or is no longer responding to treatment. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVAdvanced Malignant PEComa

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
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197

Recent clinical studies

Etiology

Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature.
Zizzo M, Ugoletti L, Tumiati D, Castro Ruiz C, Bonacini S, Panebianco M, Sereni G, Manenti A, Lococo F, Carlinfante G, Pedrazzoli C
Pancreatology 2018 Apr;18(3):238-245. Epub 2018 Feb 19 doi: 10.1016/j.pan.2018.02.007. PMID: 29478828
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
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
PEComa: morphology and genetics of a complex tumor family.
Thway K, Fisher C
Ann Diagn Pathol 2015 Oct;19(5):359-68. Epub 2015 Jun 9 doi: 10.1016/j.anndiagpath.2015.06.003. PMID: 26144278
Renal artery embolization-indications, technical approaches and outcomes.
Muller A, Rouvière O
Nat Rev Nephrol 2015 May;11(5):288-301. Epub 2014 Dec 23 doi: 10.1038/nrneph.2014.231. PMID: 25536394

Diagnosis

Update on Uterine Mesenchymal Neoplasms.
Kertowidjojo EC, Bennett JA
Surg Pathol Clin 2022 Jun;15(2):315-340. Epub 2022 May 20 doi: 10.1016/j.path.2022.02.008. PMID: 35715164
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
Update on the Diagnosis and Management of Renal Angiomyolipoma.
Flum AS, Hamoui N, Said MA, Yang XJ, Casalino DD, McGuire BB, Perry KT, Nadler RB
J Urol 2016 Apr;195(4 Pt 1):834-46. Epub 2015 Nov 21 doi: 10.1016/j.juro.2015.07.126. PMID: 26612197
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

Therapy

New targeted treatments for advanced sarcomas.
Li CC, Chen TW
Curr Opin Oncol 2023 Jul 1;35(4):309-314. Epub 2023 May 3 doi: 10.1097/CCO.0000000000000955. PMID: 37222206
Prospective nanoparticle treatments for lymphangioleiomyomatosis.
Landh E, Wang R, Moir LM, Traini D, Young PM, Ong HX
Expert Opin Drug Deliv 2022 Jan;19(1):75-86. Epub 2022 Jan 21 doi: 10.1080/17425247.2022.2029401. PMID: 35043744
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
Medical treatment of advanced malignant perivascular epithelioid cell tumors.
Fabbroni C, Sbaraglia M, Sanfilippo R
Curr Opin Oncol 2020 Jul;32(4):301-306. doi: 10.1097/CCO.0000000000000649. PMID: 32541317
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

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
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
Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature.
Zizzo M, Ugoletti L, Tumiati D, Castro Ruiz C, Bonacini S, Panebianco M, Sereni G, Manenti A, Lococo F, Carlinfante G, Pedrazzoli C
Pancreatology 2018 Apr;18(3):238-245. Epub 2018 Feb 19 doi: 10.1016/j.pan.2018.02.007. PMID: 29478828
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
Diffuse cystic lung diseases.
Ryu JH, Tian X, Baqir M, Xu K
Front Med 2013 Sep;7(3):316-27. Epub 2013 May 11 doi: 10.1007/s11684-013-0269-z. PMID: 23666611

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
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
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
Towards personalised therapy for lymphangioleiomyomatosis: lessons from cancer.
El-Chemaly S, Henske EP
Eur Respir Rev 2014 Mar 1;23(131):30-5. doi: 10.1183/09059180.00008813. PMID: 24591659Free PMC Article
Diffuse cystic lung diseases.
Ryu JH, Tian X, Baqir M, Xu K
Front Med 2013 Sep;7(3):316-27. Epub 2013 May 11 doi: 10.1007/s11684-013-0269-z. PMID: 23666611

Recent systematic reviews

Primary pancreatic perivascular epithelioid cell tumor (PEComa): A surgical enigma. A systematic review of the literature.
Zizzo M, Ugoletti L, Tumiati D, Castro Ruiz C, Bonacini S, Panebianco M, Sereni G, Manenti A, Lococo F, Carlinfante G, Pedrazzoli C
Pancreatology 2018 Apr;18(3):238-245. Epub 2018 Feb 19 doi: 10.1016/j.pan.2018.02.007. PMID: 29478828

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