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Metastatic Malignant PEComa

MedGen UID:
1720611
•Concept ID:
C5236088
•
Neoplastic Process

Definition

A malignant PEComa that has spread from its original site of growth to another anatomic site. [from NCI]

Professional guidelines

PubMed

Addition of Antiestrogen Treatment in Patients with Malignant PEComa Progressing to mTOR Inhibitors.
Sanfilippo R, Fabbroni C, Fucà G, Fumagalli E, Morosi C, Sbaraglia M, Gronchi A, Collini P, Dei Tos AP, Casali PG
Clin Cancer Res 2020 Oct 15;26(20):5534-5538. Epub 2020 Jun 30 doi: 10.1158/1078-0432.CCR-20-1191. PMID: 32605908
Clear Cell Atypical Fibroxanthoma: Clinicopathological Study of 6 Cases and Review of the Literature With Special Emphasis on the Differential Diagnosis.
Tardío JC, Pinedo F, Aramburu JA, Martínez-González MÁ, Arias D, Khedaoui R, Suárez-Massa D, Santonja C
Am J Dermatopathol 2016 Aug;38(8):586-92. doi: 10.1097/DAD.0000000000000465. PMID: 26848640
Aetiology, diagnosis and management of spontaneous perirenal haematomas.
Brkovic D, Moehring K, Doersam J, Pomer S, Kaelble T, Riedasch G, Staehler G
Eur Urol 1996;29(3):302-7. doi: 10.1159/000473765. PMID: 8740036

Recent clinical studies

Etiology

Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923

Diagnosis

A rare metastatic mesenteric malignant PEComa with TSC2 mutation treated with palliative surgical resection and nab-sirolimus: a case report.
Meredith L, Chao T, Nevler A, Basu Mallick A, Singla RK, McCue PA, Bowne WB, Jiang W
Diagn Pathol 2023 Apr 11;18(1):45. doi: 10.1186/s13000-023-01323-x. PMID: 37041531Free PMC Article
Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923

Therapy

A rare metastatic mesenteric malignant PEComa with TSC2 mutation treated with palliative surgical resection and nab-sirolimus: a case report.
Meredith L, Chao T, Nevler A, Basu Mallick A, Singla RK, McCue PA, Bowne WB, Jiang W
Diagn Pathol 2023 Apr 11;18(1):45. doi: 10.1186/s13000-023-01323-x. PMID: 37041531Free PMC Article

Prognosis

A rare metastatic mesenteric malignant PEComa with TSC2 mutation treated with palliative surgical resection and nab-sirolimus: a case report.
Meredith L, Chao T, Nevler A, Basu Mallick A, Singla RK, McCue PA, Bowne WB, Jiang W
Diagn Pathol 2023 Apr 11;18(1):45. doi: 10.1186/s13000-023-01323-x. PMID: 37041531Free PMC Article
Update on the imaging of malignant perivascular epithelioid cell tumors (PEComas).
Phillips CH, Keraliya AR, Shinagare AB, Ramaiya NH, Tirumani SH
Abdom Radiol (NY) 2016 Feb;41(2):368-76. doi: 10.1007/s00261-015-0568-8. PMID: 26867923

Clinical prediction guides

A rare metastatic mesenteric malignant PEComa with TSC2 mutation treated with palliative surgical resection and nab-sirolimus: a case report.
Meredith L, Chao T, Nevler A, Basu Mallick A, Singla RK, McCue PA, Bowne WB, Jiang W
Diagn Pathol 2023 Apr 11;18(1):45. doi: 10.1186/s13000-023-01323-x. PMID: 37041531Free PMC Article

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