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Sclerosing PEComa

MedGen UID:
888878
•Concept ID:
C4054048
•
Neoplastic Process

Definition

A tumor with perivascular epithelioid cell differentiation characterized by the presence of cords of neoplastic cells in a densely collagenous stroma. [from NCI]

Term Hierarchy

CClinical test,  RResearch test,  OOMIM,  GGeneReviews,  VClinVar  
  • CROGVSclerosing PEComa

Professional guidelines

PubMed

The diagnosis and management of diabetic mastopathy.
Thorncroft K, Forsyth L, Desmond S, Audisio RA
Breast J 2007 Nov-Dec;13(6):607-13. doi: 10.1111/j.1524-4741.2007.00489.x. PMID: 17983405

Recent clinical studies

Etiology

Sclerosing variant of PEComa: report of a case and review of the literature.
Santi R, Franchi A, Villari D, Paglierani M, Pepi M, Danielli D, Nicita G, Nesi G
Histol Histopathol 2012 Sep;27(9):1175-81. doi: 10.14670/HH-27.1175. PMID: 22806904
The morphologic spectrum of uterine PEC-cell associated tumors in a patient with tuberous sclerosis.
Lim GS, Oliva E
Int J Gynecol Pathol 2011 Mar;30(2):121-8. doi: 10.1097/PGP.0b013e3181fa5a99. PMID: 21293289
Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum.
Hornick JL, Fletcher CD
Am J Surg Pathol 2008 Apr;32(4):493-501. doi: 10.1097/PAS.0b013e318161dc34. PMID: 18223480

Diagnosis

Sclerosing PEComa of the kidney: clinicopathologic analysis of 2 cases and review of the literature.
Zhao Y, Bui MM, Spiess PE, Dhillon J
Clin Genitourin Cancer 2014 Oct;12(5):e229-32. Epub 2014 Jun 12 doi: 10.1016/j.clgc.2014.04.009. PMID: 25044147
Pararenal sclerosing PEComa.
Leão RR, Pereira BJ, Grenha V, Coelho H
BMJ Case Rep 2013 Apr 17;2013 doi: 10.1136/bcr-2013-009097. PMID: 23598939Free PMC Article
Retroperitoneal sclerosing PEComa with melanin pigmentation and granulomatous inflammation-a rare association within an uncommon tumor.
Rekhi B, Sable M, Desai SB
Indian J Pathol Microbiol 2012 Jul-Sep;55(3):395-8. doi: 10.4103/0377-4929.101757. PMID: 23032844
The morphologic spectrum of uterine PEC-cell associated tumors in a patient with tuberous sclerosis.
Lim GS, Oliva E
Int J Gynecol Pathol 2011 Mar;30(2):121-8. doi: 10.1097/PGP.0b013e3181fa5a99. PMID: 21293289

Prognosis

Pararenal sclerosing PEComa.
Leão RR, Pereira BJ, Grenha V, Coelho H
BMJ Case Rep 2013 Apr 17;2013 doi: 10.1136/bcr-2013-009097. PMID: 23598939Free PMC Article
Sclerosing variant of PEComa: report of a case and review of the literature.
Santi R, Franchi A, Villari D, Paglierani M, Pepi M, Danielli D, Nicita G, Nesi G
Histol Histopathol 2012 Sep;27(9):1175-81. doi: 10.14670/HH-27.1175. PMID: 22806904
Malignant variant of sclerosing perivascular epithelioid cell tumor arising in the adnexa.
Ramaiah S, Ganesan R, Mangham DC, McNally O, Klys HS, Hirschowitz L
Int J Gynecol Pathol 2009 Nov;28(6):589-93. doi: 10.1097/PGP.0b013e3181a3a4de. PMID: 19851212
Sclerosing PEComa: clinicopathologic analysis of a distinctive variant with a predilection for the retroperitoneum.
Hornick JL, Fletcher CD
Am J Surg Pathol 2008 Apr;32(4):493-501. doi: 10.1097/PAS.0b013e318161dc34. PMID: 18223480

Clinical prediction guides

Sclerosing variant of PEComa: report of a case and review of the literature.
Santi R, Franchi A, Villari D, Paglierani M, Pepi M, Danielli D, Nicita G, Nesi G
Histol Histopathol 2012 Sep;27(9):1175-81. doi: 10.14670/HH-27.1175. PMID: 22806904

Supplemental Content

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