Perivascular epithelioid cell tumourperivascular epithelioid cell tumours
Perivascular epithelioid cell tumour, also known as PEComa or PEC tumour, is a family of mesenchymal tumours consisting of perivascular epithelioid cells PECs1 These are rare tumours that can occur in any part of the human body
The cell type from which these tumours originate remains unknown Normally, no perivascular epitheloid cells exist; the name refers to the characteristics of the tumour when examined under the microscope2
Establishing the malignant potential of these tumours remains challenging although criteria3 have been suggested; some PEComas display malignant features whereas others can cautiously be labeled as having 'uncertain malignant potential'2 The most common tumours in the PEComa family are renal angiomyolipoma and pulmonary lymphangioleiomyomatosis, both of which are more common in patients with tuberous sclerosis complex The genes responsible for this multi-system genetic disease have also been implicated in other PEComas2
Many PEComa types shows a female predominance in the sex ratio
- 1 Cause
- 2 Diagnosis
- 21 Histology
- 22 Immunohistochemical markers
- 23 Differential diagnosis
- 3 References
The precursor cell of PEComas is currently unknown; there is no normal counterpart "perivascular epitheloid cell"1 Genetically, PECs are linked to the tuberous sclerosis genes TSC1 and TSC2, although this link is stronger for angiomyolipoma and lymphangioleiomyomatosis than for other members of the PEComa family
PECs consist of perivascular epithelioid cells with a clear/granular cytoplasm and central round nucleus without prominent nucleoli
PECs typically stain for melanocytic markers HMB-45, Melan A Mart 1, Mitf and myogenic markers actin, myosin, calponin
PECs bear significant histologic and immunohistochemical similarity to:
- clear-cell sugar tumour CCST,
- lymphangioleiomyomatosis, and,
- clear-cell myomelanocytic tumour of ligamentum teres/falciform ligament
- abdominopelvic sarcoma of perivascular epitheloid cells2
- primary extrapulmonary sugar tumour2
Thus, it has been advocated that the above could be classified PEComas1
PEComas are rare and can have myriad features; therefore, they can be confused with carcinomas, smooth muscle tumours, adipocytic tumours, clear cell sarcomas, melanomas and gastrointestinal stromal tumours GIST2
- ^ a b c Martignoni G, Pea M, Reghellin D, Zamboni G, Bonetti F February 2008 "PEComas: the past, the present and the future" Virchows Arch 452 2: 119–32 PMC 2234444 PMID 18080139 doi:101007/s00428-007-0509-1
- ^ a b c d e f Folpe, AL; Kwiatkowski DJ 2009 "Perivascular epitheloid cell neoplasms: pathology and pathogenesis" Human Pathology 41 1: 1–15 PMID 19604538 doi:101016/jhumpath200905011
- ^ Folpe AL, Mentzel T, Lehr HA, Fisher C, Balzer BL, Weiss SW Dec 2005 "Perivascular epithelioid cell neoplasms of soft tissue and gynecologic origin: a clinicopathologic study of 26 cases and review of the literature" Am J Surg Pathol 29 12: 1558–75 PMID 16327428 doi:101097/01pas00001732322211737
|Not otherwise specified||
|Connective tissue neoplasm||
|Complex mixed and stromal||
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