Perivascular epithelioid cell tumors (PEComas)
Updates to Article Attributes
Perivascular epithelioid cells tumours (PEComas) are a group of related mesenchymal tumours and tumour-like conditions found in many locations. This group includes:
- angiomyolipoma (AML)
- clear cell 'sugar' tumour of the lung
- lymphangioleiomyomatosis (LAM)
-
clear cell myomelanocytic tumour (CCMMT)
- of the falciform ligament / ligamentum teres
- unusual clear tumours of other organs
- pancreas
- rectum
- abdominal serosa
- uterus
-: considered one of the commonest sites of involvement in the female pelvis - vulva
- heart
- orbit
Epidemiology
PEComas are far more common in females 4.
Associations
Pathology
Histology
All PEComas share similar and distinctive cellular morphology – peripheral epitheloid cells – which have no counterpart in normal tissue 4. These tumours appear as sheets and nests of epithelioid or spindle-shaped cells associated with blood vessel walls. They have granular eosinophilic cytoplasms.
Immunohistochemistry
PEComas are characterised by immunoreactivity to smooth muscle and melanocytic markers 4.
- smooth muscle: actin, desmin
- melanocytic: HMB-45, melan-A
Radiographic features
Unfortunately, due to non-specific imaging appearances and the scarcity of reported cases no specific imaging features are recognised. These lesions seem to usually be solid but can be centrally necrotic 5-7. They generally demonstrate prominent contrast enhancement 6.
Treatment and prognosis
PEComas usually behave in a benign fashion although local recurrence and even malignant behaviour are encountered, mimicking malignant sarcomas.
Surgical Surgical excision is usually curative.
Differential diagnosis
The differential diagnosis is broad and largely depends upon the location of the mass.
-<li>uterus - considered one of the commonest sites of involvement in the female pelvis</li>- +<li>uterus: considered one of the commonest sites of involvement in the female pelvis</li>
-<li>smooth muscle: <a title="Smooth muscle actin" href="/articles/smooth-muscle-actin">actin</a>, desmin</li>- +<li>smooth muscle: <a href="/articles/smooth-muscle-actin">actin</a>, desmin</li>
-</ul><h4>Radiographic features</h4><p>Unfortunately, due to non-specific imaging appearances and the scarcity of reported cases no specific imaging features are recognised. These lesions seem to usually be solid but can be centrally necrotic <sup>5-7</sup>. They generally demonstrate prominent contrast enhancement <sup>6</sup>. </p><h4>Treatment and prognosis</h4><p>PEComas usually behave in a benign fashion although local recurrence and even malignant behaviour are encountered, mimicking malignant sarcomas. </p><p>Surgical excision is usually curative. </p><h4>Differential diagnosis</h4><p>The differential diagnosis is broad and largely depends upon the location of the mass.</p>- +</ul><h4>Radiographic features</h4><p>Unfortunately, due to non-specific imaging appearances and the scarcity of reported cases no specific imaging features are recognised. These lesions seem to usually be solid but can be centrally necrotic <sup>5-7</sup>. They generally demonstrate prominent contrast enhancement <sup>6</sup>. </p><h4>Treatment and prognosis</h4><p>PEComas usually behave in a benign fashion although local recurrence and even malignant behaviour are encountered, mimicking malignant sarcomas. Surgical excision is usually curative. </p><h4>Differential diagnosis</h4><p>The differential diagnosis is broad and largely depends upon the location of the mass.</p>
References changed:
- 1. Lai H, Chen C, Lee Y, Tsai P, Chen J, Shen W. Multicentric Aggressive Angiomyolipomas: A Rare Form of PEComas. AJR Am J Roentgenol. 2006;186(3):837-40. <a href="https://doi.org/10.2214/AJR.04.1639">doi:10.2214/AJR.04.1639</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16498117">Pubmed</a>
- 2. Fadare O, Parkash V, Yilmaz Y et al. Perivascular Epithelioid Cell Tumor (PEComa) of the Uterine Cervix Associated with Intraabdominal "PEComatosis": A Clinicopathological Study with Comparative Genomic Hybridization Analysis. World J Surg Oncol. 2004;2(1):35. <a href="https://doi.org/10.1186/1477-7819-2-35">doi:10.1186/1477-7819-2-35</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/15494070">Pubmed</a>
- 3. Armah H & Parwani A. Malignant Perivascular Epithelioid Cell Tumor (PEComa) of the Uterus with Late Renal and Pulmonary Metastases: A Case Report with Review of the Literature. Diagn Pathol. 2007;2(1):45. <a href="https://doi.org/10.1186/1746-1596-2-45">doi:10.1186/1746-1596-2-45</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/18053181">Pubmed</a>
- 4. Hornick J & Fletcher C. PEComa: What Do We Know So Far? Histopathology. 2006;48(1):75-82. <a href="https://doi.org/10.1111/j.1365-2559.2005.02316.x">doi:10.1111/j.1365-2559.2005.02316.x</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16359539">Pubmed</a>
- 5. Lubo I, Fermín I, Massarelli O, Gobbi R, Cossu Rocca P. Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature. Case Rep Pathol. 2016;2016:1936421. <a href="https://doi.org/10.1155/2016/1936421">doi:10.1155/2016/1936421</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26925281">Pubmed</a>
- 6. Shen H, Chen D, Sun X et al. MRI Diagnosis of Perivascular Epithelioid Cell Tumor (PEComa) of the Liver. Rom J Morphol Embryol. 2013;54(3):643-7. - <a href="https://www.ncbi.nlm.nih.gov/pubmed/24068418">Pubmed</a>
- 7. Alam M, Mukherjee B, Krishnakumar S, Biswas J. Malignant Perivascular Epithelioid Cell Tumor of the Orbit: Report of a Case and Review of Literature. Indian J Ophthalmol. 2017;65(9):889-91. <a href="https://doi.org/10.4103/ijo.IJO_331_17">doi:10.4103/ijo.IJO_331_17</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28905842">Pubmed</a>
- 1. Lai HY, Chen CK, Lee YH et-al. Multicentric aggressive angiomyolipomas: a rare form of PEComas. AJR Am J Roentgenol. 2006;186 (3): 837-40. <a href="http://dx.doi.org/10.2214/AJR.04.1639">doi:10.2214/AJR.04.1639</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/16498117">Pubmed citation</a><div class="ref_v2"></div>
- 2. Fadare O, Parkash V, Yilmaz Y et-al. Perivascular epithelioid cell tumor (PEComa) of the uterine cervix associated with intraabdominal "PEComatosis": A clinicopathological study with comparative genomic hybridization analysis. World J Surg Oncol. 2007;2 (1): 35. <a href="http://dx.doi.org/10.1186/1477-7819-2-35">doi:10.1186/1477-7819-2-35</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC527874">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/15494070">Pubmed citation</a><span class="ref_v3"></span>
- 3. Armah HB, Parwani AV. Malignant perivascular epithelioid cell tumor (PEComa) of the uterus with late renal and pulmonary metastases: a case report with review of the literature. Diagn Pathol. 2007;2 (1): 45. <a href="http://dx.doi.org/10.1186/1746-1596-2-45">doi:10.1186/1746-1596-2-45</a> - <a href="http://www.ncbi.nlm.nih.gov/pmc/articles/PMC2213634">Free text at pubmed</a> - <a href="http://www.ncbi.nlm.nih.gov/pubmed/18053181">Pubmed citation</a><span class="ref_v3"></span>
- 4. Hornick JL, Fletcher CD. PEComa: what do we know so far?. Histopathology. 48 (1): 75-82. <a href="https://doi.org/10.1111/j.1365-2559.2005.02316.x">doi:10.1111/j.1365-2559.2005.02316.x</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/16359539">Pubmed</a> <span class="ref_v4"></span>
- 5. Lubo, Idania, Fermín, Ileana, Massarelli, Olindo, Gobbi, Roberta, Cossu Rocca, Paolo. Perivascular Epithelioid Cell Tumour with Intraorbital Location: Report of a Case and Review of the Literature. (2018) Case Reports in Pathology. 2016: 1936421. <a href="https://doi.org/10.1155/2016/1936421">doi:10.1155/2016/1936421</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/26925281">Pubmed</a> <span class="ref_v4"></span>
- 6. Shen HQ, Chen DF, Sun XH, Li X, Xu J, Hu XB, Li MQ, Wu T, Zhang RY, Li KZ. MRI diagnosis of perivascular epithelioid cell tumor (PEComa) of the liver. (2013) Romanian journal of morphology and embryology = Revue roumaine de morphologie et embryologie. 54 (3): 643-7. <a href="https://www.ncbi.nlm.nih.gov/pubmed/24068418">Pubmed</a> <span class="ref_v4"></span>
- 7. Md Shahid Alam, Bipasha Mukherjee, S Krishnakumar, Jyotirmay Biswas. Malignant perivascular epithelioid cell tumor of the orbit: Report of a case and review of literature. (2017) Indian Journal of Ophthalmology. 65 (9): 889. <a href="https://doi.org/10.4103/ijo.IJO_331_17">doi:10.4103/ijo.IJO_331_17</a> - <a href="https://www.ncbi.nlm.nih.gov/pubmed/28905842">Pubmed</a> <span class="ref_v4"></span>
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