Perivascular Epithelioid Cell Neoplasms

A family of mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelioid cells. These cells do not have a normal anatomic homolog. (From Fletcher CDM, et. al., World Health Organization Classification of Tumors: Pathology and Genetics of Tumors of Soft Tissue and Bone, 2002).
Also Known As:
CCMMT; Neoplasms, Perivascular Epithelioid Cell; Perivascular Epithelioid Cell Tumors; Clear Cell Myomelanocytic Tumors; Clear Cell Sugar Tumors; Clear-Cell Myomelanocytic Tumor; Clear-Cell Sugar Tumor; Myomelanocytic Tumor, Clear-Cell; Myomelanocytic Tumors, Clear-Cell; PEComa; Sugar Tumor, Clear-Cell; Sugar Tumors, Clear-Cell; Tumor, Clear-Cell Myomelanocytic; Tumor, Clear-Cell Sugar; Tumors, Clear-Cell Myomelanocytic; Tumors, Clear-Cell Sugar; Clear-Cell Myomelanocytic Tumors; Clear-Cell Sugar Tumors; PEComas
Networked: 113 relevant articles (2 outcomes, 8 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1. Neoplasms (Cancer)
2. Perivascular Epithelioid Cell Neoplasms
3. Lymphangioleiomyomatosis (Lymphangiomyomatosis)
4. Renal Cell Carcinoma (Grawitz Tumor)
5. Alveolar Soft Part Sarcoma


1. Martignoni, Guido: 6 articles (03/2015 - 02/2008)
2. Bonetti, Franco: 4 articles (03/2015 - 02/2008)
3. Pea, Maurizio: 4 articles (03/2015 - 02/2008)
4. Rao, Qiu: 4 articles (11/2014 - 03/2013)
5. Folpe, Andrew L: 4 articles (01/2012 - 06/2002)
6. Fisher, Cyril: 3 articles (10/2015 - 12/2005)
7. Brunelli, Matteo: 3 articles (04/2015 - 01/2012)
8. Zamboni, Giuseppe: 3 articles (03/2015 - 02/2008)
9. Zhou, Xiao-Jun: 3 articles (11/2014 - 06/2013)
10. Xia, Qiu-Yuan: 3 articles (11/2014 - 06/2013)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Perivascular Epithelioid Cell Neoplasms:
1. Sirolimus (Rapamycin)FDA Link
2. Glycoproteins (Glycoprotein)IBA
3. Cathepsin KIBA
4. Microphthalmia-Associated Transcription FactorIBA
5. MART-1 AntigenIBA
6. Carbon MonoxideIBA
7. temsirolimusFDA Link
8. Adenocarcinoma of lungIBA
01/28/2015 - "Therefore, we finally made a diagnosis of malignant perivascular epithelioid cell tumor (PEComa) arising in the gastric serosa, combined with primary lung adenocarcinoma. "
09/01/2015 - "The most significant changes in this edition involve (1) use of immunohistochemistry throughout the classification, (2) a new emphasis on genetic studies, in particular, integration of molecular testing to help personalize treatment strategies for advanced lung cancer patients, (3) a new classification for small biopsies and cytology similar to that proposed in the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (4) a completely different approach to lung adenocarcinoma as proposed by the 2011 Association for the Study of Lung Cancer/American Thoracic Society/European Respiratory Society classification, (5) restricting the diagnosis of large cell carcinoma only to resected tumors that lack any clear morphologic or immunohistochemical differentiation with reclassification of the remaining former large cell carcinoma subtypes into different categories, (6) reclassifying squamous cell carcinomas into keratinizing, nonkeratinizing, and basaloid subtypes with the nonkeratinizing tumors requiring immunohistochemistry proof of squamous differentiation, (7) grouping of neuroendocrine tumors together in one category, (8) adding NUT carcinoma, (9) changing the term sclerosing hemangioma to sclerosing pneumocytoma, (10) changing the name hamartoma to "pulmonary hamartoma," (11) creating a group of PEComatous tumors that include (a) lymphangioleiomyomatosis, (b) PEComa, benign (with clear cell tumor as a variant) and "
9. StreptavidinIBA
10. PolymersIBA

Therapies and Procedures

1. Drug Therapy (Chemotherapy)
2. Hysterectomy
3. Cystectomy
4. Radiotherapy
5. Nephrectomy