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Perivascular Epithelioid Cell Neoplasms

Subscribe to New Research on 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

Neoplasms: 649836
- Neoplasms by Histologic Type: 1
  - Connective and Soft Tissue Neoplasms
    - Perivascular Epithelioid Cell Neoplasms: 113
      - Lymphangioleiomyomatosis: 722
      - Angiomyolipoma: 613

Related Diseases

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

Experts

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 06/01/2015 - "The exact genetic alterations underlying the pathophysiology of PEComas are largely unknown, although it has been shown that activation of the Mammalian target of rapamycin (mTOR) signaling pathway plays a pivotal role. " 04/01/2013 - "Preliminary reports have indicated that the mTOR inhibitors sirolimus and related drugs have activity in some patients with non-TSC-associated PEComa. " 09/01/2012 - "Then, in the face of a strong rationale, mammalian target of rapamycin (mTOR) inhibitors are active in a proportion of PEComas (perivascular epithelioid cell tumours) and crizotinib in ALK-rearranged inflammatory myofibroblastic tumours. " 08/20/2010 - "Resistance to mammalian target of rapamycin inhibitor therapy in perivascular epithelioid cell tumors." 02/10/2010 - "On this mechanistic basis, we treated three consecutive patients with metastatic PEComa with an oral mTOR inhibitor, sirolimus. "
2.	Glycoproteins (Glycoprotein)IBA 03/01/2011 - "A recent study has suggested that perivascular epithelioid cell neoplasms routinely express CD1a, a Langerhans cell-associated transmembrane glycoprotein involved in antigen presentation and that expression of this marker may be helpful in the distinction of perivascular epithelioid cell neoplasms from various mimics. " 08/01/1998 - "HMB-45, an antibody directed against a premelanosome glycoprotein, although used predominantly for the diagnosis of MM, has shown consistent staining in angiomyolipoma (AML), lymphangiomyoma/lymphangiomyomatosis (LAM), and clear cell sugar tumor (CCST), a group of tumors proposed to be related on the basis of their common perivascular epithelioid cells, which exhibit various degrees of smooth muscle differentiation, melanogenesis, and intracytoplasmic membrane bound granules. "
3.	Cathepsin KIBA 01/01/2012 - "The aim of this study is to evaluate cathepsin K immunohistochemically in the entire spectrum of PEComa lesions in the kidney. " 06/01/2013 - "Cathepsin K immunostaining demonstrates high sensitivity and specificity in PEComa, better than other commonly employed immunomarkers. " 03/01/2013 - "Our aim was to investigate the expression of cathepsin K in a wide spectrum of extrarenal PEComas and evaluate its potential diagnostic usefulness in comparison with other commonly used markers. " 01/01/2012 - "In all of the renal PEComas, cathepsin K was found to be constantly and strongly expressed and seems to be a more powerful marker than other commonly used markers for their identification, especially to confirm the diagnosis on needle biopsies." 10/01/2010 - "All PEComas with TFE3 genetic alterations immunolabeled strongly for Cathepsin K, similar to other PEComas. "
4.	Microphthalmia-Associated Transcription FactorIBA 06/01/2002 - "Histologic examination showed the classic features of CCMMT, and immunohistochemical studies confirmed co-expression of smooth muscle actin, HMB-45, and microphthalmia transcription factor." 01/01/2014 - "Recent studies have demonstrated that cathepsin K seems to be a powerful marker in identifying the microphthalmia associated transcription factor (MITF) family tumors such as renal perivascular epithelioid cell neoplasms (PEComas), alveolar soft part sarcoma, and translocation-associated renal cell carcinomas. " 04/01/2007 - "Four cases of malignant PEComas were stained with smooth muscle actin, muscle specific actin, desmin, human melanoma black-45, melan-A, microphthalmia transcription factor, S100 and cyclin D1. "
5.	MART-1 AntigenIBA 05/01/2015 - "The unique combination of immunoreactivity for melanocytic markers, such as HMB-45 and Melan A, and myogenic markers, such as smooth muscle actin, is the hallmark of PEComas biological behavior, and prognosis of malignant PEComas is yet to be fully understood. " 01/01/2013 - "Hepatic PEComas are quite rare and diagnosis involves positivity of Melan-A and HMB45 on immunohistochemistry. " 12/01/2011 - "Specifically: (1) Melanoma markers (Melan A, MiTF) typical of PEComas are expressed by a substantial fraction of GISTs. " 09/01/2000 - "HMB-45/melan-A and smooth muscle actin-positive clear-cell epithelioid tumor arising in the ligamentum teres hepatis: additional example of clear cell 'sugar' tumors." 05/07/2015 - "The lesion pathologically showed eosinophilic proliferation, in addition to immunohistochemical positivity for human melanoma black 45 and Melan-A, thereby leading to the diagnosis of a hepatic perivascular epithelioid cell tumor (PEComa). "
6.	Carbon MonoxideIBA 09/01/2012 - "Tumours of perivascular epithelioid cells (PEComas) are a heterogeneous group of uncommon mesenchymal neoplasms which exhibit a peculiar immunohistochemical co-expression of muscle and melanocytic markers. " 01/01/2012 - "Perivascular epithelioid cell (PEC) tumors (PEComas) are a family of related mesenchymal tumors composed of PECs which co-express melanocytic and smooth muscle markers. " 06/01/2005 - "Perivascular epithelioid cell tumor (PEComa) is a group of rare tumors composed of epithelioid cells with characteristic perivascular distribution and co-expression of the melanogenic marker HMB-45 and muscular markers. " 01/01/2015 - "Defined as a family of scarce mesenchymal neoplasm which distinctively co-express melanocytic markers and muscle markers, perivascular epithelioid cell tumors (PEComas) have been reported almost everybody site. " 01/01/2014 - "Perivascular epithelioid cell tumors (PEComas) are rare tumors characterized by co-expression of melanocytic and smooth muscle markers. "
7.	temsirolimus FDA Link 05/01/2010 - "Treatment with the mTOR inhibitor temsirolimus in patients with malignant PEComa." 07/01/2014 - "Our study confirms that mTOR inhibition with sirolimus/temsirolimus is well-tolerated with good radiological responses, albeit short-lived, and supports its use in PEComas." 07/01/2014 - "A retrospective study of patients with malignant PEComa receiving treatment with sirolimus or temsirolimus: the Royal Marsden Hospital experience." 06/01/2010 - "Inhibitors of mTOR (sirolimus, temsirolimus) demonstrated an antitumoral activity in the PEComas. " 01/01/2014 - "Since the mTOR pathway is often altered in PEComas and responses have been reported with mTOR-inhibitors such as sirolimus or temsirolimus, we decided to start a neoadjuvant treatment with sirolimus. "
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 03/01/2011 - "A streptavidin-biotin and polymer-based detection system immunohistochemical study of perivascular epithelioid cell neoplasms and their morphologic mimics."
10.	PolymersIBA 03/01/2011 - "Ten Carolinas Medical Center positive perivascular epithelioid cell neoplasms were negative when retested a Mayo Clinic Rochester, using a polymer-based detection system. " 03/01/2011 - "A streptavidin-biotin and polymer-based detection system immunohistochemical study of perivascular epithelioid cell neoplasms and their morphologic mimics."

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2015 - " present 2 cases of GI PEComas, one of which showed an aggressive histologic behavior that underwent multiple combined chemotherapies. " 01/01/2012 - "Cytotoxic chemotherapy and radiation therapy have shown little benefit in treating PEComa-NOS; mTOR inhibition is emerging as a treatment option. " 05/01/2010 - "We reported a 14-year-old patient of unresectable malignant PEComa, who was treated with multiple combined chemotherapies. " 09/01/2009 - "Conversely, patients with PEComa who initially received surgery and chemotherapy or radiation therapy were associated with a higher disease recurrence rate (P =.024). " 01/01/2007 - "To date, most malignant cases of PEComa have been resistant to chemotherapy, and as such, an appropriate therapy is not known. "
2.	Hysterectomy 01/01/2002 - "Seven of the eight patients with PEComas were treated by hysterectomy. " 01/01/2010 - "Only four cervical PEComa cases have been described, and were treated with hysterectomy and radiotherapy.We" 03/01/2011 - "We report a 59-year-old woman with tuberous sclerosis, initially diagnosed with a uterine malignant mixed müllerian tumor, in whom the hysterectomy specimen also showed 3 other incidental tumors: a subserosal angiomyolipoma consisting of blood vessels of varying caliber, mature adipose tissue, and nests of spindle cells arranged in a perivascular location, a sclerosing PEComa consisting of small nests of clear epithelioid cells present in a background of marked sclerosis centered in the lower uterine segment/endocervix, and diffuse lymphangiomyomatosis involving the endocervix, lower uterine segment, and uterine corpus. "
3.	Cystectomy 04/01/2015 - "Surgical treatment (partial or radical cystectomy) is the most common management.A long-term follow-up and larger series are required to better understand the best clinical approach to bladder PEComa." 05/01/2013 - "We report the case of a 64-year-old man who underwent total cystectomy because of a primary malignant PEComa of the bladder in August 2010. "
4.	Radiotherapy 01/01/2010 - "Only four cervical PEComa cases have been described, and were treated with hysterectomy and radiotherapy.We" 03/01/2009 - "An 11-year-old girl was diagnosed with ALL secondary to chemoradiotherapy (vincristine, ifosfamide, and anthracycline) and radiotherapy comprising 45 Gy to the whole pelvis for PEComa. "
5.	Nephrectomy 02/01/2013 - " The patient underwent a right radical nephrectomy, and pathology revealed a renal epithelioid PEComa." 06/01/2004 - "She underwent a right radical nephrectomy that showed a PEComa and an adjacent renal cell carcinoma. "