Perivascular epithelioid cell tumor of the uterus: Report of two cases and mini-review of the literature

Theofanakis, Charalampos; Thomakos, Nikolaos; Sotiropoulou, Maria; Rodolakis, Alexandros

doi:10.1016/j.ijscr.2016.09.017

Cited by 7 publications

(9 citation statements)

References 13 publications

Supporting

Mentioning

Contrasting

Unclassified

Order By: Relevance

“…Immunohistochemically, a perivascular epithelioid cell is characterized by positivity for melanocytic markers, such as HMB-45, melan-A, tyrosinase, microphthalmia transcription factor, and NKI/C3, and smooth muscle markers, such as smooth muscle actin, desmin, h-caldesmon, pan-muscle actin, muscle myosin, and calponin. [4,11,12] In agreement with a previous systematic review published in 2015, [9] the most sensitive marker to diagnose PEComa is HMB-45, and nearly all PEComas were found to be positive staining for HMB-45 (Table 3).…”

Section: Discussionsupporting

confidence: 86%

“…Our search yielded 12 citations. A total of 114 cases were described in this report, [1,4–14] and the clinical characteristics of these cases are summarized in Table 3. The age of patients with gynecological PEComa ranged from 9 to 79 years, with a peak incidence around the mid-40 s. The uterus was the most commonly involved site (n = 82) followed by the cervix (n = 12).…”

Section: Resultsmentioning

confidence: 99%

“…Furthermore, the optimal treatment for recurrence and/or distant metastases of PEComa remains uncertain. [4]…”

Section: Discussionmentioning

confidence: 99%

See 2 more Smart Citations

Malignant perivascular epithelioid cell tumor in the female genital tract

Liu

Chao

Lin³

et al. 2019

Medicine

View full text Add to dashboard Cite

Background:Perivascular epithelioid cell tumor (PEComa) is a rare mesenchymal tumor, located at various anatomic sites, including the female genital tract. This study aimed to evaluate the clinicopathological characteristics of patients with PEComa arising from the female genital tract.Methods:A retrospective study was conducted in Taipei Veterans General Hospital (Taipei VGH) between 2008 and 2018. All published English cases based on the Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) statement were also included in the current review.Results:A total of 114 women from PRISMA and 3 women from Taipei VGH were identified. The uterus was the most commonly involved site (82/114, 71.9%), followed by the cervix (12/114, 10.5%). Immunohistochemical staining showed that nearly all gynecological PEComas were positive for human melanoma black 45 (113/114, 99.1%). More than half of the gynecological PEComas were immunoreactive for desmin (50/85, 58.8%). Multi-modality treatment, including surgery and mammalian target of rapamycin (mTOR) inhibitors as targeted therapy, provided long-term disease-free survival (cure rate ranging from 50% to 100%, based on the different anatomic sites of the female genital tract).Conclusion:Multi-modality treatment, including cytoreductive surgery and mTOR inhibitors with/without chemotherapy and/or radiation, should be considered for the management of women with PEComas in the genital tract.

show abstract

Section: Discussionsupporting

confidence: 86%

Section: Resultsmentioning

confidence: 99%

See 1 more Smart Citation

Malignant perivascular epithelioid cell tumor in the female genital tract

Liu

Chao

Lin³

et al. 2019

Medicine

View full text Add to dashboard Cite

show abstract

“…5 Immunohistochemically, a perivascular epithelioid cell is characterized by positivity for melanocytic markers, such as HMB-45, melan-A, tyrosinase, microphthalmia transcription factor, and NKI/C3, and smooth muscle markers, such as smooth muscle actin, desmin, hcaldesmon, pan-muscle actin, muscle myosin and calponin. [6][7][8] PEComas are classified into benign, uncertain malignant potential and malignant based on the presence of worrisome features. PEComas that do not possess any of the worrisome features are benign.…”

Section: Introductionmentioning

confidence: 99%

Perivascular epithelioid cell tumour of the uterus: what do we know?

Wong

Kang

2020

Int J Reprod Contracept Obstet Gynecol

View full text Add to dashboard Cite

Perivascular epithelioid cell neoplasm (PEComa) is a rare mesenchymal tumour characterized by distinctive histological and immunohistochemical perivascular epithelioid cells. These tumours can be found in various anatomic sites, with gynaecologic PEComas accounting for nearly one-fourth of reported cases in the literature. However, due to its non-specific clinical presentation and a lack of definitive radiological appearance, the diagnosis of PEComas remains challenging. In this case report, we describe a 45-year old lady suffering from urinary retention secondary to large uterine fibroids, who then underwent a total hysterectomy. Postoperative histopathology with immunohistochemical stains confirmed an unexpected finding of uterine PEComa. Although the treatment of gynaecologic PEComas remains controversial, complete surgical resection with negative margins is recommended. A multidisciplinary approach will be beneficial in determining the necessity of adjuvant therapy such as targeted therapy with mTOR inhibitors, especially for PEComas which exhibit aggressive and high-risk features.

show abstract

“…Tumors involving the gynecologic tract account for just over 40% of the cases, and the uterus is the most commonly reported site. To our knowledge, Nicolas I, et al Perivascular epitheliod cell tumors: rare gynecological entities 5 87 cases of uterine PEComa and 3 cases of vaginal location have been described to date [1][2][3][4][5][6][7][8][9][10][11] .…”

Section: Introductionmentioning

confidence: 99%

Perivascular epitheliod cell tumors: Study of three gynecological cases

Nicolás

Fusté

Saco

et al. 2019

Medicina Clínica (English Edition)

View full text Add to dashboard Cite

Antecedents and objectives: Perivascular epitheliod cell tumor (PEComa) is a rare mesenchymal tumor composed of perivascular epitheliod cells (PEC). They are rare in the field of gynecology and have a heterogeneous clinical presentation, which makes them difficult for gynecologists to consider as a possible diagnostic option. We aim to contribute with our experience and review to ease clinical practice to others gynecologists. Patients and methods: We contribute to literature with three gynecological cases; uterine, vaginal and retroperitoneal PEComas with different evolution and treatment. Results: The uterine and vaginal PEComa, have only required surgical treatment, and are free of disease at 9 and 5 months respectively. The retroperitoneal PEComa has recurred at 72 months of follow-up in form of retroperitoneal mass and pulmonary lymphangioleomyomatosis, continues treatment with sirolimus with good tolerance and partial response. Discussion and conclusion: Given the scarcity of cases, there are no clinical trials and the literature consists of case reports and mini reviews. Some authors have categorized the PEComas based on prognostic factors, although it has not been possible to agree on a follow-up and treatment based on it. Most authors agree to perform surgical treatment. In case of recurrence or malignancy, the adjuvant treatment found in the literature is heterogeneous, with a lack of evidence regarding chemotherapy and radiotherapy. New therapies with inhibitory m-TOR open a hopeful therapeutic window. Nicolas I, et al. Perivascular epitheliod cell tumors: rare gynecological entities 3 F18-FDG PET/CT can help characterize these lesions showing intense or low uptake of FDG in malignant or benign PEComas respectively.

show abstract

Perivascular epithelioid cell tumor of the uterus: Report of two cases and mini-review of the literature

Cited by 7 publications

References 13 publications

Malignant perivascular epithelioid cell tumor in the female genital tract

Malignant perivascular epithelioid cell tumor in the female genital tract

Perivascular epithelioid cell tumour of the uterus: what do we know?

Perivascular epitheliod cell tumors: Study of three gynecological cases

Contact Info

Product

Resources

About