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Case Reports
. 2019 Jan;98(2):e14072.
doi: 10.1097/MD.0000000000014072.

Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses

Chia-Hao Liu  1   2   3 , Wei-Ting Chao  1   3   4 , Shih-Chieh Lin  2   5   6 , Hei-Yu Lau  1   3   7 , Hua-Hsi Wu  1   3   8 , Peng-Hui Wang  1   2   3   9
Affiliations
Case Reports

Malignant perivascular epithelioid cell tumor in the female genital tract: Preferred reporting items for systematic reviews and meta-analyses

Chia-Hao Liu et al. Medicine (Baltimore). 2019 Jan.

Abstract

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.

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Conflict of interest statement

The authors declare that they have no competing interests.

Figures

Figure 1
Figure 1
Operative image and specimen. (A) A significant vaginal tumor could be seen via the vagina cuff.
Figure 2
Figure 2
Hematoxylin–eosin staining. The neoplastic cells are arranged in solid nests or lobules with round to ovoid nuclei with clear to eosinophilic cytoplasm. Hypercellularity, marked nuclear pleomorphism, tumor necrosis, and carcinoma-like feature are noted. Frequent mitotic figures are found (13/10 high-power fields), × 200.
Figure 3
Figure 3
The neoplastic cells are immunoreactive for melan-A and focally positive for human melanoma black 45, actin, and transcription factor E3. The S-100, cytokeratin, and paired-box gene 8 immunostains are negative, × 4.
Figure 4
Figure 4
PRISMA flow diagram.
See this image and copyright information in PMC

References

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