AI Article Synopsis
- PEComa is a rare type of tumor mainly found in gynecology, making diagnosis challenging for doctors.
- Three cases of gynecological PEComas (in the uterus, vagina, and retroperitoneum) are discussed, highlighting their treatment and follow-up results.
- There is little consensus on the best treatment options due to the rarity of these tumors, but surgical removal is standard, while new therapies like m-TOR inhibitors show promise for recurrent cases.
Article Abstract
Antecedents: Perivascular epitheliod cell tumor (PEComa) is a rare mesenchymal tumor. They are rare in the field of gynecology, which makes them difficult to consider as a possible diagnostic. We aim to contribute with our experience to ease clinical practice to others gynecologists.
Patients And Methods: We contribute to literature with three gynecological cases; uterine, vaginal and retroperitoneal PEComas.
Results: The uterine and vaginal PEComa, have 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: Given the scarcity of cases, the literature consists of case reports and mini-reviews. Some authors have categorized the PEComas based on prognostic factors, but there is no agreement regarding the follow-up and treatment. F-FDG-PET/CT can help characterize these lesions. The surgery is the standard. In recurrent or malignant cases, there is a lack of evidence regarding chemotherapy and radiotherapy. New therapies with inhibitory m-TOR open a hopeful therapeutic window.
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| http://dx.doi.org/10.1016/j.medcli.2019.01.009 | DOI Listing |
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Article Synopsis
- PEComa is a rare type of tumor mainly found in gynecology, making diagnosis challenging for doctors.
- Three cases of gynecological PEComas (in the uterus, vagina, and retroperitoneum) are discussed, highlighting their treatment and follow-up results.
- There is little consensus on the best treatment options due to the rarity of these tumors, but surgical removal is standard, while new therapies like m-TOR inhibitors show promise for recurrent cases.
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