Fertility sparing treatment of a malignant uterine perivascular epithelioid cell tumor: A case report☆
نویسندگان
چکیده
•Perivascular epithelioid cell tumors (PEComas) are a family of rare, poorly defined mesenchymal tumors of uncertain malignant potential.•Treatment for PEComas has most commonly involved excisional biopsy or surgical resection.•The use of mTOR inhibitors may provide the best medical treatment as well as a fertility-sparing treatment option.
منابع مشابه
Fertility-sparing operation for recurrence of uterine cervical perivascular epithelioid cell tumor
Perivascular epithelioid cell tumors (PEComa) are mesenchymal tumors composed of histologically and immunohistochemically distinctive perivascular epithelial cells. Although the uterine corpus seems to be one of the most prevalent sites of involvement, PEComa of the uterine cervix are very rare. Only four cervical PEComa cases have been described, and were treated with hysterectomy and radiothe...
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BACKGROUND Perivascular epithelioid cell tumor (PEComa) is a rare subtype of mesenchymal origin tumor composed of epithelioid cells which exhibits immunohistochemical co-expressions of melanocytic markers and smooth muscle markers. CASE PRESENTATION In the first case, malignant uterine PEComa with vaginal and multiple lung metastasis was misdiagnosed preoperatively as uterine leiomyosarcoma d...
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Uterine perivascular epithelioid cell tumors (PEComas) are rare mesenchymal tumors. Many have malignant behavior, and no successful treatment strategy has been established. Identification of mutations in the tuberous sclerosis 1 (TSC1) and TSC2 genes producing constitutive activation of the mammalian target of rapamycin (mTOR) pathway presents an opportunity for targeted therapy. Patients with ...
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BACKGROUND Perivascular epithelioid cell tumor (PEComa), other than angiomyolipoma (AML), clear cell sugar tumor (CCST), and lymphangioleiomyomatosis (LAM), is a very rare mesenchymal tumor with an unpredictable natural history. The uterus is the most prevalent reported site of involvement of PEComa-not otherwise specified (PEComa-NOS). To the best of our knowledge, about 100 PEComa-NOS have be...
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Perivascular epithelioid cell tumors (PEComas) represent a rare group of tumours with uncertain malignancy potential exhibiting an immunophenotype characterized by actin and Human Melanoma Black 45 (HMB45) immunoreactivity. Our case concerns about a rare malignant uterine perivascular epithelioid cell tumour diagnosed in a patient underwent to subtotal hysterectomy with unclear diagnosis, 12 ye...
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