Abstract
Perivascular epithelioid cell tumor is a rare tumor. To date, there is no consensus of therapy to be recommended for unresectable disease. For a low incidence and a rarely curable disease, the finding of new therapy is essential. Here we report the first case of a patient with perivascular epithelioid cell tumor whose disease had a rapid progression after surgery and had a rapid remarkable response of combination therapy of a VEGFR inhibitor, sorafenib, with an mTOR inhibitor, sirolimus. This result may have potential to deliver a new treatment option and inhibiting the mTOR pathway combined with inhibiting the VEGF pathways may be a useful strategy for malignant PEComas.
Keywords:
Combination therapy; VEGFR inhibitor; mTOR inhibitor; perivascular epithelioid cell tumor; sirolimus; sorafenib.
MeSH terms
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Antineoplastic Agents / administration & dosage
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Antineoplastic Agents / pharmacology
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Antineoplastic Agents / therapeutic use*
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Female
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Humans
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Middle Aged
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Niacinamide / administration & dosage
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Niacinamide / analogs & derivatives*
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Niacinamide / pharmacology
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Niacinamide / therapeutic use
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Perivascular Epithelioid Cell Neoplasms / drug therapy*
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Perivascular Epithelioid Cell Neoplasms / pathology
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Phenylurea Compounds / administration & dosage
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Phenylurea Compounds / pharmacology
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Phenylurea Compounds / therapeutic use*
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Sirolimus / administration & dosage
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Sirolimus / pharmacology
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Sirolimus / therapeutic use*
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Sorafenib
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TOR Serine-Threonine Kinases
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Uterine Neoplasms / drug therapy*
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Uterine Neoplasms / pathology
Substances
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Antineoplastic Agents
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Phenylurea Compounds
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Niacinamide
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Sorafenib
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MTOR protein, human
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TOR Serine-Threonine Kinases
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Sirolimus