A Case of Malignant Perivascular Epithelioid Cell Carcinoma of the Adrenal Gland

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  • 副腎に発生した悪性PEComaの1例
  • 症例 副腎に発生した悪性PEComaの1例
  • ショウレイ フクジン ニ ハッセイ シタ アクセイ PEComa ノ 1レイ

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Abstract

Perivascular epithelioid cell carcinoma (PEComa) or PEC tumor is a family of mesenchymal tumors which exists in the perivascular areas, has a multi-differentiating potential and is derived from the perivascular epithelioid cells (PEC).<BR>A 65-year-old woman presented to our hospital because of heart burn. An abdominal CT scan revealed a clearly demarcated tumor with the diameter of about 7 cm and the heterogeneous center part at the dorsal aspect of the stomach. Angiography showed the main feeders to be the left renal and adrenal arteries. A FDG-PET study revealed an abnormal uptake of FDG to the tumor, so that we decided to carry out surgery. During surgery, we confirmed that the tumor was adjacent to the left adrenal gland, suggesting that the tumor might have arisen from the left adrenal gland. Histopathology revealed proliferation of spindle-shaped cells which showed a change to multinuclear cells with abundant in cytoplasm, and tumor cells were HMB-45 positive and Melan A positive, with a high positive conversion rate to MIB1. Malignant PEComa was thus diagnosed. She developed multiple hepatic and pulmonary metastases 2 months and died 6 months after the surgery.<BR>This paper deals with a case of malignant PEComa of the adrenal gland, together with a review of the literature.

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