副腎に発生した悪性PEComaの1例  [in Japanese] A Case of Malignant Perivascular Epithelioid Cell Carcinoma of the Adrenal Gland  [in Japanese]

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Abstract

Perivascular epithelioid cell tumor(PEComa)は血管周囲に存在し多分化能を有するperivascular epithelioid cell(PEC)に由来する間葉系腫瘍の一群である.症例は65歳の女性,胸やけを主訴に受診.腹部CT検査で胃背側に径約7cmの境界明瞭・内部不均一の腫瘤を認め,血管造影検査でmain feederは左腎動脈と副腎動脈であった.PET検査では異常集積を認め,手術方針となった.術中所見で腫瘍は左副腎に近接しており,左副腎原発と考えられた.病理所見は紡錘状細胞が豊富な胞体を有する多核巨細胞などの変化を示し増殖しており,HMB45陽性,MelanA陽性,MIB1陽性率高率で悪性 PEComaの診断に至った.術後2カ月で多発肝・肺転移が出現し,術後6カ月で死亡した.副腎原発悪性PEComaの1例を経験したので,文献的考察を含めて報告する.

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.

Journal

  • Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)

    Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 79(7), 1528-1533, 2018

    Japan Surgical Association

Codes

  • NII Article ID (NAID)
    130007584467
  • NII NACSIS-CAT ID (NCID)
    AA11189709
  • Text Lang
    JPN
  • ISSN
    1345-2843
  • NDL Article ID
    029183588
  • NDL Call No.
    Z19-227
  • Data Source
    NDL  J-STAGE 
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