肺高血圧症を伴った Pulmonary Meningotheliomatosis の一剖検例

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  • イガク ケンキュウ ジッシュウ レポート ハイ コウケツアツショウ オ トモナッタ Pulmonary Meningotheliomatosis ノ イチ ボウケンレイ
  • An Autopsy Case of Pulmonary Meningotheliomatosis Associated with Pulmonary Hypertension

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We report an autopsy case of 86-year-old female with 10 years follow-up medically treated for mitral and aortic valve regurgitation. Her heart failure got worse associated with pulmonary hypertension for the past two years. The patient was found to be dead at home most probably of cardiopulmonary arrest. At autopsy multiple minute lesions pigmented with brownish discoloration were found in both lungs. Histologically these nodules were composed of neoplastic cell foci with pathognomonic whorl formation. Immunohistochemically, the tumor cells were positive for EMA, NCAM and progesterone receptor, and negative for S-100 protein and neuroendocrine markers. These lesions were recognized as minute pulmonary meningothelial-like nodules/meningothelioma (so-called chemodectoma). Macroscopically, We also observed cor pulmonale and dilation of the pulmonary trunk artery. Histologically, vascular medial and intimal thickening as well as small vessel wall thickness were displayed, indicating the complication of pulmonary hypertension. Only eleven cases of meningotheliomatosis and chemodectomatosis have been reported so far. Here, we attempted pathological reevalution of pulmonary meningotheliomatosis associated with pulmonary hypertension.

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