An autopsy case of intrahepatic cholangiocarcinoma associated with neurofibromatosis type 1 (von Recklinghausen's disease)

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  • 神経線維腫症1型（von Recklinghausen病）に合併した肝内胆管癌の1剖検例
  • 症例報告 神経線維腫症1型(von Recklinghausen病)に合併した肝内胆管癌の1剖検例
  • ショウレイ ホウコク シンケイ センイ シュショウ 1ガタ(von Recklinghausenビョウ)ニ ガッペイ シタ カン ナイタンカンガン ノ 1 ボウケンレイ

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Abstract

A 69-year old man, who had neurofibromatosis type 1 (von Recklinghausen's disease), was referred to our hospital because of a symptom of persistent abdominal fullness. The extensive examinations revealed huge hepatic mass in the left lobe, suggesting a diagnosis of intrahepatic cholangiocarcinoma. One month later, the patient showed a rapid progress of anemia and bloody ascites suggestive of tumor rupture. Hepatic anteriography showed a large heterogeneous tumor in the left lateral lobe and transcathetel arterial embolization was performed. However, the patient died from hepato-renal failure. An autopsy revealed moderately-differentiated intrahepatic cholangiocarcinoma with necrosis and vascular invasion, metastasizing to the lungs, hilar lymph nodes, parietal pleura, right kidney, perisplenic fat, and adrenal glands. Intrahepatic cholangiocarcinoma associated with neurofibromatosis type 1 is rare.<br>

Journal

  • Kanzo

    Kanzo 53 (4), 231-236, 2012

    The Japan Society of Hepatology

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