棚橋 義直

Sarcoidosis is a multisystemic granulomatous disease of unknown etiology. Hepatic involvement was reported in about 11% of patients with sarcoidosis. However, cases of sarcoidosis in which the granuloma is solitary and limited in the liver are very rare. A 51-year-old woman with tumors in the liver underwent extended left lobectomy with caudate lobectomy and bile duct resection. The tumor was located between segment 4 and the hilar region. Some daughter nodules were found in the left lobe, which were regarded as intrahepatic metastasis. Our case displayed clinical and radiologically distinct findings, which are very similar to those of hilar cholangiocarcinoma restricted to the liver. This report demonstrates that sarcoidosis can show solitary hepatic involvement in the absence of thoracic lymphadenopathy. In such a case, it is difficult to distinguish the diagnosis from other malignant neoplasms. In conclusion, the diagnosis of hepatic sarcoidosis has to be made through prudent and comprehensive investigations that include a full clinical history of sarcoidosis in other organs. Despite utilizing several detailed diagnostic modalities, the definitive diagnosis of cases of solitary sarcoidosis may remain difficult. In these cases, surgical treatment including liver resection should be considered in order to avoid missing a suitable opportunity for treatment. © 2011 S. Karger AG, Basel.

Cholangiocarcinoma, arising from bile duct epithelium, is categorized into intrahepatic cholangiocarcinoma (ICC) and extrahepatic cholangiocarcinoma (ECC), including hilarcholangiocarcinoma. Recently, there has been a worldwide increase in the incidence and mortality from ICC. Complete surgical resection is the only approach to cure the patients with ICC. However, locoregional extension of these tumors is usually advanced with intrahepatic and lymph-node metastases at the time of diagnosis. Resectability rates are quite low and variable (18%-70%). The five-year survival rate after surgical resection was reported to be 20%-40%. Median survival time after ICC resection was 12-37.4 mo. Only a small number of ICC cases, accompanied with ECC, gall bladder carcinoma, and ampullary carcinoma, have been reported in the studies of chemotherapy due to the rarity of the disease. However, in some reports, significant anti-cancer effects were achieved with a response rate of up to 40% and a median survival of one year. Although recurrence rate after hepatectomy is high for the patients with ICC, the residual liver and the lung are the main sites of recurrence after tentative curative surgical resection. Several patients in our study had a long-term survival with repeated surgery and chemotherapy. Repeated surgery, combined with new effective regimens of chemotherapy, could benefit the survival of ICC patients. © 2010 Baishideng.

Background: The results of 12 consecutive patients with unresectable advanced biliary tract carcinoma treated with first line chemotherapy of S1/cisplatin, combined surgical resection and second line chemotherapy of gemcitabine are evaluated. Patients and Methods: Eight patients with intrahepatic cholangiocarcinoma, 1 with extrahepatic cholangiocarcinoma and 3 with gallbladder carcinoma were included in the study. All patients were treated with S1/cisplatin. Two of the patients underwent combined surgical resection before and 2 after therapy. Second line chemotherapy of gemcitabine was administerd in 6 patients. Results: MST of the patients was 14.9 months. With S1/cisplatin therapy, 6 patients had PR and 4 had SD. Two patients with surgical resection after the therapy survived more than 3 years. Second line chemotherapy of gemcitabine with moderate effects and mild adverse effects was well tolerable. Conclusion: S1/cisplatin showed considerable anti-cancerous effects. Employing surgical resection for patients with good response may lead to the chance of long-term survival.

【目的】肝疾患の病態進行に伴う血小板減少の原因は未だ不明な部分も多い.血小板減少時におけるADAMTS-13の関与を明らかにすることを目的とした.【対象と方法】2004年6月〜2006年8月までに当院で生体肝移植術を施行した15歳以下の小児14例(男児8例,女児6例)を対象とした.周術期の血小板数,ADAMTS-13活性及びvon Willebrand因子活性を測定し,様々な因子との因果関係を検討した.【結果】生体肝移植後の血小板数は,術後2日目に最低値をとり,術後2週間までに術前値に回復した.術後2日目の血小板数と有意な相関がみられたのは,術前因子では,患者年齢,血小板数,PELDスコア,術中因子ではGRWRであった.血小板数回復率(術後7日/術後2日血小板数)は平均1.27±0.47であり,血液型不適合の3症例は全例が1未満であった.ADAMTS-13活性は7例で測定したが,術前と比べ術後7日目に有意な低下がみられた(123.38±65.81% vs.71.84±23.66%).術後7日目のADAMTS-13活性はプロトロンビン時間と有意に正の相関を示した.血小板数回復率1以上の症例は,1未満の症例と比べ術後7日目のADAMTS-13活性が有意に高かった(93.87±17.94% vs. 55.33±7.50%).血小板数回復率1未満の症例におけるADAMTS-13活性は, vWF活性と負の相関を示し,血小板数とは正の相関を示した.【結論】ADAMTS-13活性は肝移植前には肝疾患の進行度を,肝移植後にはグラフト機能を反映すると思われる.肝移植後のADAMTS-13活性の低下は血小板減少をきたしうる.

症例は57歳,男性.B型慢性肝炎による肝機能障害にて当院通院中であった.平成16年10月,突然の腹痛が出現し近医を受診.腹部CTにて腹腔内の血腫と肝に腫瘍性病変を認め当院に緊急入院となった.入院時の腹部CTではSpiegel葉に4.5cm×4.0cm大,S8に2.5cm大の早期濃染と網嚢内に充満する血腫を認めた.腹部血管造影では明らかなextravasationは認めなかったが,血腫の局在からSpiegel葉原発肝癌の破裂と診断しTAEを施行した.TAE後2ヵ月目にSpiegel葉切除,S8部分切除を施行した.病理組織学的にSpiegel葉の腫瘍は広範な壊死と凝血塊を伴う中分化型肝細胞癌と診断された.術後16ヵ月目に残肝多発再発,多発肺転移,傍大動脈リンパ節転移のため死亡した.本邦報告例13例の集計から網嚢内血腫はSpiegel葉を原発とする肝細胞癌の破裂に特徴的な画像所見と考えられた.(著者抄録)