肝原発悪性末梢神経鞘腫の1例 [in Japanese] A Case of Malignant Peripheral Nerve Sheath Tumor in the Hepatic Portal Region Successfully Treated with Surgical Resection [in Japanese]
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症例は71歳，男性．近医にて多発肝嚢胞の経過観察中，肝胆道系酵素の上昇を認め，精査にて肝内胆管癌が疑われ，手術目的に当科紹介となった．腹部造影CT検査にて肝S4/5にかけて40mm大の辺縁に造影効果を伴う腫瘍性病変を認め，肝内胆管癌の診断となり，肝中央2区域切除・尾状葉切除・肝外胆管切除・胆管空腸吻合術を施行した．病理組織検査の結果，悪性末梢神経鞘腫（Malignant Peripheral Nerve Sheath Tumor:MPNST）の診断となった．術後胆汁漏を併発したが，保存的加療にて軽快し第72病日に退院となった．<BR>MPNSTはSchwann細胞由来の悪性腫瘍であり，軟部組織悪性腫瘍の約5％を占め，中でも予後の悪い疾患の一つである．肝原発のMPNSTは非常に稀な疾患であり，術前の鑑別を含め若干の文献的考察を加え報告する．
A 71-year-old man with multiple hepatic cysts was referred to our hospital because of a solitary liver tumor. Abdominal CT scan revealed a solid 40-mm mass in hepatic segments 4 and 5 with heterogeneous enhancement of its marginal lesion. Under the diagnosis of hilar cholangiocarcinoma, he underwent surgical resection by central bisegmentectomy with caudate lobe and extrahepatic bile duct resection and reconstruction by intrahepaticojejunostomy.<BR>Pathological analysis revealed that the tumor predominantly comprised severely atypical spindle-shaped cells with fascicular pattern and severe perineural invasion. Immunohistochemical analysis revealed that the tumor was positive for S-100 and vimentin and negative for CD34, c-kit, desmin, and Smooth muscle actin (SMA), typical for a malignant peripheral nerve sheath tumor (MPNST). So far, he has survived for 12 months without recurrence.<BR>MPNSTs are relatively rare soft tissue sarcomas that develop from Schwann cells. They account for approximately 5% of all soft tissue sarcomas. An MPNST developing in the hepatic portal region is very rare. We report a case of a patient successfully treated with aggressive surgical resection.
- Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association)
Nihon Rinsho Geka Gakkai Zasshi (Journal of Japan Surgical Association) 74(11), 3140-3144, 2013
Japan Surgical Association