非典型的な画像所見・病理所見を呈した小児多発性肝限局性結節性過形成類似病変の1例 [in Japanese] A pediatric case of multiple hepatic focal nodular hyperplasia-like lesions presenting as atypical imaging and histopathological findings [in Japanese]
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画像所見が非典型的で，肝細胞癌との鑑別が困難な小児多発性肝限局性結節性過形成類似病変（FNH-like lesion）の症例を経験した．症例は10歳女児で，右下肢痛の経過観察中，MRIで偶然多発肝腫瘤影を指摘された．ガドキセト酸ナトリウム（Gd-EOB-DTPA）による造影MRIで，大部分の腫瘤は限局性結節性過形成（FNH）に一致する所見だった．ところがS3の腫瘤が高分化型肝細胞癌を疑う所見だったため，腹腔鏡下肝腫瘍切除を行った．切除組織の病理診断は肝細胞の過形成性病変で，肝細胞癌は否定された．しかし，FNH以外の他の過形成性病変との区別は困難で，最終診断はFNH-like lesionとした．<br/>FNHと同様に肝内血管走行異常が原因で生じる過形成性病変で，FNHの病理所見を満たさないものをFNH-like lesionと呼ぶ．本症は組織像だけでなく画像上もFNHに非典型的な所見を呈し得る．画像上肝細胞癌が疑われるが，他の臨床像が悪性を示唆しない場合，本疾患も念頭に置く必要がある．
We encountered the case of a 10-year-old girl presenting with multiple hepatic focal nodular hyperplasia (FNH)-like lesions, which were difficult to differentiate from hepatocellular carcinoma (HCC) because of their atypical presentation. She was referred to our institution for an incidentally detected multiple liver mass during the follow-up for thigh pain. Enhanced MRI with gadolinium ethoxybenzyl diethylenetriamine-pentaacetic acid (Gd-EOB-DTPA) showed compatible observation in most nodules. However, a mass in S3 showed hypointensity in the hepatocellular phase, which is characteristic of HCC. We performed laparoscopic tumorectomy to rule out malignancy. A histopathological study showed benign hyperplastic lesions, but further classification was difficult. Thus, our final diagnosis was FNH-like lesions.<br/>FNH-like lesions are hyperplastic lesions caused by a vascular abnormality similar to a typical FNH, although their features are not compatible with those of FNH. This disease may be difficult to distinguish from FNH or HCC because the imaging findings as well as the histopathological findings are atypical. We have to consider this disease when we see an imaging study indicating HCC, despite a benign clinical picture.
- The Japanese Journal of Pediatric Hematology / Oncology
The Japanese Journal of Pediatric Hematology / Oncology 52(5), 430-434, 2015
The Japanese Society of Pediatric Hematology / Oncology