分子標的薬による逐次的治療を行ったXp11.2転座型腎細胞癌

Metastatic Xp11.2 translocation renal cell carcinoma treated with sequential therapy of molecular targeted agents : A case report

　21歳男性．主訴は腹部膨満と食思不振であった．CTで右腎の巨大な腫瘍，多発肝・肺転移，下大静脈腫瘍血栓が認められた．腫瘍生検の免疫染色でTFE3抗体に対し陽性，Fluorescence in situ hybridization法で，Xp11.2領域の転座陽性細胞が証明された．MSKCCのリスク分類では，予後不良群であり，治療はファーストラインがテムシロリムス，セカンドラインがスニチニブ，サードラインがソラフェニブ，4thラインがアキシチニブ，5thラインが再度テムシロリムスを投与し，生存期間は26か月であった．転移性Xp11.2転座型腎細胞癌に対する分子標的薬の逐次的治療の有効性が示された．

Abstract

We report a case of Xp11.2 translocation renal cell carcinoma (RCC) treated with sequential therapy of molecular targeted agents. A 21-year-old man presented abdominal distension and anorexia. The computed tomography showed the right kidney occupied with huge, calcificated tumor with extension to inferior vena cava and multiple metastasis to liver and lung. Immunohistochemical stainig of needle biopsy of right kidney showed that the expression of intracellular TFE was positive. Cytogenic analysis conducted using TFE3 split-fluorescence in-situ hybridization confirmed Xp11.2 translocation RCC. Classified as being in the Memorial Sloan Kettering Cancer Center poor prognosis group he received sequential therapy of targeted agents with temsirolimus, sunitinib, sorafenib, axitinib and rechallenge of temsirolimus. Overall survival was 26 months. This paper has demonstrated that currently available molecular targeted agents could be of practical options for patients with metastatic Xp11.2 translocation RCC (Rinsho Hinyokika 70 : 547-551, 2016).