*Department of Pathology, Japanese Red Cross Nagoya Daiichi Hospital, 3-35 Michishita-cho, Nakamura-ku, Nagoya-city, Aichi 453-8511, Japan.
Jpn J Clin Oncol. 2014 Jan;44(1):93-6. doi: 10.1093/jjco/hyt173. Epub 2013 Nov 24.
Inflammatory myofibroblastic tumor is a rare tumor deriving from mesenchymal tissue. Approximately 50% of inflammatory myofibroblastic tumors harbor an anaplastic lymphoma kinase fusion gene. Pulmonary inflammatory myofibroblastic tumors harboring tropomyosin3-anaplastic lymphoma kinase or protein tyrosine phosphatase receptor-type F polypeptide-interacting protein-binding protein 1-anaplastic lymphoma kinase have been reported previously. However, it has not been reported that inflammatory myofibroblastic tumors harbor echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene which is considered to be very specific to lung cancers. A few tumors harboring echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene other than lung cancers have been reported and the tumors were all carcinomas. A 67-year-old man had been followed up for a benign tumor for approximately 3 years before the tumor demonstrated malignant transformation. Lobectomy and autopsy revealed that an inflammatory myofibroblastic tumor harboring echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion gene had transformed into an undifferentiated sarcoma. This case suggests that echinoderm microtubule-associated protein-like 4-anaplastic lymphoma kinase fusion is an oncogenic event in not only carcinomas but also sarcomas originating from stromal cells.
炎性肌纤维母细胞瘤是一种罕见的来源于间叶组织的肿瘤。大约 50%的炎性肌纤维母细胞瘤含有间变性淋巴瘤激酶融合基因。先前已有报道携带有原肌球蛋白 3-间变性淋巴瘤激酶或蛋白酪氨酸磷酸酶受体型 F 多肽-相互作用蛋白结合蛋白 1-间变性淋巴瘤激酶的肺炎性肌纤维母细胞瘤。然而，尚未报道炎性肌纤维母细胞瘤含有被认为对肺癌非常特异的棘皮动物微管相关蛋白样 4-间变性淋巴瘤激酶融合基因。除肺癌外，也有少数报道携带有棘皮动物微管相关蛋白样 4-间变性淋巴瘤激酶融合基因的肿瘤，这些肿瘤均为癌。一名 67 岁男性，其良性肿瘤已被随访约 3 年，后肿瘤发生恶性转化。肺叶切除术和尸检显示，携带有棘皮动物微管相关蛋白样 4-间变性淋巴瘤激酶融合基因的炎性肌纤维母细胞瘤已转化为未分化肉瘤。该病例提示棘皮动物微管相关蛋白样 4-间变性淋巴瘤激酶融合不仅是癌，而且也是来源于间质细胞的肉瘤的致癌事件。
