Kim Eric Eunshik, Park Chul-Kee, Kang Koung Mi, Kwak Yoonjin, Park Sung-Hye, Won Jae-Kyung
Departments of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
Departments of Neurosurgery, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea.
J Pathol Transl Med. 2024 May;58(3):141-145. doi: 10.4132/jptm.2024.04.12. Epub 2024 May 14.
An aggressive subtype of inflammatory myofibroblastic tumor, epithelioid inflammatory myofibroblastic sarcoma occurs primarily inside the abdominal cavity, followed by a pulmonary localization. Most harbor anaplastic lymphoma kinase (ALK) gene rearrangements, with RANBP2 and RRBP1 among the well-documented fusion partners. We report the second case of primary epithelioid inflammatory myofibroblastic sarcoma of the brain, with a well-known EML4::ALK fusion. The case is notable for its intra-axial presentation that clinico-radiologically mimicked glioma.
上皮样炎性肌纤维母细胞肉瘤是炎性肌纤维母细胞瘤的一种侵袭性亚型,主要发生于腹腔内,其次为肺部。大多数病例存在间变性淋巴瘤激酶(ALK)基因重排,RANBP2和RRBP1是其中有充分文献记载的融合伴侣。我们报告了第二例原发性脑上皮样炎性肌纤维母细胞肉瘤,伴有著名的EML4::ALK融合。该病例因其轴内表现而值得关注,临床放射学表现酷似胶质瘤。