Primary epithelioid inflammatory myofibroblastic sarcoma of the brain with EML4::ALK fusion mimicking intra-axial glioma: a case report and brief literature review

Author: Eric Eunshik KIM ¹ ; Chul-Kee PARK ; Koung Mi KANG ; Yoonjin KWAK ; Sung-Hye PARK ; Jae-Kyung WON
Author Information

1. Departments of Pathology, Seoul National University Hospital, Seoul National University College of Medicine, Seoul, Korea
Publication Type:Case Study
From:Journal of Pathology and Translational Medicine 2024;58(3):141-145
CountryRepublic of Korea
Language:EN
Abstract: 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.