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Sunil Kumar 1 Article
Primary pulmonary epithelioid inflammatory myofibroblastic sarcoma: a rare entity and a literature review
Priyanka Singh, Aruna Nambirajan, Manish Kumar Gaur, Rahul Raj, Sunil Kumar, Prabhat Singh Malik, Deepali Jain
J Pathol Transl Med. 2022;56(4):231-237.   Published online July 7, 2022
DOI: https://doi.org/10.4132/jptm.2022.05.08
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AbstractAbstract PDF
Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is an aggressive subtype of inflammatory myofibroblastic tumor (IMT) harboring anaplastic lymphoma kinase (ALK) gene fusions and is associated with high risk of local recurrence and poor prognosis. Herein, we present a young, non-smoking male who presented with complaints of cough and dyspnoea and was found to harbor a large right lower lobe lung mass. Biopsy showed a high-grade epithelioid to rhabdoid tumor with ALK and desmin protein expression. The patient initially received 5 cycles of crizotinib and remained stable for 1 year; however, he then developed multiple bony metastases, for which complete surgical resection was performed. Histopathology confirmed the diagnosis of EIMS, with ALK gene rearrangement demonstrated by fluorescence in situ hybridization. Postoperatively, the patient is asymptomatic with stable metastatic disease on crizotinib and has been started on palliative radiotherapy. EIMS is a very rare subtype of IMT that needs to be included in the differential diagnosis of ALKexpressing lung malignancies in young adults.

Citations

Citations to this article as recorded by  
  • Mediastinal epithelioid inflammatory myofibroblastic sarcoma with the EML4‐ALK fusion: A case report and literature review
    Tingyu Pan, Xinyu Sun, Xiao Wu, Futing Tang, Xianmei Zhou, Qian Wang, Shi Chen
    Respirology Case Reports.2024;[Epub]     CrossRef
  • Case report: Epithelioid inflammatory myofibroblastic sarcoma treated with an ALK TKI ensartinib
    Mengmeng Li, Ruyue Xing, Jiuyan Huang, Chao Shi, Chunhua Wei, Huijuan Wang
    Frontiers in Oncology.2023;[Epub]     CrossRef
  • Epithelioid Inflammatory Myofibroblastic Sarcoma With Poor Response to Crizotinib: A Case Report
    Soheila Aminimoghaddam, Roghayeh Pourali
    Clinical Medicine Insights: Case Reports.2023;[Epub]     CrossRef
  • Epithelioid inflammatory myofibroblastic sarcoma: a case report and brief literature review
    Weidong Dou, Yu Guan, Tao Liu, Hang Zheng, Shuo Feng, Yingchao Wu, Xin Wang, Zhanbing Liu
    Frontiers in Oncology.2023;[Epub]     CrossRef

J Pathol Transl Med : Journal of Pathology and Translational Medicine