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Epithelioid inflammatory myofibroblastic sarcoma: a case report.

Daniel E Sarmiento1, Jessica A Clevenger1, Gregory A Masters1

  • 11 Department of Surgery, 2 Department of Pathology, Christiana Care Health System, Newark, DE 19718, USA ; 3 Department of Medical Oncology, 4 Department of Thoracic Surgery, Helen F. Graham Cancer Center & Research Institute, Newark, DE 19713, USA.

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Epithelioid inflammatory myofibroblastic sarcoma (EIMS), a rare lung malignancy, showed significant response to ALK-targeted therapy. This targeted approach offers a promising treatment for EIMS patients with ALK gene rearrangement.

Keywords:
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Area of Science:

  • Oncology
  • Genetics
  • Pathology

Background:

  • Inflammatory myofibroblastic tumor (IMT) is a rare lung malignancy with variable clinical behavior.
  • Epithelioid inflammatory myofibroblastic sarcoma (EIMS) is a malignant variant of IMT, often affecting young individuals.

Observation:

  • A case of pleural EIMS initially treated with surgery and chemotherapy is presented.
  • The tumor exhibited an anaplastic lymphoma kinase (ALK) gene rearrangement.

Findings:

  • Treatment with an ALK-directed tyrosine kinase inhibitor resulted in an impressive and sustained remission.
  • This highlights the efficacy of targeted therapy in EIMS with ALK alterations.

Implications:

  • ALK-targeted therapy represents a potential breakthrough for managing EIMS.
  • Further research into the pathogenesis and targeted treatment of EIMS is warranted.