Onkologie. 2025:19(2):108-110 | DOI: 10.36290/xon.2025.022
Management of epitheloid inflammatory myofibroblastic sarcoma of pulmonary origin
- 1 Oddelenie pneumológie a ftizeológie, Fakultná nemocnica F. D. Roosevelta v Banskej Bystrici
- 2 Klinika pneumológie a ftizeológie, Univerzita Komenského v Bratislave, Jesseniova lekárska fakulta v Martine
Epitheloid inflammatory myofibroblastic sarcoma (EIMS) is a rare variant of the inflammatory myofibroblastic tumour (IMT). Unlike the classic IMT, EIMS typically occurs intra-abdominally and is characterized by an adverse prognosis due to its aggressive nature, high rate of recurrence, and metastatic potential. Histologically, it exhibits epitheloid morphology and a unique immunohistochemical profile, which allows to distinguish EIMS from other mesenchymal tumours, thus significantly contributing to diagnostic accuracy. Immunohistochemically, ALK (anaplastic lymphoma kinase) protein positivity is characteristic with perinuclear or membrane staining pattern. The most common fusion partners of the ALK gene (2p3) are the RANBP2, RRBP1, and EML4 genes. This genetic aberration plays a crucial role in diagnosis and represents a potential target for targeted therapy. EIMS is a rare diagnostic entity whose rarity and aggressive biological behaviour pose significant diagnostic and therapeutic challenges. The article presents a case of a 45-year-old man diagnosed with EIMS of primary pulmonary origin after right upper lobectomy suffering from repeated headache episodes and diplopia. Imaging studies revealed metastatic brain involvement that, despite targeted therapy and repeated metastasectomies, tended to recur over the years. This case highlights the extremely aggressive nature of EIMS and its high metastatic potential that complicates the treatment of patients with this diagnosis. Despite advancements in targeted therapy, the treatment of metastatic EIMS remains a major challenge for the oncologist.
Keywords: epitheloid inflammatory myofibroblastic sarcoma, targeted therapy, ALK rearrangement, metastatic brain involvement.
Accepted: April 25, 2025; Published: May 2, 2025 Show citation
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