A rare inflammatory myofibroblastic tumor appearing both inside and outside the heart

Li, Jiarong; Liu, Jijia; Yao, Xingwang; Yang, Jinfu

doi:10.1186/s40001-024-01710-0

Cited by 2 publications

(1 citation statement)

References 32 publications

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“…Some rare locations include the esophagus [ 15 ], the pericardium [ 12 ], the heart [ 7 ], the spinal meninges [ 15 ], and the adrenal glands [ 16 , 17 ]. A case describing the simultaneous location of IMT lateral to the ascending aorta near the root and inside the atrial septum has been reported [ 18 ]. IMT may also occur in pregnant women, potentially leading to pregnancy complications and worsening symptoms after delivery [ 19 ].…”

Section: Introductionmentioning

confidence: 99%

Inflammatory myofibroblastic tumor from molecular diagnostics to current treatment

CHMIEL,

SłOWIKOWSKA,

BANASZEK

et al. 2024

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Inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with intermediate malignancy characterized by a propensity for recurrence but a low metastatic rate. Diagnostic challenges arise from the diverse pathological presentation, variable symptomatology, and lack of different imaging features. However, IMT is identified by the fusion of the anaplastic lymphoma kinase (ALK) gene, which is present in approximately 70% of cases, with various fusion partners, including ran-binding protein 2 (RANBP2), which allows confirmation of the diagnosis. While surgery is the preferred approach for localized tumors, the optimal long-term treatment for advanced or metastatic disease is difficult to define. Targeted therapies are crucial for achieving sustained response to treatment within the context of genetic alteration in IMT. Crizotinib, an ALK tyrosine kinase inhibitor (TKI), was officially approved by the US Food and Drug Administration (FDA) in 2020 to treat IMT with ALK rearrangement. However, most patients face resistance and disease progression, requiring consideration of sequential treatments. Combining radiotherapy with targeted therapy appears to be beneficial in this indication. Early promising results have also been achieved with immunotherapy, indicating potential for combined therapy approaches. However, defined recommendations are still lacking. This review analyzes the available research on IMT, including genetic disorders and their impact on the course of the disease, data on the latest targeted therapy regimens and the possibility of developing immunotherapy in this indication, as well as summarizing general knowledge about prognostic and predictive factors, also in terms of resistance to systemic therapy.

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