Inflammatory myofibroblastic tumours: treatments and research
Due to the low metastasis rate observed in inflammatory myofibroblastic tumours (IMTs), surgery typically stands out as the preferred and most effective option. Surgical resection of the tumour is often definitive and curative; however, it is crucial to ensure the complete removal of all tumour cells due to the high likelihood of recurrence. Consequently, during the surgical procedure, the surgeon must submit a biopsy of the resected site for analysis. This biopsy serves to confirm or rule out the presence of any residual tumour cells in the resection margins.
When surgery is not feasible, either due to the tumour's dissemination to other tissues or to the inaccessibility or high risk associated with the tumour site, alternative treatments such as chemotherapy or targeted therapy may become viable options. Nevertheless, surgery is generally considered the optimal choice and can potentially be curative. If tumour cells persist after surgery, chemotherapy can serve as a suitable adjunct to complete the treatment. The prognosis following surgery tends to be highly favourable, although it ultimately depends on factors such as the tumour's location and size.
It is noteworthy to mention that 50% of inflammatory myofibroblastic tumours (IMTs) have been reported to test positive for the enzyme anaplastic lymphoma kinase (ALK). ALK plays a crucial role in the growth, proliferation, and differentiation of cells. The expression and activation of this gene entail the rearrangement of the ALK gene, resulting in the synthesis of the ALK protein. This, in turn, triggers uncontrolled growth and proliferation of these cells.
On the other hand, though, 50% of IMTs are negative for ALK. These are described as more aggressive tumours with a higher potential for metastasis. Other gene rearrangements such as ROS1, ETV6 and NTRK3 have also been described, but are less frequent than ALK. In any case, it is key to characterise the role of these genes, especially when developing future therapeutic targets.
Closing thoughts
There is still work to do to fully understand the nature and pathogenesis of inflammatory myofibroblastic tumours. Although they are usually regarded as benign tumours, in some cases, metastasis can occur. Moreover, as IMTs grow fast, they may threaten the integrity of many body organs, veins, arteries and nerves.
Share4Rare is willing to create a global community of patients with IMTs to collect medical information and increase research in this area. Join our community and help us find effective treatments for IMTs!
