Inflammatory myofibroblastic tumors: treatments and research
Because of the low index of metastasis in inflammatory myofibroblastic tumours (IMTs), surgery is normally the preferred and best option. The surgical resection of the tumour can be definite and curative, but it is vital to remove all of the tumour cells as it has a very high probability of recurrence. This means that during surgery, the surgeon must send a biopsy of the surgical site and this biopsy will confirm or discard whether there are tumour cells left in the margins of resection.
If surgery is not possible because the tumour has already spread to other tissues or the site of the tumour is not accessible or too risky, other treatments like chemotherapy or targeted therapy may be an option for treatment, but surgery is practically always the best option. And it can be curative! If tumour cells are left out during the intervention, chemotherapy can also be adequate to complete the treatment. When surgery is performed prognosis tends to be very good but it depends greatly on the location and size of the tumour.
It is significant to mention that 50% of IMTs have been described to be positive for the enzyme anaplastic lymphoma kinase (ALK). ALK is involved in the growth, proliferation and differentiation of cells. The expression and activation of this gene involves the rearrangement of the ALK gene, which will lead to the synthesis of the ALK protein, and this will trigger an 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 been described too, but appear a lot less than ALK. In any case, it is important to acknowledge the role of these genes, especially as therapeutic targets in the near future.
There is still a lot of work to do to fully understand the nature and pathogenesis of inflammatory myofibroblastic tumours. Although they are usually regarded as benign tumours, there are cases in which metastasis can occur. Moreover, as IMTs proliferate rapidly, they may put in risk the integrity of many body organs, veins, arteries and nerves. Share4Rare is willing to create a global community of patients with IMTs in order to collect medical information and increase research in this area. Join our community and help us find effective treatments for IMTs!