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What is IM?
Infantile
Myofibromatosis (IM)
Infantile Myofibromatosis is a rare condition in which benign (non-cancerous) tumors, called myofibromas, grow in soft tissues such as the skin, muscle, bone, or - in some cases, internal organs.
Understanding IM
Infantile Myofibromatosis causes benign
(non-cancerous) tumors, called myofibromas, to form in the body.
Myofibromas (tumors) are made of fibrous tissue - these tumors are not cancer, and many grow, shrink or stabilize over time. The way IM affects each child can vary wildly - from a single lump under the skin to multiple tumors throughout the body.
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Because IM is so rare, often doctors may never encounter it in their careers — which is why many families often feel isolated and uncertain after diagnosis.
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At IM Strong, our goal is to provide the critical support that families need through providing clear, trustworthy information in one place.
Types of IM
​There are three forms of Myofibromas - Solitary, Multi-centric, and Generalized
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Solitary - This is when there is only one tumor, usually in the skin, muscle, or bone.​
Multi-centric - IM is multi-centric when multiple tumors are found in different parts of the body,but usually not involving internal organs.​
Generalized/Visceral - When tumors affect internal organs (like the lungs, liver, or intestines) this is diagnosed as the visceral form of IM. This form can be more serious and may require more aggressive forms of treatment.
What Causes IM?
IM is often linked to genetic mutations in the PDGFRB or NOTCH3 genes.
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These mutations are inherited in an autosomal dominant pattern, meaning a parent carrying the gene has a 50% chance of passing it on to their child.​ While research is still ongoing, these genetic discoveries are helping doctors understand better treatments.
Diagnosing IM
Diagnostic Tools
Because Infantile Myofibromatosis is so rare, diagnosis can sometimes take time and may involve several specialists. IM can appear in different ways depending on where the tumors form - from small skin nodules, to deeper growths in muscle, bone or internal organs.
​Typically, Doctors will begin by gathering a detailed medical history and performing a physical exam to identify any visible or palpable lumps.
From there, imaging and tissue testing are often used top confirm the diagnosis. Below is a list of some of the methods specialists may use to diagnose IM;
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• Physical exams (visible or palpable tumors)
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• Imaging (like ultrasounds, MRIs, or CT scans)
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• Biopsy (to confirm the tumor type under a microscope)
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• Genetic testing (to check for PDGFRB or NOTCH3 mutations)
Treatment Options
How is Infantile Myofibromatosis Treated?
Watch and Wait
Remarkably, many IM cases resolve on their own over time. These cases use a 'Watch & Wait' approach in which a team of doctors and or specialists may monitor the child monthly/yearly to access tumor growth.
Surgery
Sometimes surgery is needed to remove tumors if tumors interfere with function or cause pain.
Targeted Therapy
Immunotherapy medications like Imatinib have shown effectiveness in some cases with PDGFRB mutations.
Chemotherapy
Chemotherapy may be used for cases with severe visceral involvement.