Inflammatory Myofibroblastoma in Children and Adolescents: Diagnosis, Treatment, and What Families Need to Know
Inflammatory myofibroblastoma (IMT) is a rare yet impactful tumor that primarily affects children and young adults—but many families and even some healthcare providers know little about it. While most cases are benign, IMT’s ability to invade organs or occasionally turn malignant makes it a complex condition to manage. A 2019 study from Zhengzhou University offers critical insights into how to diagnose and treat IMT in kids, giving hope to families navigating this rare disease.
What Is IMT?
IMT is classified as an “intermediate” tumor—meaning it sits between benign (non-cancerous) and malignant (cancerous). It can grow in almost any organ, but the most common sites are the lungs, abdomen/pelvis, or retroperitoneum (the space behind the abdominal organs). Doctors once thought IMT arose from simple inflammation, but research shows some cases can behave aggressively—invading nearby tissues or, rarely, spreading to distant parts of the body. There are no universal treatment guidelines for IMT, which adds to the challenge of care.
The Study: 16 Kids, 5 Years of Data
The research, led by Yi Ding, He-Ying Yang, and colleagues from the Department of Pediatric Surgery at The First Affiliated Hospital of Zhengzhou University, analyzed 16 patients aged 0.9 to 18 years diagnosed with IMT between 2013 and 2018. The team tracked symptoms, diagnostic tests, treatments, and outcomes to identify patterns in care.
Symptoms and Diagnostics
Symptoms varied based on where the tumor was located:
- Abdominal/pelvic IMT: Bloating, pain, blood in stool, yellowed skin (jaundice), or digestive issues.
- Lung IMT: Cough, mucus production, shortness of breath, or chest pain.
- Bladder IMT: Blood in urine.
- Neck/facial IMT: Swelling or a palpable lump.
Seven kids also had “systemic” symptoms—fever, loss of appetite, or weight loss—signaling the body was fighting an abnormal process.
Lab tests revealed:
- 38% had elevated white blood cells (a sign of inflammation).
- 69% had hypochromic microcytic anemia (a type of anemia where red blood cells are small and pale, often from chronic disease).
- 75% had non-specific tumor markers (proteins that suggest a tumor but don’t confirm IMT).
Imaging (like CT scans) was key to diagnosing IMT, showing clear borders in some cases or invasion of critical structures (e.g., mediastinal blood vessels, liver hila) in others.
Treatment: Surgery First, Adjunct Therapies When Needed
Surgery is the gold standard for IMT—and for good reason. If doctors can remove the entire tumor with “clear margins” (no leftover cancer cells under a microscope), the cure rate is high.
Complete Resection (Full Tumor Removal)
Eleven kids had tumors with clear borders that were fully removed using procedures like:
- Lung lobectomy (removing a section of the lung).
- Excision of mediastinal tumors with partial rib removal.
- Removing tumors along with adjacent bowel or bladder tissue.
- Neck mass resection.
Nine of these kids survived without recurrence, one was lost to follow-up, and one died from a post-operative chest infection.
Palliative Resection (Partial Tumor Removal)
For tumors that invaded vital organs or structures (e.g., a lung IMT squeezing mediastinal blood vessels or a liver IMT in all three hepatic hila), doctors removed as much of the tumor as possible. Examples include:
- A left lung IMT patient who got chemotherapy after palliative surgery and is now tumor-free.
- An omental IMT patient who had a recurrence but was cured with surgery plus chemotherapy.
- A liver IMT patient who underwent an autologous liver transplant (using their own liver tissue) and succeeded.
Adjunct Therapies
When complete surgery wasn’t possible, the team used:
- Chemotherapy: Drugs like pirarubicin (AVCP regimen) or etoposide (IEV regimen) shrank residual tumors in some cases.
- Targeted Therapy: Two kids with ALK-positive IMT (a genetic mutation) received crizotinib, a drug that targets ALK proteins. More research is needed to confirm its effectiveness.
The team strongly advised against waiting for “spontaneous regression” (the tumor disappearing on its own)—active treatment is always better.
Outcomes: Survival, Recurrence, and What Matters Most
Most pediatric IMT cases are benign, with an overall survival rate of over 90%. Recurrence rates depend on where the tumor is:
- Less than 2% for lung tumors.
- Up to 25% for extrapulmonary tumors (e.g., abdomen, neck).
Most recurrences happen within 12 months of surgery. The single most important factor in preventing recurrence? Complete surgery with clear margins.
Of the 13 kids the team followed:
- 12 were in “complete remission” (no signs of tumor) at their last check-up.
- 1 survived with “partial remission” (some tumor remaining).
- 2 were lost to follow-up (one after complete resection, one who refused treatment).
Follow-Up: Catching Recurrence Early
Because IMT can occasionally turn malignant, the team recommends:
- CT or MRI scans at 3, 6, and 12 months after surgery.
- Annual color Doppler ultrasound or CT/MRI scans thereafter.
This schedule helps catch recurrence or metastasis (rare, but possible) early when treatment is most effective.
The Bottom Line
Pediatric IMT is a rare but manageable condition—especially with early diagnosis and aggressive treatment. Surgery is the first line of care, and complete resection with clear margins is the best way to prevent recurrence. For tumors that can’t be fully removed, palliative surgery plus chemotherapy or targeted therapy can work. Most kids with IMT go on to live healthy lives—with the right care.
This article is based on research published in Chinese Medical Journal (2019) by Yi Ding, He-Ying Yang, Da Zhang, Fei Guo, Jia-Xiang Wang, Ya-Ping Li, and Yan-An Li from the Department of Pediatric Surgery at The First Affiliated Hospital of Zhengzhou University, Zhengzhou, Henan 450052, China.
doi.org/10.1097/CM9.0000000000000176
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