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Case Report: Inflammatory Myofibroblastic Tumor in a 6 Year-old Boy

 Introduction: Inflammatory myofibroblastic tumor (IMT) is a rare mesenchymal neoplasm characterized by spindle-shaped myofibroblastic cells accompanied by an inflammatory infiltrate. Although IMTs can occur at any age, they are more frequently diagnosed in children and young adults. This report presents a case of a 5-year-old boy from Bangladesh diagnosed with an IMT.

Case Presentation: A 5-year-old boy from Bangladesh presented with difficulty walking and persistent pain in his right thigh for six months. In 2017, he underwent an excisional biopsy, and the lesion was initially diagnosed as fibrosarcoma. However, no treatment was initiated, and there was no consultation with a pediatric oncologist following the diagnosis.







Clinical Progression: After 2.5 years, in 2019, the patient developed a new mass in the same area. MRI revealed an altered signal intensity lesion in the anterolateral aspect of the right upper thigh, suggestive of possible sarcoma. Surgical intervention was performed, and biopsy results initially suggested inflammatory fibrosarcoma. Immunohistological analysis confirmed the diagnosis of an inflammatory myofibroblastic tumor (IMT).

Histopathological Findings: Immunohistochemistry revealed positivity for vimentin and smooth muscle actin (SMA), while ALK1, pancytokeratin (AE1/AE3), epithelial membrane antigen (EMA), desmin, myogenin, S-100, and CD34 were all negative. Additionally, p53 was positive in some tumor cells, and the proliferation index marker Ki-67 was positive in 1–2% of tumor cells.

Management: The patient underwent complete surgical excision of the tumor. No chemotherapy was administered, and the patient was placed under regular follow-up care.

Follow-Up: The patient has been attending regular follow-ups and, as of 2025, has not experienced any recurrence of the tumor. Clinical and radiological evaluations during follow-up visits have shown no signs of disease progression.

Discussion: IMT is a rare tumor with a variable clinical course and can be easily misdiagnosed due to its similarity with malignant soft tissue sarcomas. This case underscores the importance of immunohistochemistry in achieving an accurate diagnosis. The absence of ALK1 expression, alongside other markers, and the low Ki-67 index suggest a lower proliferative potential. Regular follow-up is critical due to the risk of recurrence.

Conclusion: This case highlights the need for precise diagnostic evaluation of soft tissue masses in children. Early surgical intervention and vigilant long-term monitoring are essential to ensure favorable patient outcomes.

References: [To be added based on relevant literature]

Acknowledgments: The authors extend their gratitude to the patient and his family for their cooperation and consent for the publication of this case report.

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