Intrathoracic Inflammatory Myofibroblastic Tumor in a Nigerian Child: A Rare Case Presentation and Review of Literature
Pauline Kasarachi Akowundu
*
Department of Paediatrics, Redeemers Health Village (RHV), Redemption City, Mowe, Ogun State, Nigeria.
Blessing Ebele Kene-Udemezue
Department of Paediatrics, Orile-Agege General Hospital, Lagos, Nigeria.
*Author to whom correspondence should be addressed.
Abstract
Background: An inflammatory myofibroblastic tumor (IMT) is a rare, predominantly myofibroblastic neoplasm accompanied by inflammatory cells, including lymphocytes and eosinophils. It is predominantly found in children and adolescents. Extra-pulmonary sites for IMTs include the head and neck region. IMT of the thorax is rare, accounting for 0.04%-0.1% of all pulmonary tumors. Because the clinical presentation of IMT is nonspecific and rare, it poses a major diagnostic challenge. Hence, the need for IMT to be differentiated from other chronic infections and malignant lesions based on histopathologic findings and immunohistochemical analysis. The mainstay of care for localized tumors is a surgical resection. Spontaneous regression has also been reported in some people. A response rate of approximately 50% has been reported with the use of chemotherapy.
Case Presentation: An 8-year-old male with complaints of generalized body swelling, which progressed to weakness of both lower limbs and later inability to walk, with associated urinary and fecal incontinence of about 8 months duration. However, 5 weeks before presentation, developed cough, difficulty with breathing, progressive weight loss, and drenching night sweats. An initial diagnosis of disseminated tuberculosis was made, but this was changed following a histology report of an inflammatory myofibroblastic tumor (IMT). Further treatment was not done as the child passed on.
Conclusion: Inflammatory Myofibroblastic Tumor is a rare tumor that is easily misdiagnosed. This case report contributes significant clinical insight into the presentation and diagnosis of a rare tumor in a low-resource tertiary healthcare setting. Its importance lies in highlighting the non-specific features at presentation and possible treatment approaches in the context of limited diagnostic infrastructure. Additionally, the case provides valuable insights into the management of IMT, improving knowledge of clinicians practicing in similar environments.
Keywords: Inflammatory myofibroblastic tumor (IMT), anaplastic lymphoma receptor tyrosine kinase (ALK) gene, inflammation