Tracheal inflammatory myofibroblastic tumor in a 3-year-old boy

Author:

Vilotijevic-Dautovic Gordana1,Jovic Rajko2ORCID,Vuckovic Nada3ORCID,Bjelica Milena1,Plazacic Milica4

Affiliation:

1. University of Novi Sad, Faculty of Medicine Novi Sad + Institute for Child and Youth Health Care of Vojvodina, Department of Pediatrics, Novi Sad

2. University of Novi Sad, Faculty of Medicine Novi Sad + Institute for Child and Youth Health Care of Vojvodina, Clinic for Otorhinolaryngology and Head and Neck Surgery, Novi Sad

3. University of Novi Sad, Faculty of Medicine Novi Sad + Center for Pathology and Histology, Novi Sad

4. Institute for Child and Youth Health Care of Vojvodina, Department of Pediatrics, Novi Sad

Abstract

Introduction. Inflammatory myofibroblastic tumors predominantly manifest in the lungs of children and young adults, with tracheal localization being very rare. Genetic alternations involving the anaplastic lymphoma kinase gene are identified in 50 to 70% of cases. A conclusive diagnosis relies on biopsy and histopathological analysis. Surgical resection stands as the primary treatment modality. Case Report. We present a case involving a 3-year-old boy with an inflammatory myofibroblastic tumor who had experienced recurrent wheezing attributed to respiratory infections since the age of eight months. Long-term therapy with budesonide and montelukast was initiated, which effectively managed his wheezing until the age of 3 years. Subsequently, despite ongoing medication, he began experiencing severe bronchial obstructions every month, necessitating repeated hospital admissions. At the age of 3 years and 8 months, he was admitted to our hospital due to persistent wheezing, prompting a bronchoscopy. During the procedure, a tumor-like mass was identified in the lower part of the trachea. Bronchoscopic removal of the tumor was performed, followed by cauterization of the remaining tumor tissue. Histopathological examination confirmed the presence of an inflammatory myofibroblastic tumor. Conclusion. Inflammatory myofibroblastic tumors are uncommon neoplasms associated with a borderline risk of malignancy. Given that the symptoms can resemble those of common childhood respiratory conditions, it is crucial to consider this diagnosis in cases of persisting wheezing despite standard therapy. In such instances, performing a bronchoscopy is necessary for accurate diagnosis.

Publisher

National Library of Serbia

Reference19 articles.

1. Casanova M, Brennan B, Alaggio R, Kelsey A, Orbach D, van Noesel MM, et al. Inflammatory myofibroblastic tumor: the experience of the European pediatric Soft Tissue Sarcoma Study Group (EpSSG). Eur J Cancer. 2020;127:123-9.

2. Da M, Qian B, Mo X, Xu C, Wu H, Jiang B, et al. Inflammatory myofibroblastic tumors in children: a clinical retrospective study on 19 cases. Front Pediatr. 2021;9:543078.

3. Goldblum JR, Folpe AL, Weiss SW. Enzinger and Weiss’s soft tissue tumors. 7th ed. Philadelphia: Elsevier; 2020. Chapter 9, Borderline and malignant fibroblastic/myofibroblastic tumors; p. 322-30.

4. Wu RI. Inflammatory myofibroblastic tumor [Internet]. [updated 2016 May 1

5. cited 2022 Jul 19]. Available from: https:// www.pathologyoutlines.com/topic/lungtumorinflammatorypseudotumor.html

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