Molecularly guided management of pediatric endobronchial inflammatory myofibroblastic tumor: a report of two cases on surgical and targeted intervention.

BACKGROUND: Pediatric inflammatory myofibroblastic tumors (IMTs) of the airway are rare mesenchymal neoplasms of intermediate biological potential characterized by clonal proliferation and diverse clinical presentations. Owing to their nonspecific symptoms, such as persistent cough and stridor, these tumors are frequently misdiagnosed as more common pediatric conditions, including refractory asthma or foreign-body aspiration, often leading to delays in definitive treatment.

CASE DESCRIPTION: We described 2 pediatric cases of endobronchial IMT managed according to anatomical location and molecular features. Case 1 was an 8-year-old boy with an anaplastic lymphoma kinase (ALK)-negative tumor located at the opening of the posterior segment of the right lower lobe bronchus, who underwent robot-assisted sleeve resection with preservation of functional lung parenchyma. Case 2 was a 6-year-old boy with an ALK-positive carinal tumor causing critical airway narrowing, who underwent primary endoscopic resection followed by adjuvant crizotinib therapy based on multidisciplinary assessment of molecular status and recurrence risk. No evidence of recurrence was observed at 6-month follow-up in Case 1 or at 3-month follow-up in Case 2.

CONCLUSIONS: These two cases suggest that integrating molecular diagnostics, particularly ALK status, with a stepwise multidisciplinary approach may help guide individualized management in pediatric airway IMT. However, the short follow-up duration of 6 months and 3 months limits conclusions regarding long-term recurrence and durability of treatment outcomes.