Tracheobronchial inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with a pediatric prevalence. Due to its early symptoms and limited size at diagnosis, the treatment of choice is generally surgical with eventual airway reconstruction. We present the case of an 18-year-old patient with ALK-positive IMT involving left lung hilum and infiltrating the left upper lobe. The clinical, radiological, and histopathological findings are described, highlighting the diagnostic challenges of cytological samples. After surgical evaluation, to minimize the extent of resection, given the patient’s young age, an approach with alectinib was attempted with a rapid favorable response and no significant side effects. Upon achieving maximum response, a subsequent segmentectomy of the left upper lobe was performed, ensuring complete excision of the lesion. This case underlines the significance of accurate diagnosis and the effectiveness of preoperative therapy with alectinib in ALK-positive IMT for parenchyma-sparing surgery.

Downstaging of ALK Rearranged Inflammatory Myofibroblastic Tumor of the Lung Using Alectinib Before Surgery: Case Report

Iose' Di Stefano;Iacopo Petrini;Elisabetta Macerola;Olivia Fanucchi;Greta Ali'
2024-01-01

Abstract

Tracheobronchial inflammatory myofibroblastic tumor (IMT) is a rare neoplasm with a pediatric prevalence. Due to its early symptoms and limited size at diagnosis, the treatment of choice is generally surgical with eventual airway reconstruction. We present the case of an 18-year-old patient with ALK-positive IMT involving left lung hilum and infiltrating the left upper lobe. The clinical, radiological, and histopathological findings are described, highlighting the diagnostic challenges of cytological samples. After surgical evaluation, to minimize the extent of resection, given the patient’s young age, an approach with alectinib was attempted with a rapid favorable response and no significant side effects. Upon achieving maximum response, a subsequent segmentectomy of the left upper lobe was performed, ensuring complete excision of the lesion. This case underlines the significance of accurate diagnosis and the effectiveness of preoperative therapy with alectinib in ALK-positive IMT for parenchyma-sparing surgery.
2024
Di Stefano, Iose'; Petrini, Iacopo; Pezzuto, Federica; Macerola, Elisabetta; Fanucchi, Olivia; Cannone, Giorgio; Calabrese, Fiorella; Ali', Greta...espandi
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Utilizza questo identificativo per citare o creare un link a questo documento: https://hdl.handle.net/11568/1259247
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