Successful role of adjuvant radiotherapy in a rare case of tracheal inflammatory myofibroblastic tumor: a case report

Abstract

Background: Inflammatory myofibroblastic tumor (IMT) is a rare benign cancer that can express a more aggressive phenotype related to the genetic mutation of the anaplastic lymphoma kinase receptor (ALK). Involvement of trachea is extremely rare and due to the clinical and radiologic nonspecificity, the definitive diagnosis is based on the histologic evaluation of tissue specimens. Total surgical excision is curative and chemotherapy or radiotherapy has been employed in the treatment of unresectable tumors or as adjuvant therapies. Case presentation: The case described here is being reported because of the rare tracheal location and the atypical treatment approach used for an ALK-positive IMT. A 7-week pregnant woman voluntary interrupted pregnancy and underwent total surgical excision that resulted to have close margins. Although ALK-positive expression indicated the use of ALK inhibitors, she refused any type of adjuvant therapy that could affect ovarian function. Thus, 3D conformational external beam radiotherapy was performed with a daily dose of 180 cGy, 5 times per week, up to 45 Gy at the level of trachea. A total of 62 months of follow-up showed and no signs of disease recurrence or late radiation therapy-related toxicity. Conclusions: This report describes an extremely rare case of a tracheal IMT, underlying the key role of radiotherapy as adjuvant treatment able to definitively cure IMT, limiting systemic chemotherapy-related toxicity.

Limited airway resection and reconstruction for paediatric tracheobronchial inflammatory myofibroblastic tumour

“…Chemotherapy has more side effects than targeted therapy and is uncommonly used in IMT patients [ 23 ]; however, chemotherapy can be used as a treatment option for patients regardless of their gene condition. Local radiotherapy, which has few systematic side effects, can be considered when patients are unwilling to receive surgery or systemic treatment [ 24 ]. Since no official management or guidelines are available, participants in our study did not receive postoperative adjuvant therapy.…”

A clinical signature predicting the malignant transformation of inflammatory myofibroblastic tumor in the head and neck

“…Although surgery remains the main option for IMT treatment, 1 there are sporadic case reports about successful treatment by radiotherapy in IMT located in the maxillary sinus 24 and trachea. 25 However, the effectiveness of radiotherapy for IMT treatment has not been verified through randomized controlled trials. Our retrospective study strongly recommends that treatment of HNIMT with a high risk of malignant transformation should include postoperative radiotherapy.…”

Successful Treatment of Inflammatory Myofibroblastic Tumor with Surgical Residue by Radiochemotherapy: Case Report and Review of the Literature

“…Radiotherapy also showed effectiveness in IMT [4,[17][18][19]. It may be a choice for localized IMT patients who cannot tolerate surgery or could be used as postoperative adjuvant therapy in unresectable, progressive, or metastatic cases.…”