A 7-year-old male child presented with recurrent episodes of cough and breathing difficulty, and occasional wheeze. The child had been treated as asthma for more than a year, without clinical improvement, and was subsequently, the child was referred to us.

The detailed clinical evaluation revealed that his symptoms were progressive in the last 6 months, with no history of hemoptysis, and no family history of asthma or atopy. His vital signs and laboratory investigations were within normal limits. Chest radiography showed bilateral hyperinflation. Multidetector computed tomography (MDCT) showed a mass lesion with calcification, at the anterolateral aspect of the trachea.

Flexible bronchoscopic examination showed a mass lesion attached to the anterior wall of lower trachea (Fig. 1). The lesion was a smooth-surfaced, sessile about 0.7 cm x 0.5 cm in size causing near total obstruction of trachea. Biopsy was done by rigid bronchoscopy which was suggestive of inflammatory fibromyoma of the trachea.

Fig. 1 Bronchoscopy view and HPE of inflammatory myofibroblastic tumor and photomicrograph showing a lesion composed of elongated spindle cells (arrow) admixed with inflammatory cell population (arrow head) in the background (H&E x 400).

Under total intravenous anesthesia using ventilating bronchoscope, diode laser excision of the mass was done. Histopathological examination of the specimen showed squamous lining epithelium and spindle cells admixed with inflammatory cell population. Immunohistochemical findings were positive for Vimentin, anaplastic lymphoma kinase (ALK) and Desmin which were consistent with inflammatory myofibroblastic tumor (Fig. 1).

Tracheal tumors are easily treatable when diagnosed in the early stages. Due to the low incidence and the variability of clinical presentation, the diagnosis and treatment of tracheal IMT are often delayed. If a child does not respond to asthma management, alternatives like endobronchial mass lesions need to be considered.

Although IMT have been described in virtually every anatomic location, there are only few documented reports with tracheal localization [4]. The index case presented with slowly progressive symptoms mimicking asthma but other studies have reported acute presentation with severe respiratory distress mimicking as foreign body [5].

Based on the location in the pulmonary system, IMTs are divided into two groups as parenchymal and airway IMTs. Most IMTs manifest as a solitary lesions in the lung parenchyma, and only 10% of these develop within the airway. The analysis of bronchoscopically treated IMT of the tracheobronchial tree in the age group of 16-45 years revealed that roughly half of them (43%) were confined to the trachea [6].

The index case started his symptoms from 6 years of age. Though IMT are reported in adults, these are the most common benign lung tumor in children under 16 years of age [7]. Though initial CT reported as papilloma, which is a common tracheal tumor, excision biopsy confirmed as IMF. Endobronchial excision was made possible by rigid bronchoscopy diode laser. Transportability, cost, penetration depth (0.3-1.0 mm) and effective photocoagulation of the diode laser are the advantageous features. In endobronchial surgery, the advantages of various lasers have repeatedly been debated but in our experience diode laser appears to be more precise and less invasive [8]. The immuno-histochemistry of the specimen was typically reactive to vimentin (99% specific), desmin (69% specific) with ALK-1 expression (highly specific), which were all consistent with IMT (Fig. 1) [9].

As IMF is considered to have low-grade malignant potential, bronchoscopic excision of the tumor with the diode laser appears to have advantages in resource-poor settings [10]. The appropriate surgical intervention will ensure a normal life if such children are referred early.

Contributions: DVS: conception and design of the study; TNS: surgical management and final version; SR: anaesthetic management.

Funding: None; Competing Interest: None stated.

1. Özgül MA, Toru Ü, Acat M, Özgül G, Çetinkaya E, Dinçer HE, et al. A rare tumor of trachea: Inflammatory myofibroblastic tumor diagnosis and endoscopic treatment. Respir Med Case Rep. 2014;13:57-60.

2. Aihole JS. LokanathH, Munianjinappa NB. Primary pleural inflammatory pseudotumor in a Child. Indian Pediatr. 2018;55:341-2.

3. Liu L, Kong X, Lu X, Cao D. Pediatric endobronchial inflammatory myofibroblastic tumor: A case report and review of the literature.　Clin Pract. 2016;6:853.

4. Venizelos I, Papathomas T, Anagnostou E, Tsanakas J, Kirvassilis F, Kontzoglou G. Pediatric Inflammatory myofibroblastic tumor of the trachea: A case report and review of the literature. Pediatr Pulmonol. 2008;43:831-5.

5. Ray A, Suri JC, Bhattacharya D, Gupta A. Bronchoscopy resection of endobronchial inflammatory myofibroblastic tumor: A case report and systematic review of the literature. Lung India. 2014;31:172-5.

6. Sivanandan S, Lodha R, Agarwala S, Sharma, Kabra SK. Inflammatory myofibroblastic tumor of the trachea. Pediatr Pulmonol. 2007; 42:847-50.

7. Oztuna F1, Pehlivanlar M, Abul Y, Tekinbas C, Ozoran Y, Ozlu T. Adult inflammatory myofibroblastic tumor of the trachea: Case report and literature review. Respir Care. 2013;58:e72-6.

8. Arroyo HH, Neri L, Fussuma CY, Imamura R. Diode Laser for Laryngeal Surgery: A Systematic Review. Int Arch Otolaryngol. 2016;20:172-9.

9. Palaskar S, Koshti S, Maralingannavar M, Bartake A. Inflammatory myofibroblastic tumor. Contemp Clin Dent. 2011;2:274-77.