Inflammatory Myofibroblastic Tumor of the Larynx

Abstract

Objectives:

1) Describe a case of inflammatory myofibroblastic tumor (IMFT) of the larynx. 2) Analyze the literature regarding the disease process. 3) Discuss appropriate management options and prognosis.

Methods:

Retrospective review of a single case of IMFT involving the subglottic region at the University of Texas Medical Branch.

Results:

Here we present IMFT of the larynx, a benign condition often believed to be a malignancy due to its aggressive proliferative nature. The majority of extra-laryngeal cases are pulmonary; however, only 25 cases in the larynx have been described. Involvement of the subglottis is even rarer, representing only 5 cases. The origin is still unknown; however, many believe IMFT to be of clonal origin with chromosomal rearrangements of 2p23 and the ALK1 gene. These abnormalities are found in up to 47% of cases either immunohistochemically or using fluorescence in situ hybridization. Microsuspension laryngoscopy with laser excision has been the mainstay of treatment, and long term results have been excellent. Our patient’s tumor was ALK1 positive on immunohistochemistry, confirming the diagnosis. As observed in previous cases, our patient has responded well to conservative resection with no recurrence at this time.

Conclusions:

IMFT is an aggressive tumor of the larynx. However, conservative excision is adequate for tumor control and good long term survival. Knowledge of the disease is important to prevent overtreatment.