Laryngeal Myofibroblastic Tumor: A New Therapeutic Approach. A Case Report

Abstract

Myofibroblastic tumors are rare lesions which can affect any part of the body. Although benign, their mass effect causes symptoms that can become life-threatening. Supraglottic laryngeal involvement is extremely rare, with only 4 cases described in the English literature. Because the pathophysiology is unknown and the incidence is so low, there is no standardized therapeutic management, although for laryngeal tumors surgery has traditionally been the preferred initial option. Another less common option is intravenous and oral corticosteroid therapy, but this is usually reserved for myofibroblastic tumors in other head and neck sites that are more difficult to access surgically, or patients who cannot undergo surgery. These lesions have a very high tendency to recur, and morbidity rates are therefore also high. We present a case of supraglottic myofibroblastic tumor in which we elected high-dose corticosteroid therapy as the only form of treatment. With this new therapeutic approach, we avoided the undesirable effects of the usual type of surgery. At the 12-month follow-up, the patient is in complete remission.

Larynx myofibroblastic tumor, a rare case report

“…8,12,15,16,19,20 F I G U R E 4 Fibrolaryngoscopic examination 6 months after surgery, with an area of nodular fibrotic appearance suspicious for recurrence in the area of surgical resection Given the marked inflammatory component of the lesion, a second-line corticosteroid therapy is widely validated in cases with contraindications for surgery or tumor recurrence, and it is also used for head and neck IMT in locations other than the larynx. 1 Genetic alterations are opening new lines of research: series of treatment with ALK inhibitor crizotinib have been published in cases of ALK-positive ITM, as a secondline therapy for those cases of contraindication to surgery, partial resection, or positive surgical margins. 12,21…”

“…[6][7][8][9] In the larynx, they mainly affect the vocal cords, with cases described at the supraglottic and subglottic levels. 1,10 The pathogenesis of this entity is related to reactive processes, infections, autoimmune diseases, laryngeal trauma, and other neoplasms, with no clear etiology described. 8,11 It affects mostly children and young adults.…”

“…Myofibroblastic tumors of the larynx are a very rare finding with approximately 40 cases published in the scientific literature 1 . They are benign neoplasms with a marked inflammatory character that affect soft tissues and can become locally invasive, malignant, or metastatic.…”

“…They are less frequent in head and neck areas, with described involvement of parapharyngeal spaces, oropharyngeal, nasopharyngeal, pterygopalatine fossa, infratemporal fossa, paranasal sinuses, orbit, and oral cavity 6‐9 . In the larynx, they mainly affect the vocal cords, with cases described at the supraglottic and subglottic levels 1,10 …”

“…Myofibroblastic tumors of the larynx are a very rare finding with approximately 40 cases published in the scientific literature. 1 They are benign neoplasms with a marked inflammatory character that affect soft tissues and can become locally invasive, malignant, or metastatic. In most cases, they are asymptomatic space‐occupying lesions, which require differential diagnosis with other laryngeal neoplasms.…”