Leiomyosarcoma of the Larynx: A Complex Diagnosis

Abstract

An 84-year-old man was referred to our institution for dysphonia present for 2 months. Fiberoptic laryngoscopy demonstrated a polypoid lesion of the right true vocal fold with normal motility. Excisional biopsy of a submucosal thickening of the right vocal fold with vocal ligament calcification was performed. Histology revealed squamous cell epithelium without malignancy. Postoperative endoscopic follow-up was regular. Six months after surgery, dysphonia and dyspnea occurred. Flexible laryngoscopy demonstrated right hemilaryngeal paralysis, edema, and partial airway obstruction. Necrotic tissue and purulent secretions were visible at the anterior commissure, right vocal fold, and ipsilateral vestibule. A second microlaryngoscopic surgery included multiple biopsies and debridement of the necrotic tissue. Histological report was negative for malignancy, and purulent chondritis was diagnosed. Intravenous antibiotic therapy was administered with improvement of infection, inflammation, dysphonia, and dyspnea. The patient was discharged. Two months later, a new sudden and severe dyspnea occurred. He was admitted to the emergency department, and oropharyngeal intubation was necessary because of respiratory failure. Computed tomography (CT) scan showed thickening of the right laryngeal wall with wide fluid collection (48-43 mm diameter) that almost completely obstructed the airway, with erosion of the right thyroid and cricoid cartilage (Figure 1). Debridement of the necrotic tissue and drainage of the purulent collection was performed with direct laryngoscopy. Culture was positive for Staphylococcus epidermidis and Candida albicans. Intravenous antibiotic, antimicotic, and corticosteroid therapy was administered, and other 3 surgical debridements and drainage were performed, but without clinical or radiological improvements. Moreover, patient developed signs of sepsis. Since no improvement had been achieved, total laryngectomy was planned even without a positive histologic biopsy. Intraoperative findings showed transglottic extension of the ulcerated material with full-thickness infiltration of the laryngeal wall and external spread through the thyroid cartilage, with invasion of prelaryngeal tissues (Figure 2). The patient’s clinical condition improved, and he started oral feeding without pharyngocutaneous fistula. Morphological and immunohistochemical features were consistent with leiomyosarcoma (Fédération Nationale des Centres de Lutte Contre Le Cancer [FNCLCC] grade 3; Figure 3). Multidisciplinary consultation suggested adjuvant radiation therapy, but preirradiation staging CT scan showed rapid local recurrence and lung metastasis. The poor clinical condition of the patient contraindicated first-line chemotherapy. Figure 1. Computed tomography image showing an almost complete erosion of the laryngeal framework cartilages due to LLMS. Residual airway is minimal therefore an emergency oropharyngeal intubation is needed.