Extraosseous osteosarcoma of the larynx: an unusual tumor managed with radical surgery - case report

Introduction and importance: Extraosseous osteosarcoma (EOO) is a malignant tumor that arises in soft tissue without affecting bone or periosteum but produces osteoid, osseous, or chondroid material. It is a very rare malignancy that accounts for approximately less than 1% of all tissue sarcomas and similar to 2-4% of all osteosarcomas. EOO in the head and neck region is extremely rare; however, in the larynx, it is exceptional, with only one case reported worldwide in the literature to date. Case presentation: A 72-year-old male with no personal history of interest in the current condition presented with dysphonia, hoarseness, and dyspnea for 6 months. A flexible nasofibrolaryngoscopy (FNFL) and computed tomography (CT) scan exhibited a glottic tumor with a lumen obstruction of 90%. An endoscopic resection was performed, reporting an EOO, osteoblastic, and ulcerated type. During follow-up, tumoral persistence was detected, leading to the decision to perform a definitive radical treatment with a total laryngectomy. Clinical discussion: Its presentation in the head and neck region is exceptional, with only a few isolated cases reported in the literature. Available data on treatment recommendations have emphasized the importance of timely surgical intervention with adjuvant radiation therapy if necessary for the head and neck. Conclusion: Extraosseous osteoblastoma of the larynx is an extremely rare and infrequent disease. Correct diagnosis and oncological surgical management of this disease are essential for optimal and even curative treatment of the patient.