Publication:

How rare is too rare to suspect? A case of a well-differentiated liposarcoma of the piriform recess of the larynx

Liposarcomas are among the rarest soft tissue sarcomas in the larynx, accounting for less than 1% of cases with the piriform recess of the larynx being one of the least common sites. These tumors present significant diagnostic delays due to their comparison to benign lipoma lesions. The aim is to emphasize the diagnostic utility of molecular markers such as murine double minute 2 (MDM2) and cyclin-dependent kinase 4 (CDK4), and the importance of maintaining long-term surveillance in patients with atypical adipocytic tumors in the head and neck region. A 56-year-old female with a prior history of hypopharyngeal lipoma presented with progressive dysphagia, hoarseness, and inspiratory stridor. Flexible endoscopic examination revealed a well-circumscribed mass originating from the right piriform recess. Magnetic resonance imaging (MRI) was performed. Initial transoral excision confirmed well-differentiated liposarcoma (WDL) via histopathological analysis and positive MDM2 and CDK4 immunochemistry, further supported by FISH. Following multidisciplinary evaluation in sarcoma tumor board, a second, wide-field excision with clear margins was performed using CO2 transoral laser microsurgery (TOLMS). Postoperative recovery was complicated by dysphagia. At 24-month follow up, the patient remained clinically disease-free with preserved speech and swallowing functions following dedicated rehabilitative therapy. Involvement of the piriform recess, confirmed by MDM2 and CDK4 gene amplification, and the successful management through CO2-TOLMS features both the diagnostic complexity and the therapeutic significance of prompt, molecular guided intervention in this rare clinical context. Complete surgical excision with negative margins remains the therapeutic mainstay. Long-term clinical surveillance is essential due to the risk of recurrence and the anatomic complexity of the laryngeal region.