Giant Cervico-Mediastinal Desmoid-Type Fibromatosis Mimicking A Retrosternal Thyroid Mass: A Challenging Airway Case
XXIII. World Congress of Otorhinolaryngology Head and Neck Surgery – IFOSTANBUL 2026, İstanbul, Türkiye, 9 - 13 Eylül 2026, (Özet Bildiri)
- Yayın Türü: Bildiri / Özet Bildiri
- Basıldığı Şehir: İstanbul
- Basıldığı Ülke: Türkiye
- Açık Arşiv Koleksiyonu: AVESİS Açık Erişim Koleksiyonu
- Sivas Cumhuriyet Üniversitesi Adresli: Evet
Özet
BACKGROUND & AIM
Desmoid-type fibromatosis (DTF) is a rare benign fibroblastic tumor characterized by locally aggressive, infiltrative growth without metastatic potential. Involvement of the head and neck region is uncommon, and cervico-mediastinal extension is exceptionally rare. Owing to their location and growth pattern, such lesions may mimic thyroid-origin masses, resulting in considerable diagnostic complexity and potential airway compromise.
MATERIAL & METHODS
A 62-year-old woman presented with progressively worsening dysphagia and airway-related symptoms. She reported having undergone a presumed thyroid surgery 25 years earlier for similar complaints, after which she experienced prolonged symptomatic relief. Over the preceding five years, her symptoms gradually recurred, with marked deterioration during the final year. Contrast-enhanced computed tomography of the neck and chest revealed a giant 12-cm mass occupying the left thyroid lobe, with posterior extension into the paravertebral space and inferior extension into the superior mediastinum, mimicking a retrosternal thyroid mass. Complete surgical excision was successfully achieved via a transcervical approach. Histopathological and immunohistochemical evaluation confirmed the diagnosis of DTF. Histopathological examination revealed a spindle cell mesenchymal neoplasm. Immunohistochemistry showed diffuse positivity for Vimentin and SMA, and crucially, nuclear positivity for Beta-catenin. Markers for other mesenchymal tumors (CD34, S100, Desmin, CD117, DOG1) were negative. The final diagnosis was DTF.
RESULTS
No complications were observed during the early postoperative period or at the 6-month follow-up. The patient remains under clinical observation.
CONCLUSIONS
This case highlights a rare presentation of giant cervico-mediastinal desmoid-type fibromatosis associated with significant airway compromise, underscoring the critical importance of radiologic–pathologic correlation and a multidisciplinary management approach.
KEYWORDS
Desmoid-type fibromatosis; Aggressive fibromatosis; Cervical mass; Mediastinal extension; Airway management