Solitary fibrous tumour (SFT) is a rare, benign, mesenchymal neoplasm that usually arises in the pleura, but rarely involves other sites outside the serosal space (mediastinum, lung, liver, thyroid gland); larynx involvement is very rare with only sporadic cases reported in the literature. We report a case of SFT in a 41-year-old woman with supraglottic laryngeal invovlement; symptoms included dysphonia and mild odynophagia lasting 2 years, and fibre-optic laryngeal evaluation showed a sub-mucosal mass involving the left supraglottis and medial wall of the pyriform sinus. MRI represents the gold standard tool for differential diagnosis (with schwannoma, paraganglioma and haemangioma) and correct staging, while immunohistochemical and cytomorphologic analysis (bcl-2 and CD34 positivity in 90% of cases) is needed for definitive diagnosis. Surgery is the main treatment (endoscopic and open conservative technique), and its goal is a balance between safe oncological resection and good preservation of laryngeal functions; in this particular case an open laryngeal approach was scheduled due to the size of the tumour. Prognosis is good and in only a few cases (especially in pleural SFT) does the biological behaviour take a malignant course.
Solitary fibrous tumour of the supraglottic larynx.
Ravanelli M;Nicolai P.
2016-01-01
Abstract
Solitary fibrous tumour (SFT) is a rare, benign, mesenchymal neoplasm that usually arises in the pleura, but rarely involves other sites outside the serosal space (mediastinum, lung, liver, thyroid gland); larynx involvement is very rare with only sporadic cases reported in the literature. We report a case of SFT in a 41-year-old woman with supraglottic laryngeal invovlement; symptoms included dysphonia and mild odynophagia lasting 2 years, and fibre-optic laryngeal evaluation showed a sub-mucosal mass involving the left supraglottis and medial wall of the pyriform sinus. MRI represents the gold standard tool for differential diagnosis (with schwannoma, paraganglioma and haemangioma) and correct staging, while immunohistochemical and cytomorphologic analysis (bcl-2 and CD34 positivity in 90% of cases) is needed for definitive diagnosis. Surgery is the main treatment (endoscopic and open conservative technique), and its goal is a balance between safe oncological resection and good preservation of laryngeal functions; in this particular case an open laryngeal approach was scheduled due to the size of the tumour. Prognosis is good and in only a few cases (especially in pleural SFT) does the biological behaviour take a malignant course.File | Dimensione | Formato | |
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