Diagnosis: Solitary fibrous tumor (SFT) of the hypopharynx Solitary fibrous tumors are rare spindle cell neoplasms of mesenchymal tissue origin.1 They were originally described as a form of mesothelioma of the pleural or peritoneal cavities2; however, more recently, they have been described throughout the body, including the head and neck, gastrointestinal tract, male reproductive system, and urinary bladder.3 Collectively, SFTs often appear as exophytic masses on gross examination and usually measure between 5 and 6 cm in diameter. The cut surface reveals a nodular, firm, gray-white mass with scant myxoid material and variable areas of hemorrhage or necrosis. The tumors may show a hemangiopericytic pattern with gaping vascular spaces and surrounding short spindle cell proliferation as well as variable histologic patterns, such as areas of hyalinization with clusters of spindle cells adjacent to dense collagen and a patternless proliferation of spindle cells with prominent vascularity. They often demonstrate minimal mitotic activity and only mild cellular atypia. The malignant potential of extrathoracic SFTs has been estimated at 10%4 and is associated with high mitotic activity, nuclear pleomorphism, and hypercellularity.5 Most SFTs consistently express CD34 and bcl-2 on immunohistochemical staining and are usually negative for keratin, desmin, and other mesothelial markers. Solitary fibrous tumors of the head and neck have been reported in the larynx, hypopharynx, buccal mucosa, paranasal sinuses, meninges, thyroid gland, lacrimal gland, salivary glands, and orbit.6 To our knowledge, there are only 8 reports of SFTs in the larynx and 1 report of an SFT occurring in the hypopharynx.7 Clinically, SFTs of the larynx or hypopharynx have occurred in adults (7 men and 2 women) between 29 and 71 years of age (mean age, 47 years) and present with progressive dysphonia, dysphagia, or shortness of breath. Computed tomography or magnetic resonance imaging reveals homogeneous, densely enhancing lesions that are round and sharply circumscribed. No specific features are radiologically diagnostic.8 All reported cases of laryngeal SFTs have occurred in the supraglottis: 3 were found in the false vocal fold; 3 were found in the epiglottis; and 2 were described as involving the “supraglottic larynx.”9 All SFTs were removed surgically: 4 were removed endoscopically with a carbon dioxide laser, 2 via a supraglottic partial laryngectomy, and 2 with a lateral pharyngotomy. The single case of hypopharyngeal SFT that was reported was removed via a lateral pharyngotomy and did not require a tracheotomy. All cases were histologically benign, positive for CD34 on immunohistochemical stains, and without recurrence at follow-up. As SFTs of the head and neck are nearly always benign, surgical treatment is based on the symptoms, and adequate excision should prevent any recurrence. Although several reported cases have been removed with a transcervical approach, an endoscopic laser excision has been the primary surgical approach to these tumors. The postoperative period in this case was mostly uneventful. The patient had a full recovery, including normal vocal fold motion and no mucosal abnormalities. His piriform sinuses were symmetrical and normal in contour. He did note a significant improvement with deglutition, with occasional dysphagia to solid foods. A modified barium swallow examination 2 months after surgery did not reveal penetration or aspiration of liquids. The patient was still free of recurrence at the 9-month follow-up visit. Return to Quiz Case 2. References 1. Enzinger FM, Weiss SW. Soft Tissue Tumors. 4th ed. St Louis, MO: Mosby–Year Book Inc; 2001 2. de Perrot M, Fischer S, Bründler MA, Sekine Y, Keshavjee S. Solitary fibrous tumors of the pleura. Ann Thorac Surg. 2002;74(1):285-29312118790PubMedGoogle ScholarCrossref 3. van de Rijn M, Lombard CM, Rouse RV. Expression of CD34 by solitary fibrous tumors of the pleura, mediastinum, and lung. Am J Surg Pathol. 1994;18(8):814-8207518652PubMedGoogle ScholarCrossref 4. Vallat-Decouvelaere AV, Dry SM, Fletcher CD. Atypical and malignant solitary fibrous tumors in extrathoracic locations: evidence of their comparability to intra-thoracic tumors. Am J Surg Pathol. 1998;22(12):1501-15119850176PubMedGoogle ScholarCrossref 5. Hanau CA, Miettinen M. Solitary fibrous tumor: histological and immunohistochemical spectrum of benign and malignant variants presenting at different sites. Hum Pathol. 1995;26(4):440-4497705824PubMedGoogle ScholarCrossref 6. Brunnermann RB, Ro JY, Ordonez NG, et al. Extrapleural solitary fibrous tumor: a clinicopathologic and immunohistochemical study. Am J Clin Pathol. 1996;106:2178712177PubMedGoogle Scholar 7. Thompson LDR, Karamurzin Y, Wu ML, Kim JH. Solitary fibrous tumor of the larynx. Head Neck Pathol. 2008;2(2):67-7420614325PubMedGoogle ScholarCrossref 8. Ganly I, Patel SG, Stambuk HE, et al. Solitary fibrous tumors of the head and neck: a clinicopathologic and radiologic review. Arch Otolaryngol Head Neck Surg. 2006;132(5):517-52516702568PubMedGoogle ScholarCrossref 9. Dotto JE, Ahrens W, Lesnik DJ, Kowalski D, Sasaki C, Flynn S. Solitary fibrous tumor of the larynx: a case report and review of the literature. Arch Pathol Lab Med. 2006;130(2):213-21616454566PubMedGoogle Scholar
Archives of Otolaryngology - Head & Neck Surgery – American Medical Association
Published: Aug 15, 2011
Keywords: cd34 antigens,deglutition disorders,paranasal sinuses,surgical procedures, operative,vocal folds,hypopharynx,neoplasms,head and neck,solitary fibrous tumor,excision,laryngectomy, partial,tracheotomy,dysphonia,hyaline degeneration,carbon dioxide lasers,endotracheal aspiration,pulmonary aspiration,postoperative period,endoscopy,follow-up,hyperplasia,lasers,mesothelioma,magnetic resonance imaging,computed tomography,hemorrhage,dyspnea
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