ICDOcode88503

Primary liposarcomas of the larynx are rare, comprising less than 20% of all head and neck liposarcomas and fewer than 0.5% of all laryngeal neoplasms. Patients of all ages are affected, with a median of 64 years. There is a marked male to female predominance (nearly 10:1). The tumours, which occur almost exclusively in the supraglottic larynx or hypopharynx (pyriform sinus), most commonly cause airway obstruction. Imaging, especially with MR or CT, will document the lipomatous nature and extent of the mass. The tumours are firm, polypoid pedunculated, up to 10 cm in greatest dimension and demonstrate a lobulated, glistening, translucent cut surface often traversed by bands of fibrous tissue. The mucosa is usually intact. The majority of cases are well-differentiated lipoma-like liposarcomas (grade I), similar to their histologic counterparts in other anatomic sites, with infrequent reports of myxoid and pleomorphic types. Lipoblasts may be scanty necessitating multiple sections. Atypical cells, scattered lipoblasts, and infiltrative growth pattern differentiate liposarcomas from lipomas. In spite of surgical treatment, multiple recurrences are not uncommon (80% of patients). Metastasis has not been reported and the long-term prognosis is excellent (90% 5-year survival) {733,918,1155, 1371,2765,2772}.

Ii JV

Well Differentiated Whabdomyosarcoma

Fig. 3.49 Rhabdomyosarcoma. A Cells with eosinophilic and striated cytoplasm contain eccentrically placed nuclei with prominent eosinophilic nucleoli. Degeneration is noted. B The neoplastic cells are strongly immunoreactive with desmin, accentuated in both the "epithelioid" and spindled cells.

Ii JV

Fig. 3.49 Rhabdomyosarcoma. A Cells with eosinophilic and striated cytoplasm contain eccentrically placed nuclei with prominent eosinophilic nucleoli. Degeneration is noted. B The neoplastic cells are strongly immunoreactive with desmin, accentuated in both the "epithelioid" and spindled cells.

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