Читать книгу Anterior Skull Base Tumors - Группа авторов - Страница 54

Chordoma is a low- to intermediate-grade malignant neoplasm originating from the notochord. Base of skull involvement occurs through the sphenoid occipital region, which is the site for approximately 20% of all chordomas. Chordomas of the head and neck region frequently affect patients in the 5th and 6th decades of life [89–93]. Patients typically present with neurological symptoms, headache, and progressive pain. Grossly, chordoma characteristically presents as a lobulated myxoid, expansive mass with mucous, and a slippery appearance. Histologically, chordoma is divided into classic, chondroid, and dedifferentiated phenotypes. The classic type is characterised by a lobulated growth pattern with cords and islands of polygonal and vacuolated cells in a myxomucoid background. The characteristic vacuolated and eosinophilic cell is called a physaliphorous cell. The chondroid type manifests the same features along with areas of hyaline cartilaginous tissue. Dedifferentiation signifying transformation to a high-grade sarcoma shows marked cytologic atypia and high cellularity.