Clinicocytopathological spectrum, including uncommon forms, of nine cases of chordomas with immunohistochemical results, including brachyury immunostaining: A single institutional experience.

OBJECTIVES:To present clinical and cytopathological features of nine cases of chordomas, diagnosed over 9 years and confirmed by brachyury (T) immunostaining. METHODS:Conventional cytological smears, stained with Papanicolaou and May-Grünwald Giemsa, along with corresponding histopathological (n = 8) and immunostained sections (n = 8) were reviewed. Immunohistochemical staining was performed on tissue sections by polymer detection technique. RESULTS:Nine tumours occurred in seven males and two females, with age ranging from 36 to 72 years (average = 58.7), in the sacrum (seven) and spine (two). On fine needle aspiration cytology, five cases were either diagnosed with or diagnosed with a suggestion of a chordoma, while three cases were diagnosed with chordoma as a differential diagnosis. On review, smears were moderately cellular, comprising myxoid stroma (9/9), epithelioid cells (9/9), physaliphorous cells (8/9), including binucleation (7/9), prominent nucleolisation (2/9), pleomorphic cells (2/9) and intranuclear inclusions (3/9). Immunohistochemically, tumour cells expressed cytokeratin (4/4), pan cytokeratin (4/4), epithelial membrane antigen (8/8), S100 protein (6/8) and brachyury (8/8). Five patients underwent surgical excision, including two who underwent adjuvant radiotherapy (RT) and four patients who underwent RT. During follow-up (n = 8), a single patient developed recurrence and another presented with metastatic lesions. Finally, five patients were alive with disease (7-53 months); a single patient was free of disease (4 months), and two patients died of disease; the latter cases displayed pleomorphic cells and intranuclear inclusions. CONCLUSIONS:Chordomas can be primarily diagnosed by fine needle aspiration cytology in a typical clinicoradiological setting with a combination of key cytomorphological features. Pleomorphic cells and intranuclear inclusions are associated with a relatively aggressive subtype. An exact diagnosis has treatment implications and requires confirmation by brachyury immunostaining.

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