Multiple primary chordomas of the lung

Abstract

We here report the case of a 40-year-old man with primary pulmonary chordomas. Although an abnormality had been noted on a chest radiograph at age 26 years, the patient had not undergone further examination at that time because he was asymptomatic. Standard chest radiographs and computed tomography showed slow-growing, multiple bilateral pulmonary nodules. Two tumors were resected thoracoscopically to obtain a diagnosis. Pathologic examination resulted in a diagnosis of chordomas. Subsequent systemic examination revealed no additional lesions, not even in the axial skeleton. The patient is alive without any new lesions 38 months after surgery. These clinical and pathological findings suggest that our patient has multiple primary chordomas of the lung, which is an extremely rare condition.

Keywords: AFP, alpha-fetoprotein; BNCT, benign notochordal cell tumor; Benign notochordal cell tumor; Brachyury; CEA, carcinoembryonic antigen; CT, computed tomography; Chordoma; FDG-PET, fluorine-18 fluorodeoxyglucose positron emission tomography; H&E, hematoxylin and eosin; IL-2R, interleukin-2 receptor; Lung; hCG, human chorionic gonadotrophin.

Fig. 1

Pre-operative chest computed tomography images showing solid tumors in the left upper lobe (A), right lower lobe, and left lingular segment (B).

Fig. 2

Photomicrograph at low magnification revealing a well-demarcated nodule in the lung parenchyma (A). The nodule consists of a solid sheet of cells with lightly eosinophilic or prominent vacuolated cytoplasm (physaliphorous cells) (B). The tumor cells have hyperchromatic eccentric nuclei and are surrounded by a myxoid extracellular matrix that is Alcian blue positive (C, D). The nuclei of the tumor cells are positive for brachyury (E). A–C, H&E stain; D, Alcian blue–Periodic acid Schiff; E, immunostaining for brachyury.