Primary Chordoma of the Lung
ABSTRACT We report the case of a 79-year-old woman referred to our institution for persistent cough and right-sided chest pain. A computed tomographic scan revealed a 2-cm round nodule in the right lower lobe. A wedge resection of the lesion was achieved by video-assisted thoracic surgery. Pathologic examination was consistent with the diagnosis of chordoma. Magnetic resonance imaging of the whole spine and skull basis was normal. Therefore, a diagnosis of primary lung chordoma, an exceptional condition, could be established.
Article: Les chordomesNeurochirurgie 06/2014; DOI:10.1016/j.neuchi.2014.02.003 · 0.47 Impact Factor
Article: [Chordoma.][Show abstract] [Hide abstract]
ABSTRACT: To review in the literature, all the epidemiological, clinical, radiological, histological and therapeutic data regarding chordomas as well as various notochordal entities: ecchordosis physaliphora, intradural and intraparenchymatous chordomas, benign notochordal cell tumors, parachordomas and extra-axial chordomas. To identify different types of chordomas, including familial forms, associations with tuberous sclerosis, Ollier's disease and Maffucci's syndrome, forms with metastasis and seeding. To assess the recent data regarding molecular biology and progress in targeted therapy. To compare the different types of radiotherapy, especially protontherapy and their therapeutic effects. To review the largest series of chordomas in their different localizations (skull base, sacrum and mobile spine) from the literature.
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ABSTRACT: Soft tissue chordomas (STCs) have never been systematically studied because of their rarity and the difficulty in separating them from similar-appearing lesions. Using brachyury to confirm the diagnosis, we have analyzed our experience with 11 cases. Cases coded as "chordoma" or "parachordoma" were retrieved from institutional and consultation files (1989 to 2011) and were excluded from further analysis if they arose from the bone or in a patient with previous axial chordoma. Eleven of 27 cases met inclusion criteria. Patients (8 male; 3 female) ranged in age from 13 to 71 years (mean 44 y). Tumors were located on the buttock (n=2), wrist (n=2), leg (n=2), toe (n=1), thumb (n=1), ankle (n=1), shoulder (n=1), and chest wall (n=1), ranged in size from 0.5 to 10.9 cm (mean 5.3 cm), and consisted of cords and syncytia of spindled/epithelioid cells with vacuolated eosinophilic cytoplasm and a partially myxoid background. Tumors expressed brachyury (10/10), 1 or more cytokeratins (11/11), and S100 protein (10/11). Follow-up information was available for 10 patients (69 mo; range, 2 to 212 mo). Most (n=6) were alive without disease, 2 developed local recurrence and lung metastases, and 1 developed lung metastasis only. One died with unknown disease status. STCs are histologically identical to osseous ones, but differ in their greater tendency to occur in distal locations where small size and surgical resectability result in better disease control. The existence of STC implies that notochordal remnants are not a prerequisite for chordoma development.The American journal of surgical pathology 05/2013; 37(5):719-26. DOI:10.1097/PAS.0b013e31827813e7 · 4.59 Impact Factor