Pulmonary Tumor With Notochordal Differentiation: Report of 2 Cases Suggestive of Benign Notochordal Cell Tumor of Extraosseous Origin
ABSTRACT Intraosseous benign notochordal cell tumor (BNCT) is a lesion postulated to be of notochordal cell origin. BNCT has recently been recognized as a potential precursor of classic chordoma, a rare malignant neoplasm usually presenting in the sacrococcygeal region, skull base, or mobile spine. Extra-axial chordoma is extremely rare, and only 2 cases of pulmonary chordoma have been reported previously. We describe herein 2 cases of hitherto-unreported lung tumors that were diagnosed as BNCT. The patients were a middle-aged asymptomatic man and woman who were each incidentally found to have a 15-mm pulmonary nodule on computed tomography. They underwent surgical resection of the tumors under a diagnosis of probable benign tumor of uncertain nature. Histopathologically, both tumors showed solid sheets of peculiar adipocyte-like univacuolated cells, multivacuolated cells, and less vacuolated cells with small, round nuclei and mildly eosinophilic cytoplasm. Mitosis was absent. These features were typical of BNCT. Immunohistochemically, the tumor cells in both cases were positive for brachyury, a transcription factor essential for notochordal cell differentiation and for other markers of notochordal cells including cytokeratins, vimentin, and S-100 protein. Postoperatively, extensive radiographic examination of the whole body revealed no evidence of a primary tumor elsewhere, and both patients are alive and well, with no evidence of disease 1 year after surgery. These 2 cases raise the possibility of a new explanation for the histogenesis of extra-axial chordomas: BNCT may be a precursor lesion of not only conventional axial chordoma but also of extra-axial chordoma.
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