[Metastatic chordoma. General review apropos of 2 cases].
The authors report on two cases of sacrococcygeal chordoma, with complications involving visceral organ metastases and distal bony metastases. Chordomas are malignant tumors which develop in adult subjects which originate from remnants of the embryonic notochord. Sacrococcygeal localization is found in 50% of the 1,300 cases reported in the medical literature, which represents 20% of sacrococcygeal tumors observed. The difficulty and the delay in diagnosing these tumors should decrease by routine CAT scan examination. Diagnostic certainty is based on histological examination, often suggestive of the diagnosis (physaliphore-like cells), possibly combined with ultrastructure and immunohistological study. The clinical course involves local recurrences, but there is a real risk of metastatic development, notably in the case of sacrococcygeal chordomas, with their incidence estimated at 17.5% of cases. Sometimes developing in later stages of their course, histological findings are similar to those of the initial lesion. Organs which are frequently the sites of metastases are the lung (48% of cases) and bone (26%), then the lymphatic organs, the liver, and subcutaneous tissue... Therapeutic management is unsatisfactory, with ideally, complete surgical excision of the initial tumor of its size permits and hence early diagnosis of this condition is a prerequisite for good results.
Raguin
J M
JM
Service de Rhumatologie, CHU de Besançon.
Wendling
D
D
Montcuquet
P
P
Guidet
M
M
D000368:Aged; D001859:Bone Neoplasms / Q000556:secondary*; D002817:Chordoma / Q000175:diagnosis / Q000473:pathology / Q000556:secondary*; D003050:Coccyx; D006801:Humans; D008297:Male; D008875:Middle Aged; D009364:Neoplasm Recurrence, Local; D012447:Sacrum; D013125:Spinal Neoplasms
Les chordomes métastasants. Revue générale à propos de deux observations.