[Spinal chordoma. II. Review of the literature since 1960].
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Biomedical subjects
Publications and source records attributed to C Wellinger.
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Vertebral chordomas develop from the intrarachidial -- and more often the intrasomatic --vestiges of the embryonic notochord, and are rare caancers that are more likely to become manifest, usually between 30 and 40 years of age, with a painful radiculo-medullary syndrome than with the clear signs of a tumour. The insidious evolution of this type of cancer, which gives rise to few metastases, and its ambiguous radiological appearance explain why it is usually diagnosed late after an anatomo-pathological examination. The fundamental histological element is the physaliphorous cell, ultrastructural studies of which have demonstrated that the large "mucous" inclusions of interstitial material. The discovery, by Murad and Murthy, of particles enclosed in a membrane in the chordomatous cells indicates possible viral involvement. Radio-surgical treatment results in more comfortable survival (very variable) although it involves repeated high-dose cobalto-therapy and repeated surgical excisions.
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The authors report the results of a study of 139 of their patients with severe rheumatoid arthritis, treated with chlorambucil of cyclophosphamide between 1965 and December 1973, with an observation period of 4 to 12 years. 8 patients were lost to follow-up, 38 died and 93 were reexamined. In this series 7 deaths were attributed to hemolymphoreticulopathy of various cytological types; 6 deaths were due to visceral cancer and 2 others to cancer of the tonsils. They confirm that such treatments increase the risk of hemopathy but apparently not the risk of visceral cancer. The risk was not a function of the total dose of the drug administered since no neoplasia was observed in the 25 subjects who received the highest doses. The addition of chlorambucil to cyclophosphamide seems to increase the risk of hemopathy. It was noticed that the 7 cases of hemolymphoreticulopathy occured after 60 years.