[Sipple's syndrome: a study through three generations. HLA--carcino-embryonic antigen system. Preliminary results (author's transl)]

Abstract

Sipple's syndrome in three members of the same family (three successive generations) is reported, confirming that this syndrome is hereditary and transmitted in an autosomal and dominant way. Whereas pheochromocytoma was clinically detectable, medullary thyroïd carcinoma could only be detected in two cases by systematic search for high levels of calcitonin before and after stimulation. In 6 other siblings, thyroïd carcinoma was eliminated in view of negative stimulation tests. HLA groups were studied, but definite conclusions as to the interest of these data in Sipple's syndrome cannot be drawn from 10 few cases. Persistingly high levels of calcitonin after surgery is known to be of severe prognosis; in the present cases, the very short delay after surgery did not allow definite conclusion, since, according to some authors, several months are necessary to allow a return to normal levels.