THREE UNUSUAL ENDOCRINOPATHIES WITH ASSOCIATED OVARIAN PATHOLOGY: I. OVARIAN AGENESIS. II. PRECOCIOUS PUBERTY. III. VIRILISM1

Abstract

INTRODUCTION THE ORIGINAL intent of the authors in grouping the following three dissimilar cases under one title was to emphasize the nonneoplastic ovarian origin of the endocrinopathies. It soon became apparent that the primary ovarian origin of the syndromes could not be upheld unequivocally. Nevertheless, inasmuch as the three cases present such a dramatic contrast, each to the other, it was thought appropriate to leave them as a triad: the first, an instance of congenital ovarian aplasia; the second, precocious hyperactivity of the ovarian function; and the third, a pathologic perversion of the same function. In all three the diagnosis was verified by surgical exploration, and in each instance benefit was obtained by appropriate therapy. I. OVARIAN AGENESIS In March 1942, Varney, Kenyon and Koch (31) published an article entitled, “Association of short stature, retarded sexual development and high urinary gonatfotrophin titers in women; ovarian dwarfism.” When they pointed out the association of either absent or at most rudimentary ovaries with this picture, they had introduced a new syndrome to the medical world. Thus was explained the mystery of the complete lack of response to pituitary gonadotropic hormones of many cases hitherto erroneously diagnosed as hypophyseal infantilism. In November of the same year, Albright, Smith and Fraser (1) further elucidated the same syndrome and carefully differentiated it from pituitary infantilism, or panhypopituitarism, as they choose to call it. Wilkins and Fleischmann (33), in August 1944, published a masterly exposition of the subject and gave the new syndrome its name, “ovarian agenesis.” They analyzed and summarized the findings in the 47 cases thus far recorded in the literature, of which 18 had been verified either by surgical exploration, peritoneoscopy, or autopsy.