Precocious puberty due to a hypothalamic hamartoma in a patient surviving to late middle age.

Precocious puberty is considered to be present when gonadal function, which is adult in type and degree, is accompanied by secondary sex development, in the male or female, some years before this would normally be expected (Simpson, 1959). Seckel (1946) regarded genital maturation as precocious when it became apparent in boys before the age of 10 years and in girls before the age of 8 years. It is usually an idiopathic acceleration of normal puberty and is considerably more common in girls than in boys (Jolly, 1951). Identical features can result from involvement of the hypothalamus either directly or indirectly in disorders of adjacent structures, such as the pineal and upper brain-stem. Such cases are more common among boys than girls (Weinberger and Grant, 1941). Of the various pathological lesions affecting the hypothalamus, a well-defined group is the hamartoma occurring on the ventral aspect of the posterior hypothalamus. Over 30 cases have now been described in the literature and the majority of these have been reviewed recently by Schmidt, Hallervorden, and Spatz (1958). Most children developing the condition due to hypothalamic causes either die young or survive only till early adult life, being especially sensitive to operative procedures (Le Marquand and Russell, 1935). That reasonable longevity may be compatible with these growths is suggested by the family reported by Rush, Bilderback, Slocum, and Rogers (1937), in which sexual prococity was found in the father and two sons as well as in other members, and is confirmed by the following study of a patient who survived until the age of 62 years. A.W., a woman aged 62, developed rapidly during childhood. Menstruation began at the age of 7, and by this time the breasts had become as large as her mother's. Growth ceased at the age of 9 when she measured 4 ft. 6j in. and weighed 15 st. 6 lb. The periods, which were heavy, continued regularly at monthly intervals 27' until the age of 21. Thereafter, they were irregular until the menopause occurred in the early thirties. At about this time she began to shave regularly. Although married, she never became pregnant. Her father, a diabetic, died from heart disease. Her mother and seven siblings were all normal. About one month before her sudden death she developed thirst, polyuria, lassitude, and loss of weight. She was found to be a strikingly short and obese woman weighing 15 stones. There …