Hypothalamic hamartomas and sexual precocity. Evaluation of treatment options
P. J. Starceski, P. A. Lee, A. L. Albright and C. J. Migeon
Department of Pediatrics, University of Pittsburgh (Pa) School of Medicine, Children's Hospital of Pittsburgh, PA.
We describe four male patients with hypothalamic hamartomas associated with
sexual precocity. Our assessment of their management suggests that
resection using current microsurgical techniques is a valid treatment
option if the patient has a normal pubertal endocrine makeup, if the
hamartoma is pedunculated, and if the patient is young enough to require
years of parenteral medical treatment. Such surgical treatment can be
curative, and subsequent growth and development can be normal (patients 1
and 2). However, if the patient is near to pubertal age (patient 3) or if
neurosurgical or gonadotropin releasing hormone analogue treatment is not
available, the natural history (patient 4) suggests that the only
undesirable effects are accelerated growth, tall stature for age, and
premature sexual development during childhood, as well as the psychosocial
problems that may accompany them. Adult height may be compromised, although
the two patients who did not undergo a surgical procedure and did not
receive gonadotropin releasing hormone analogue therapy are above the lower
limits of the normal range of adult male height. Therefore, if the
hamartoma is pedunculated and cessation of pubertal development is desired,
resection of the hamartoma is a reasonable therapeutic option.
