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Evaluation of Treatment Options

Philip J. Starceski, MD; Peter A. Lee, MD, PhD; A. Leland Albright, MD; Claude J. Migeon, MD

Am J Dis Child. 1990;144(2):225-228.

Abstract
• 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.

(AJDC. 1990;144:225-228)

Author Affiliations
From the Departments of Pediatrics (Drs Starceski and Lee) and Neurosurgery (Dr Albright), University of Pittsburgh (Pa) School of Medicine, Children's Hospital of Pittsburgh; and the Department of Pediatrics (Dr Migeon), Johns Hopkins University School of Medicine, Johns Hopkins Hospital, Baltimore, Md.

Footnotes
Accepted for publication August 30, 1989.

Reprint requests to Division of Endocrinology, Children's Hospital of Pittsburgh, 1 Children's P1, 3705 Fifth Ave at DeSoto Street, Pittsburgh, PA 15213 (Dr Lee).

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