Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty

Abstract

<jats:p>Hypothalamic hamartomas (HHs) are benign tumors that are often associated with central precocious puberty. Resection of HHs has been recommended as a treatment option for selected cases of pedunculated lesions, especially in young children. The role of surgery has to be evaluated in the light of the availability of effective medical treatment with gonadotropin-releasing hormone analogs (GnRHas). The authors report the long-term results of total resection of HHs in two children with central precocious puberty and compare it with medical management in four children.</jats:p> <jats:p>The two surgically treated patients underwent total resection of pedunculated HHs at 1.75 (Case 1) and 3.25 years (Case 2) and have received follow-up care for 9 and 8 years, respectively. There were no postoperative complications and computerized tomography scanning confirmed complete tumor removal in both patients. Both patients subsequently experienced some regression of secondary sexual characteristics. The response of luteinizing hormone to GnRH became prepubertal in one patient and was diminished in the other. However, the growth velocity remained elevated (gt 7 cm/year), bone age remained advanced (gt +2 standard deviations) 5 years after surgery, decreasing adult height prediction. In one child, GnRHa therapy was initiated 7 years postsurgery.</jats:p> <jats:p>Four children were treated solely with GnRH agonists and have received follow-up care for 2.3, 6, 9, and 9 years, respectively. These patients have had a complete regression of endocrinological abnormalities, including a normalization of growth velocity and reduction in the rate of skeletal maturation. No side effects were noted with decapeptyl treatment, and one child developed sterile abscesses while receiving Lupron-Depot.</jats:p> <jats:p>The proven efficacy of GnRHa in suppressing puberty and reducing bone age advancement leads the authors to advise against surgery as the initial management of central precocious puberty caused by HHs.</jats:p>

Gonadotropin Releasing Hormone Agonist

“…The authors recommended that the initial management for a child with GDPP due to HH, even when it is pedunculated, should be clinical treatment with GnRH-a. 18 We conclude that HH was the most frequent known cause of GDPP in our series, behaved benignly, and that GnRH-a treatment provided satisfactory and safe control of precocious pubertal development.…”

Endoscopic disconnection for hypothalamic hamartoma with intractable seizure

“…4,9,16,21 In fact, microsurgical resection in this critical area may be related to a high risk of oculomotor palsy, hemiparesis, visual field deficits, central diabetes insipidus, and nonreversible hyperphagia. 3,17,20,25,27 Most neurosurgeons are, therefore, still reluctant to undertake surgery in the case of perihypothalamic lesions.…”

Endocrine Findings in Patients with Optico-Hypothalamic Gliomas

“…Endocrine profile of OHG may be helpful for the differential diagnosis of other suprasellar tumors such as craniopharyngiomas, germinomas and hamartomas. Panhypopituitarism is much more frequent in craniopharyngiomas, germinomas (Honegger et al, 1999;Fahlbusch et al, 1999;Ramelli et al, 1998) and hamartomas (Stewart et al, 1998). Similarly, suprasellar tumors associated with the absence of endocrine deficiencies are more suggestive of optico-hypothalamic gliomas.…”

“…crine deficiencies (Honegger et al, 1999;Stewart et al, 1998). Similarly, deficient endocrine functions are expected in opticohypothalamic gliomas because of their large size.…”