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. 1999 Mar;80(3):231–234. doi: 10.1136/adc.80.3.231

Treatment of gonadotropin dependent precocious puberty due to hypothalamic hamartoma with gonadotropin releasing hormone agonist depot

V N de Brito 1, A Latronico 1, I Arnhold 1, L Lo 1, S Domenice 1, M Albano 1, M Fragoso 1, B Mendonca 1
PMCID: PMC1717869  PMID: 10325702

Abstract

The gonadotropin releasing hormone (GnRH) secreting hypothalamic hamartoma (HH) is a congenital malformation consisting of a heterotopic mass of nervous tissue that contains GnRH neurosecretory neurons attached to the tuber cinereum or the floor of the third ventricle. HH is a well recognised cause of gonadotropin dependent precocious puberty (GDPP). Long term data are presented on eight children (five boys and three girls) with GDPP due to HH. Physical signs of puberty were observed before 2 years of age in all patients. At presentation with sexual precocity, the mean height standard deviation (SD) for chronological age was +1.60 (1.27) and the mean height SD for bone age was −0.92 (1.77). Neurological symptoms were absent at presentation and follow up. The hamartoma diameter ranged from 5 to 18 mm and did not change in six patients who had magnetic resonance imaging follow up. All patients were treated clinically with GnRH agonists (GnRH-a). The duration of treatment varied from 2.66 to 8.41 years. Seven of the eight children had satisfactory responses to treatment, shown by regression of pubertal signs, suppression of hormonal levels, and improvement of height SD for bone age and predicted height. One patient had a severe local reaction to GnRH-a with failure of hormonal suppression and progression of pubertal signs. It seems that HH is benign and that GnRH-a treatment provides satisfactory and safe control for most children with GDPP due to HH.



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Selected References

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  1. Beningfield S. J., Bonnici F., Cremin B. J. Magnetic resonance imaging of hypothalamic hamartomas. Br J Radiol. 1988 Dec;61(732):1177–1180. doi: 10.1259/0007-1285-61-732-1177. [DOI] [PubMed] [Google Scholar]
  2. Lee P. A. Laboratory monitoring of children with precocious puberty. Arch Pediatr Adolesc Med. 1994 Apr;148(4):369–376. doi: 10.1001/archpedi.1994.02170040035006. [DOI] [PubMed] [Google Scholar]
  3. Lee P. A., Page J. G. Effects of leuprolide in the treatment of central precocious puberty. J Pediatr. 1989 Feb;114(2):321–324. doi: 10.1016/s0022-3476(89)80806-6. [DOI] [PubMed] [Google Scholar]
  4. Lona Soto A., Takahashi M., Yamashita Y., Sakamoto Y., Shinzato J., Yoshizumi K. MRI findings of hypothalamic hamartoma: report of five cases and review of the literature. Comput Med Imaging Graph. 1991 Nov-Dec;15(6):415–421. doi: 10.1016/0895-6111(91)90169-v. [DOI] [PubMed] [Google Scholar]
  5. Mahachoklertwattana P., Kaplan S. L., Grumbach M. M. The luteinizing hormone-releasing hormone-secreting hypothalamic hamartoma is a congenital malformation: natural history. J Clin Endocrinol Metab. 1993 Jul;77(1):118–124. doi: 10.1210/jcem.77.1.8325933. [DOI] [PubMed] [Google Scholar]
  6. Manasco P. K., Pescovitz O. H., Blizzard R. M. Local reactions to depot leuprolide therapy for central precocious puberty. J Pediatr. 1993 Aug;123(2):334–335. doi: 10.1016/s0022-3476(05)81720-2. [DOI] [PubMed] [Google Scholar]
  7. Marshall W. A., Tanner J. M. Variations in pattern of pubertal changes in girls. Arch Dis Child. 1969 Jun;44(235):291–303. doi: 10.1136/adc.44.235.291. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Marshall W. A., Tanner J. M. Variations in the pattern of pubertal changes in boys. Arch Dis Child. 1970 Feb;45(239):13–23. doi: 10.1136/adc.45.239.13. [DOI] [PMC free article] [PubMed] [Google Scholar]
  9. Neely E. K., Hintz R. L., Parker B., Bachrach L. K., Cohen P., Olney R., Wilson D. M. Two-year results of treatment with depot leuprolide acetate for central precocious puberty. J Pediatr. 1992 Oct;121(4):634–640. doi: 10.1016/s0022-3476(05)81162-x. [DOI] [PubMed] [Google Scholar]
  10. Nishio S., Morioka T., Fukui M., Goto Y. Surgical treatment of intractable seizures due to hypothalamic hamartoma. Epilepsia. 1994 May-Jun;35(3):514–519. doi: 10.1111/j.1528-1157.1994.tb02470.x. [DOI] [PubMed] [Google Scholar]
  11. Parker K. L., Baens-Bailon R. G., Lee P. A. Depot leuprolide acetate dosage for sexual precocity. J Clin Endocrinol Metab. 1991 Jul;73(1):50–52. doi: 10.1210/jcem-73-1-50. [DOI] [PubMed] [Google Scholar]
  12. Pescovitz O. H., Comite F., Hench K., Barnes K., McNemar A., Foster C., Kenigsberg D., Loriaux D. L., Cutler G. B., Jr The NIH experience with precocious puberty: diagnostic subgroups and response to short-term luteinizing hormone releasing hormone analogue therapy. J Pediatr. 1986 Jan;108(1):47–54. doi: 10.1016/s0022-3476(86)80767-3. [DOI] [PubMed] [Google Scholar]
  13. Starceski P. J., Lee P. A., Albright A. L., Migeon C. J. Hypothalamic hamartomas and sexual precocity. Evaluation of treatment options. Am J Dis Child. 1990 Feb;144(2):225–228. doi: 10.1001/archpedi.1990.02150260105040. [DOI] [PubMed] [Google Scholar]
  14. Stewart L., Steinbok P., Daaboul J. Role of surgical resection in the treatment of hypothalamic hamartomas causing precocious puberty. Report of six cases. J Neurosurg. 1998 Feb;88(2):340–345. doi: 10.3171/jns.1998.88.2.0340. [DOI] [PubMed] [Google Scholar]
  15. Valdueza J. M., Cristante L., Dammann O., Bentele K., Vortmeyer A., Saeger W., Padberg B., Freitag J., Herrmann H. D. Hypothalamic hamartomas: with special reference to gelastic epilepsy and surgery. Neurosurgery. 1994 Jun;34(6):949–958. doi: 10.1227/00006123-199406000-00001. [DOI] [PubMed] [Google Scholar]

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