DOI: http://dx.doi.org/10.18203/2349-3291.ijcp20202631

Hypothalamic hamartoma presenting as central precocious puberty: a rare case report

Priyanka Sharma, Nishant Acharya, Trilok C. Guleria

Abstract


Precocious puberty is defined as children attaining puberty more than 2.5 to 3 standard deviations (SD) earlier than the median age, or before the age of eight years in girls and nine years in boys. Hypothalamic hamartoma (HH) are rare, non progressive tumor like malformation. Precocious puberty due to HH occurs particularly at early ages, even 2 or 3 years. Treatment options for isolated CPP due to HH include GnRH analogs agonists continuously stimulates pituitary gonadotrophs, which further help in decreasing and desensitizing the release of LH, and to a lesser extent, FSH till the time puberty naturally set in. We present a case of precocious puberty due to hypothalamic hamartoma in 3 years old girl. Treated with GnRH analog lupirode and responded well to treatment with cessation of menstruation and reduction in breast size.


Keywords


Gonadotrophs, Hypothalamic hamartoma, Lupirode, Precocious

Full Text:

PDF

References


Mul D, Fredriks AM, Van Buuren S, Oostdijk W, Verloove-Vanhorick SP, Wit JM. Pubertal development in the Netherlands 1965-1997. Pediatr Res. 2001;50:479-86.

Fuqua JS. Treatment and outcome of precocious puberty: An update. J Clin Endocrinol Metab. June 2013, 98(6):2198-207.

Klein KO. Precocious puberty: Who has it? Who should be treated? J Clin Endocrinol Metab. 1999;84(2):411-4.

Kaplowitz P. Clinical characteristics of 104 children referred for evaluation of precocious puberty. J Clin Endocrinol Metab. 2004;89(8):3644-50.

Lahlou N, Carel JC, Chaussain JL, Roger M. Pharmacokinetics and pharma-codynamics of GnRH agonists: Clinical implications in pediatrics. J Pediatr Endocrinol Metab. 2000;13(Suppl 1):S723-37.

Carel JC, Roger M, Ispas S, Tondu F, Lahlou N, Blumberg J, et al. Final height after long-term treatment with triptorelin slow release for central precocious puberty: Importance of statural growth after interruption of treatment. J Clin Endocrinol Metab. 1999;84(6):1973-8.

Heger S, Muller M, Ranke M, Schwarz HP, Waldhauser F, Partsch CJ, et al. Long-term GnRH agonist treatment for female central precocious puberty does not impair reproductive function. Mol Cell Endocrinol. 2006;254-5:217-20.

Papadimitriou A, Beri D, Tsialla A, Fretzayas A, Psychou F, Nicolaidou P. Early growth acceleration in girls with idiopathic precocious puberty. J Pediatr. 2006;149(1):43-6.

Neely EK, Wilson DM, Lee PA, Stene M, Hintz RL. Spontaneous serum gonadotropin concentrations in the evaluation of precocious puberty. J Pediatr. 1995;127(1):47-52.

Weissenberger A, Dell M, Liow K, Theodore W, Frattali C, Hernandez D, et al. Aggression and psychiatric comorbidity in children with hypothalamic hamartomas and their unaffected siblings. J Am Acad Child Adolesc Psychiatr. 2001;40(6):696-703.

Biesecker L, Abbott M, Allen J, Clericuzio C, Feuillan P, Graham J, et al. Report from the workshop on Pallister-Hall syndrome and related phenotypes. Am J Med Genet. 1996;65(1):76-81.

Jaruratanasirikul S, Thaiwong M. Outcome of gonadotropin-releasing analog treatment for children with central precocious puberty: 15- year experience in southern Thailand. J Pediatr Endocrinol Metab. 2011;24(7-8):519-23.