Background: Hamartoma is a benign, focal malformation, which is composed of tissue elements normally found at that site which are arranged chaotically within the mass. It may occur in many different parts of the body and often is undetected. Hypothalamic hamartoma, unlike most such growths, is symptomatic. It may cause gelastic seisures, visual problems, rage disorders and early onset of puberty.
Objective and hypotheses: A 4.5-year-old girl was hospitalised for the first time in 2004 because of accelerated growth (height 97th centile) and thelarche (Tanner stage 3). Bone age was evaluated at 8 years and 10 months. Dynamic profile of LH and FSH after stimulation with LHRh showed values typical for puberty. PRL, DHEAS, TSH, and α-fetoprotein levels were within the range. Abdominal ultrasonography showed no abnormalities. MRI scan showed tumorous mass (20×18×15 mm) in the hypothalamic region, modelling the bottom of ventricle III and dislocating the cerebral lacuna and optic chiasm.
Method: Girl was referred to Neurosurgery Ward (The Childrens Memorial Health Institute in Warsaw), where a decision was taken to postpone the surgical intervention and start treatment with GnRH analogue.
Results: Girl has been treated with LHRH analogue in the years 20042009. Patient has been regularly evaluated undergoing hormonal tests and diagnostic imaging procedures. Subsequent MRI scans showed no progression in the size of tumour. Treatment was terminated at the age of 9 years and 7 months as the patient reached the adequate height and growth velocity of 6 cm/year with bone age evaluated at 12 years. Menarche occurred at the age of 11 years and 8 months. Currently- at the age of 13 years and 8 months girl menstruates regularly, bone age is equal to chronological age.
Conclusion: Hypothalamic hamartoma may be a rare cause of central precocious puberty in children.
20 - 22 Sep 2014
European Society for Paediatric Endocrinology