Epiphysis inhibits formation and secretion of the most pituitary hormones and at the first turn gonadotropins. Frequency of epiphysis tumors, pinealomas in children is 2.5% of all verified tumors of brain. 75% of epiphysis tumors are malignant. Endocrinological disturbances can be the first signs of pinealoma. In 10% cases there is precocious puberty syndrome. A 2.5 year-old boy presented to the endocrinology department with an 16-month history of accelerated physical development, fast growth of external genitalia, pubis pilosis, frequent erections, masculinization, low voice, forced laughter attacks. The growth velocity before treatment was 18 cm per year. Physical examination: height 1.04 m, weight 20 kg (97% higher). Masculine habitus with elongated torso and O-shape distorted legs. Head circumference 51 sm, frontal tubers was increased. Blood pressure 90/60 mm Hg. External genitalia development corresponded to 14 yrs. Pubic-hair stage was P3 on the Tanner scale. Penis length is 8 sm, diameter 2.5 sm in calm state. Testicular volume was 11 ml. Scrotum is plicate, pigmented. Boy had frequent spontaneous erections and aggressive behavior. Voice was low, rough. Laboratory tests: The content in plasma of LH was 10.0 U/L; FSH 7.5 U/L, testosterone 12.3 nmol/L. Bone age corresponded to 7 years. Craniogram showed increased intracranial pressure. Magnetic resonance imaging of the brain revealed a pinealoma. Ophthalmologist: signs of increased intracranial pressure. In neurological status - organic symtoms. Treatment: radiotherapy, cyproterone acetate 75 mg daily.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology