Ectopic hormone products are typical for cancerous cells. Cancerous cells can produce ACTH, lipotropin, alfa-melanocytostimulating hormone, beta-endorfin, vasopressin, oxytocin, insuline, glucagon, gastrin, secretin, VIP, calcitonin, hypothalamic releasing-hormones, prolactin, parathyroid hormone, growth hormone, chorionic gonadotropin, growth factors. In the majority of ectopic hormone-producing tumor cases clinical symptoms are absent. This is explained by the fact that tumors secretion either pro-hormones, or hormones, different from corresponding normal hormones, that is why their hormone activity is decreased or absent.
Presentation of case: A 2-year-old boy with precocious puberty and gonadotropin-producing hepatoblastoma.
Complains: The early sexual developmen, increased height velocity, fast growth of external genitalia, frequent erections; pubis, axillary, legs and arms pilosis, mustaches, acne, low voice, sharp abdomen aches.
Medical history: In the age of 18 months the boy started to grow rapidly. He grew 25 cm during 12 months and put on 10 kg. First pilosis appeared on pubis and axillary, there was hair growth on legs and arms, external genitalia grew much, appeared frequent erections, acnea vulgaris, voice turned into very low, sharp abdomen aches appeared.
Physical examination: Height 103 cm, weight 22.5 kg. Masculine habitus, low voice. Mild acnea on faces, hypertrichosis on torso and legs. Heart sounds are 102 per minute. Blood pressure 80/50 mm Hg. Abdomen circumference was the 65 cm. Liver is increased, especially its right lobe (10 cm from the costal arch edge), which is dense and tuberous. Pubertal stage was P3 A2 Fa2 on the Tanner scale. Penis length was 6 cm, diameter 2.2 sm in calm state. Testiclar volume was 13 ml. Scrotum is plicate, pigmented. Boy had frequent spontaneous erections and aggressive behavior. Biochemical evaluation revealed high levels of testosterone - 23.7 nmol per liter, LH - 95.0 U per liter; FSH - 4.2 U per liter. Bone age corresponded to 6 years. Liver ultrasonography: the right lobe tumor 10×8 cm. MRI: tumor right lobe of liver, 11×8×10 cm. Treatment. Resection of the right lobe of liver with tumor. Morphological study showed tumor nodular 14×10×14 cm. Microscopy identified it as hepatoblastoma. In 2 weeks after operation the content of testosteron in plasma decreased up to 0.2 nmol per liter, LH - up to 2.2 U per liter, FSH - up to 0.05 U per liter. This case showed that precocious puberty may be a result of ectopic tumor product chorionic gonadotropin.
27 - 29 Sep 2018
European Society for Paediatric Endocrinology