Gonadotropin Releasing Hormone-Independent Precocious Puberty in a 5 Year-old Girl with Suprasellar Germ Cell Tumor Secreting β-hCG and α-Fetoprotein

Abstract

A 5 year-old girl presented with typical features of isosexual precocity with breast and pubic hair development (Tanner stage 3) and menarche, following a few months history of hirsutism of the back and thighs. Stimulation testing revealed GnRH-independent precocious puberty, tertiary hypothyroidism, hyperprolactinemia and mild testosteronemia. The ovaries in ultrasound examination were prepubertal. Tumor markers beta-hCG and AFP were markedly elevated and a 2.5 x 1.5 cm suprasellar germ cell tumor (GCT) was visualized by MRI. Combined chemotherapy followed by radiotherapy resulted in normalization of pubertal features along with estrogen and marker levels. Our observations support the possibility of hCG-dependent precocious puberty (PP) in girls caused by suprasellar hCG-secreting tumor. We emphasize the need of diagnostic management of hCG-dependent PP not only in boys, but also in girls, especially when they present even slight features of androgenization. We hypothesize that the rarity of isosexual PP in girls with hCG-secreting suprasellar GCT results not only from the lower occurrence of these tumors in girls than in boys, but above all from a rare simultaneous concomitant incidence of both high tumor aromatase activity and hCG secreting potency.

Etiologies of Central Diabetes Insipidus in Thai Children

“…Although gonadotropin releasing hormoneindependent precocious puberty is a rare finding in females with germ cell tumors, one female patient in our study presented with precocious puberty and DI. It was hypothesized that high aromatase activity in the germ cell tumor caused the precocity 17 .…”

hCG-Secreting Pineal Teratoma Causing Precocious Puberty: Report of Two Patients and Review of the Literature

“…Kitay 20 suggested that PP could be caused by destruction of the normal pineal parenchymal tissue by tumors, resulting in the decrease of secretion of a pineal antigonadotrophic substance. However, recently, Starzyk et al 15 reported the fifth patient in the literature of PP in girls 15 " 19 . This hypothalamic involvement is also suggested by the other signs and symptoms observed in patients with pineal tumors, such as polyphagia, behavioral disturbance, somnolence, obesity and diabetes insipidus"' 21 .…”

“…The destruction of hypothalamic inhibitory centers by the expansion of a tumor, causing increased gonadotrophin secretion and consequently PP, has also been considered as a possible physiopathological factor 6 . Although ß-hCG has only residual FSH-like activity, Starzyk et al 15 and Kitanaka et al 16 suggested that high levels of ß-hCG secreted by intracranial germ cell tumors could have sufficient intrinsic FSH-like activity to stimulate the ovaries to secrete estradiol. However, none of our patients presented signs or symptoms of hypothalamic involvement other than PP.…”

Testosterone exposure in childhood: discerning pathology from physiology

“…Penile enlargement, macro-orchidism, café -au-lait macules, polyostotic fibrous dysplasia " T Leydig cell hyperplasia [104,105] hCG-secreting tumor Tumorigenesis; LH-like stimulation by hCG Underlying tumor effects " hCG, " T Tumor by imaging [106,107] 17 …”