Clinical features and literature review in children and adolescent patients with functioning gonadotroph pituitary neuroendocrine tumors.

PURPOSE: To analyze the clinical characteristics of children and adolescent patients with functioning gonadotroph pituitary neuroendocrine tumors (FGnPTs).

METHODS

Two cases of adolescents with FGnPTs were reported. Seven previously reported cases of child or adolescent FGnPTs were also included to summarize the clinical characteristics of this population through a literature review. Furthermore, we compared nine children/adolescent patients with FGnPTs with 11 premenopausal female patients with FGnPTs at our center.

RESULTS

The onset age of nine children/adolescent patients with FGnPTs was 11.2 ± 3.0 years. Clinical manifestations included menstrual disorders (7/7, 100.00%), multiple ovarian cysts (6/7, 85.71%), spontaneous ovarian hyperstimulation syndrome (sOHSS) (5/7, 71.42%), testicular enlargement (1/2, 50%), headache (2/7, 28.57%), and vision deterioration. The sex hormone profiles revealed a nonsuppressed follicle-stimulating hormone (FSH) (22.53 ± 15.90 IU/L) with hyperestrogenemia (2284 ± 2857pg/ml) or hypoandrogenemia (average testosterone concentration of 0.32 ng/ml). Pituitary magnetic resonance imaging (MRI) revealed macroadenomas (8/9, 88.89%). Compared with premenopausal female patients with FGnPTs, female children/adolescents with FGnPTs had lower luteinizing hormone (LH) levels [0.28 (0.07, 0.43) vs. 5.95 ± 14.17 IU/L, P = 0.044], a higher follicle-stimulating hormone/luteinizing hormone ratio (FSH/LH ratio) [67.40 (33.42, 214.29) vs. 6.10 (2.84, 24.51), P = 0.035], a greater maximum diameter of ovarian cysts (135 ± 24 vs. 88 ± 31 mm, P = 0.017), and a greater likelihood of developing hypopituitarism (3/7, 42.86% vs. 0/9, 0.00%, P = 0.043).

CONCLUSION

When children present with isosexual precocious puberty, their sex hormone profile should be tested. If there is hyperestrogenemia and hypoandrogenemia/hyperandrogenemia with noninhibited FSH or LH levels, further pituitary MRI should be performed. Transsphenoidal pituitary tumor resection is preferred to avoid unnecessary ovarian cystectomy and improve the long-term prognosis.