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