Pediatric prolactinoma: initial presentation, treatment, and long-term prognosis.

Prolactinoma is a rare pituitary adenoma secreting prolactin. Studies on diagnostics, treatment, and prognosis in pediatric prolactinoma patients are rare. We analyzed clinical presentation, response to treatment, and prognosis of 27 pediatric prolactinoma patients (10 m/17 f. based on patients' records. Tumors included 6 microadenomas (tumor volume: median 0.2 cm3, range 0.01-0.4 cm3; serum prolactin at diagnosis: median 101 ng/ml, range 33-177 ng/ml), 15 macroadenomas (volume: median 3.3 cm3, range 0.4-25.8 cm3; prolactin: median 890 ng/ml, range 87-8624), and 3 giant adenomas (volume: median 44.5 cm3, range 38.6-93.5 cm3; prolactin: median 4720 ng/ml, range 317-10,400); data for 3 patients were not available. Dopamine agonist treatment (n = 22) was safe and effective, leading to reductions in tumor size (p < 0.01) and prolactin levels (p < 0.01). Threat to vision was the indication for decompressing surgery in three of seven operated patients. No patient was irradiated. Long-term functional capacity was not impaired when compared with other sellar masses (n = 235).
CONCLUSION: In pediatric prolactinoma, diagnosis is based on hyperprolactinemia and imaging. Dopamine agonist treatment is effective and safe. Overall survival and functional capacity as a measure of quality of survival were not impaired, indicating an optimistic prognosis. Surgery should be considered only in emergency situations of threatened visual function, not presenting a fast response to dopamine agonist treatment. Severe side effects of medication and lack of efficacy should be considered as contraindications. What is Known: • In pediatric prolactinoma-a very rare pediatric neuroendocrinological disease-gender-related differences in terms of clinical presentation at initial diagnosis are known. • Due to the rareness of the disease, reports on long-term outcome and prognosis after childhood-onset prolactinoma based on prospective follow-up are not published. What is New: • Dopamine agonist treatment is efficient and safe for tumor volume reduction in pediatric prolactinoma and surgical interventions are recommended only for decompression of the optic chiasm in case of threat to vision. In case of inefficient response to medication, side effects or parental refuse, alternative therapeutic options should be considered. • Quality of life in terms of survival and functional capacity was not impaired in pediatric prolactinoma patients when compared with 235 long-term survivors of different sellar masses.