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

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Abstract

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.

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Abbreviations

MRI:

Magnetic resonance imaging

CT:

Computed tomography

FMH:

Fertigkeitenskala-Münster-Heidelberg

OS:

Overall survival

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Acknowledgements

We are grateful for the help of Margarita Neff-Heinrich, Göttingen, Germany, in proofreading and editing the manuscript.

Funding

This study was funded by the German Childhood Cancer Foundation, Bonn, Germany (grant DKS 2014.13). The authors have no financial relationship with the organization that sponsored the research.

Author information

Authors and Affiliations

  1. Department of Pediatrics and Pediatric Hematology/Oncology, Klinikum Oldenburg AöR, Medical Campus University Oldenburg, Rahel-Straus-Strasse 10, 26133, Oldenburg, Germany

    Anika Hoffmann, Sarah Adelmann, Kristin Lohle & Hermann L. Müller

  2. Department of Pediatric Oncology, University Hospital, Kiel, Germany

    Alexander Claviez

Authors
  1. Anika Hoffmann
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  2. Sarah Adelmann
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  3. Kristin Lohle
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  4. Alexander Claviez
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Contributions

Hoffmann A: Dr. Hoffmann has written and reviewed the manuscript and supervised data acquisition and analyses. She is the study assistant of the German Registry.

Adelmann S: Mrs. Adelmann analyzed retrospective data, and participated in writing and reviewing the manuscript.

Lohle K: Mrs. Lohle performed the statistical analyses, created the figures and tables, and participated in writing and reviewing the manuscript.

Claviez A: Dr. Claviez initiated the study, contributed patients, and participated in analyzing data and writing and reviewing the manuscript.

Müller HL: Dr. Müller is chairman of the German Registry. He supervised data collection, analyses, and participated in writing and review of the manuscript.

Corresponding author

Correspondence to Hermann L. Müller.

Ethics declarations

Conflict of interest

Hermann L. Müller and coauthors declare that they have no conflict of interest.

This manuscript was composed in the absence of any commercial or financial relationships that could be perceived as a potential conflict of interest.

Ethical approval

All procedures performed in KRANIOPHARYNGEOM 2000—NCT00258453 and KRANIOPHARYNGEOM 2007—NCT01272622 involving human participants were in accordance with the ethical standards of the institutional and/or national research committee and with the 1964 Helsinki declaration and its later amendments or comparable ethical standards.

Informed consent

Informed consent was obtained from all individual participants included in the studies in KRANIOPHARYNGEOM 2000—NCT00258453 and KRANIOPHARYNGEOM 2007—NCT01272622.

Trial registration: KRANIOPHARYNGEOM 2000/2007 (NCT00258453; NCT01272622)

Additional information

Communicated by Peter de Winter

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Hoffmann, A., Adelmann, S., Lohle, K. et al. Pediatric prolactinoma: initial presentation, treatment, and long-term prognosis. Eur J Pediatr 177, 125–132 (2018). https://doi.org/10.1007/s00431-017-3042-5

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  • DOI: https://doi.org/10.1007/s00431-017-3042-5

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