Purpose <p>Pituitary adenomas (PAs) are rare in pediatric patients, with limited data. The study aimed to describe the specific characteristics of pediatric PAs and highlight the challenges in their management.</p> Methods <p>We conducted a retrospective review of patients diagnosed with PAs before age 18, who were followed at the reference center in Lyon, France, between 2010–2023.</p> Results <p>29 patients (19 females) were identified. Median age at diagnosis was 14 years (range: 10-17). 19 patients had prolactinomas, 4 corticotropinomas, 3 somatotropinomas and 3 non-functioning pituitary adenomas (NFPAs). The mean diagnosis delay was 13 months (median: 6, range: 0-70). The most common symptoms were headaches (34%), weight gain (34%) and visual field defects (31%). 17 prolactinomas received dopamine agonists, 4 underwent surgery. All corticotropinomas and somatotropinomas underwent surgery. Germline mutations were identified in 4 of 22 tested patients, including <i>AIP</i> (n = 1) and <i>MEN1</i> (n = 3). Two patients exhibited resistance to first-line therapies. Two patients with corticotropinomas and two patients with somatotopinomas had growth or puberty impact.</p> Conclusion <p>Pediatric PAs pose significant diagnostic and management challenges due to their rarity, variable clinical presentation, and impact on growth and puberty. Prolactinomas are the most common subtype, followed by corticotropinomas and somatotropinomas, while non-functioning adenomas are less frequent. Genetic mutations are relatively common and genetic assessment can inform management and family surveillance. Early diagnosis and a multidisciplinary approach are essential to optimize treatment, minimize complications, and ensure normal growth and pubertal development.</p>

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Pediatric pituitary adenomas: clinical features, treatment strategies, and management challenges

  • Marina Coppens Gouttin,
  • Kevin Perge,
  • Alain Calender,
  • Emilie Doye,
  • Aude Brac de la Perrière,
  • Marc Nicolino,
  • Gérald Raverot,
  • Carine Villanueva

摘要

Purpose

Pituitary adenomas (PAs) are rare in pediatric patients, with limited data. The study aimed to describe the specific characteristics of pediatric PAs and highlight the challenges in their management.

Methods

We conducted a retrospective review of patients diagnosed with PAs before age 18, who were followed at the reference center in Lyon, France, between 2010–2023.

Results

29 patients (19 females) were identified. Median age at diagnosis was 14 years (range: 10-17). 19 patients had prolactinomas, 4 corticotropinomas, 3 somatotropinomas and 3 non-functioning pituitary adenomas (NFPAs). The mean diagnosis delay was 13 months (median: 6, range: 0-70). The most common symptoms were headaches (34%), weight gain (34%) and visual field defects (31%). 17 prolactinomas received dopamine agonists, 4 underwent surgery. All corticotropinomas and somatotropinomas underwent surgery. Germline mutations were identified in 4 of 22 tested patients, including AIP (n = 1) and MEN1 (n = 3). Two patients exhibited resistance to first-line therapies. Two patients with corticotropinomas and two patients with somatotopinomas had growth or puberty impact.

Conclusion

Pediatric PAs pose significant diagnostic and management challenges due to their rarity, variable clinical presentation, and impact on growth and puberty. Prolactinomas are the most common subtype, followed by corticotropinomas and somatotropinomas, while non-functioning adenomas are less frequent. Genetic mutations are relatively common and genetic assessment can inform management and family surveillance. Early diagnosis and a multidisciplinary approach are essential to optimize treatment, minimize complications, and ensure normal growth and pubertal development.