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Treatment-resistant pediatric giant prolactinoma and multiple endocrine neoplasia type 1

2015; Springer Science+Business Media; Volume: 2015; Issue: 1 Linguagem: Inglês

10.1186/s13633-015-0011-5

1687-9856

Hoong‐Wei Gan, Chloe Bulwer, Owase Jeelani, Michael A. Levine, Márta Korbonits, Helen Spoudeas,

Adrenal and Paraganglionic Tumors

Pediatric pituitary adenomas are rare, accounting for A) being eventually identified in intron 4 of the MEN1 gene, potentially explaining the difficulties in management of this tumor. Genetic counseling and screening has now been offered to the wider family.This case emphasizes the need to consider pituitary adenomas in the differential diagnosis of all pediatric suprasellar tumors by careful endocrine assessment and measurement of at least a serum prolactin concentration. It also highlights the lack of evidence for the optimal management of pediatric drug-resistant prolactinomas. Finally, the case we describe demonstrates the importance of a detailed family history and the role of genetic testing for MEN1 and AIP mutations in all cases of pediatric pituitary adenoma.