Extraordinary case presentations in pediatric pituitary adenoma: report of 6 cases

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Pediatric pituitary adenomas (PPAs) are rare neoplasms with a propensity for unusual presentations and an aggressive clinical course. Here, the authors describe 6 highly atypical PPAs to highlight this tendency and discuss unexpected management challenges.

A 14-year-old girl presented with acute hemiparesis and aphasia. MRI revealed a pituitary macroadenoma causing internal carotid artery invasion/obliteration without acute apoplexy, which was treated via emergent transsphenoidal resection (TSR). Another 14-year-old girl developed precocious galactorrhea due to macroprolactinoma, which was medically managed. Several years later, she re-presented with acute, severe, bitemporal hemianopia during her third trimester of pregnancy, requiring emergent induction of labor followed by TSR. A 13-year-old boy was incidentally diagnosed with a prolactinoma after routine orthodontic radiographs captured a subtly abnormal sella. An 18-year-old male self-diagnosed pituitary gigantism through a school report on pituitary disease. A 17-year-old boy was diagnosed with Cushing disease by his basketball coach, a former endocrinologist. A 12-year-old girl with growth arrest and weight gain was diagnosed with Cushing disease, which was initially treated via TSR but subsequently recurred and ultimately required 12 operations, 5 radiation treatments involving 3 modalities, bilateral adrenalectomy, and chemotherapy. Despite these efforts, she ultimately died from pituitary carcinoma.

ABBREVIATIONS ACTH = adrenocorticotropic hormone; CD = Cushing disease; GH = growth hormone; GHRH = GH-releasing hormone; ICA = internal carotid artery; IGF-1 = insulin-like growth factor 1; PG = pituitary gigantism; PPA = pediatric pituitary adenoma; SRS = stereotactic radiosurgery; TSR = transsphenoidal resection.
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Contributor Notes

Correspondence Fredric B. Meyer: Mayo Clinic, Rochester, MN. meyer.fredric@mayo.edu.INCLUDE WHEN CITING Published online October 11, 2019; DOI: 10.3171/2019.7.PEDS1950.Disclosures The authors report no conflict of interest concerning the materials or methods used in this study or the findings specified in this paper.
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