Extraordinary Presentations of Pediatric Pituitary Adenoma

Background Pediatric pituitary adenomas are rare tumors; when encountered, they characteristically present with headaches, visual disturbances, and frequent endocrinopathies. However, they have a penchant for unusual presentations, and due to their relative rarity in the pediatric population, are liable to be clinically misdiagnosed. Correspondingly, awareness of unusual presentations is an important educational point for skull base surgeons, and essential for ensuring early diagnosis and expedited treatment of this vulnerable patient population. We report three highly atypical cases of pediatric pituitary adenomas, both to illustrate the significant potential of the unexpected, and to demonstrate how these difficult-to-manage tumors provide a host of management challenges from early diagnosis, through protracted cycles of recurrent disease treatment.

Methods Case series.

Results A 14-year-old girl presenting with acute hemiparesis and aphasia; emergent MRI identified an occult, large pituitary adenoma (3.3 cm), with aggressive features including perivascular tumor spread, and complete encasement and compression of the internal carotid artery (ICA), without evidence of acute apoplexy. Emergency transsphenoidal resection preserved visual function, and recovered partial language and motor function. A second 14-year-old girl developed precocious galactorrhea, ultimately leading to MRI of the brain and identification of a macroprolactinoma. Three years later, during the third trimester of her first pregnancy, she developed acute, severe bitemporal hemianopsia. An aggressive trial of medical therapy briefly halted tumor growth; however, within several days visual loss progressed, and the patient was taken for emergent induction of labor at 36 weeks followed by transsphenoidal tumor resection on postpartum day 1. A 12-year-old girl developed combined height arrest and weight gain, and was ultimately diagnosed with an ACTH-secreting microadenoma after successful surgical resection. Although initially disease free for 10 years, she presented with late recurrence, and developed a highly resistant pituitary macroadenoma with observed recurrence with extraordinarily refractory disease. Over a total treatment period of 26 years from 12 to 38, the patient underwent 12 operations via transsphenoidal, transcranial, and skull base routes; two stereotactic radiosurgery treatments, one LINAC treatment, and two courses of EBRT; bilateral adrenalectomy precipitating Nelson–Salassa’s syndrome; and three different chemotherapy protocols, before ultimately succumbing to disease.

Conclusion This brief collection of oddities highlights how the management of pediatric pituitary adenoma is characterized by the unexpected, exceptional, and challenging—whether in making the diagnosis, managing the disease, or definitively treating what can be a quixotic and lifelong condition. Additionally, we report for the first time in a pediatric patient ICA infarct attributable to subacute critical stenosis from pituitary tumor invasion and compression, in lieu of acute apoplexy. These rare and protean tumors are fundamentally beyond the realm of contemporary evidence, and a highly nuanced, individualized approach to treatment is universally recommended.