Context: Pituitary apoplexy is rare endocrine emergency, resulting from ischemia and/or hemorrhage and necrosis of a pituitary tumor (typically an adenoma). Children and adolescents show severe headache sometimes associated with cranial neuropathies from compression of the optic chiasm or the cavernous sinus.
Case presentation: A 15-year-old boy was admitted with severe headache and vomiting since 3 days, without visual disturbances and neurological defects; normal auxologic parameters, Tanner stage G3Ph3. MRI showed a hemorrhagic intrasellar mass, hyperintense on T1 with a not homogeneous hyperintense fluid level on T2-weighted sequences. Hormone tests showed a central hypoadrenalism revealed by a low-dose ACTH test (cortisol peak 165 mg/l), a central hypothyroidism (FT4 5.8 ng/l, TSH 1.65 mU/l), low levels of IGF-1 for pubertal stage (93 nmol/l) with severe insufficient response to dynamic tests; slightly elevated prolactin levels (87 mg/l) and adequate LH, FSH and testosterone. Visual acuity and visual field were normal. In the suspicion of a pituitary hemorrhagic apoplexy in a pre-existing prolactinoma, hydrocortisone, levothyroxine, hGH and cabergoline 0.75 mg/week (doubled after 2 weeks) were started. The 3-months MRI check showed a reduction of the lesion. Weight and BMI improved and puberty progressed. A 14-year-old boy was admitted for intense throbbing headache since 2 days, associated with vomiting and photophobia without visual disturbances and neurological deficits; height +0.64 SDS (MPH +1.57 SDS), BMI +1.93 SDS; Tanner stage G4Ph4. Brain CT showed "widened appearance of the sellar cavity with an expansive intra and suprasellar formation"; MRI revealed an expansive formation (maximum longitudinal diameter 17 mm) with oval morphology and signs of intralesional bleeding, that exerted a mass effect on the optic chiasm by deforming it upwards. The picture was compatible with hemorrhagic pituitary apoplexy on a possible underlying adenoma. Visual acuity and visual field were normal. Hormonal tests showed hyperprolactinemia (237 mg/l, nv 4-23) with normal remaining pituitary tropins. cabergoline 0.75 mg/week was started. MRI at 8 weeks showed a significant reduction in volume of the lesion (max 12 mm). In both patients, an exome sequencing analysis is ongoing to search genetic defect causative of pituitary adenomas in childhood.
Conclusions: Even rare, it is critical to identify rapidly this entity due to the possible acute hypopituitarism that requires urgent steroid replacement; early surgical intervention is recommended on those with neurological defects. Our patients presented a similar clinical and radiological picture with a very different hormonal outcome.
15 Sep 2022 - 17 Sep 2022