Pituitary Macroprolactinoma Apoplexy in a Prepubertal Girl

Background: Prolactinomas are rare in children, with an incidence of 1: 10.000.000 cases, representing less than 2% of all intracranial tumors in this age group (1). Indeed, only a few cases are reported in prepubertal children. Clinical Case: Eight-year old female prepubertal child, previously healthy, presented a progressive loss of the visual acuity in the last 5 months and was admitted to the emergency department with an intensive headache associated with nauseas, started 48 hours before the admission. At the physical examination, the patient was eutrophic, with an infantile genitalia and no breast development or galactorrhea. No focal neurologic deficits were detected and presented normal pupils reflex and preserved extrinsic eye movements. The Campimetry evidenced bitemporal heteronymous hemianopsia and the nuclear magnetic resonance of the central nervous system identified a suprasellar and intrasellar expansive lesion measuring 3.2 x 2.6 x 2.3 cm, with bleeding signs and compression of the optic chiasm. The hormonal evaluation showed: free T4 0.55 ng/dL (normal value: 0.93-1.70ng/dL), total T4 4.8ng/dL (5.1-14.1ng/dL), TSH 4.06µUI/mL (0.6-5.4µUI/mL), morning serum cortisol 1.80mcg/dL (6.2-2mcg/dL), ACTH 7.0pg/mL (<46pg/mL), prolactin 3.376ng/mL (4.8-23.3ng/mL). The patient initially started glucocorticoid replacement, subsequently levothyroxine and cabergoline. A diagnosis of macroprolactinoma with pituitary apoplexy and optic nerve compression was performed and confirmed by the Immunohistochemical after the transsphenoidal resection. No history of any neoplasms were reported by family members and calcium abnormalities were excluded in the child. A significant improvement of the visual field was suddenly observed and the prolactin substantially decreased to 35ng/mL within 30 days after the intervention. CONCLUSION: We report a rare case of macroprolactinoma in a prepubertal girl associated with pituitary apoplexy. Complete assessment and prompt intervention were essential to recover the visual field and to prevent major sequelae. Reference: (1) Yang, A., Cho, S. Y., Park, H., Kim, M. S., Kong, D.S., Shin, H.J., & Jin, D.K. (2020). Clinical, Hormonal, and Neuroradiological Characteristics and Therapeutic Outcomes of Prolactinomas in Children and Adolescents at a Single Center. Frontiers in Endocrinology. 2020; 11:527.