Growing Up Too Fast - Path PPT

Abstract

A 7 ½ year old boy presented in 2000 with precocious puberty (development of pubic hair, acne and rapid linear growth). Work-up included a brain MRI revealing a suprasellar mass, consistent with a visual pathway glioma. On ophthalmologic evaluation, he was noted to be 20/25 OU with a normal funduscopic exam. He was started on leuprorelin with improvement in his symptoms and followed with serial MRIs, showing slow increase in the size of the tumor. Two years after diagnosis, he was offered radiation therapy, but the family deferred treatment. Serial visual field testing showed a stable temporal defect in the right eye and mild nasal depression in the left eye, consistent with left optic tract involvement. He was lost to follow-up from August 2008 until June 2015 when he presented to the ER with 2 months of progressive right upper and lower extremity weakness. He denied any recent changes in his vision. Eye exam showed 20/20 acuity in both eyes, normal color vision, 2+ temporal pallor in both eyes and an incomplete right homonymous hemianopia on visual field testing. Repeat MRI showed a partially cystic and partially nodular enhancing mass, centered in the region of the left hypothalamus versus left optic tract, with associated infiltration of adjacent structures including the left thalamus and inferior left basal ganglia. A diagnostic procedure was performed