A Blur During a Progesterone Peak

During pregnancy, tumors positive for estrogen or progesterone receptors undergo accelerated growth causing symptomatic aggravation. We present a rare and challenging case of a suprasellar meningioma with visual loss presenting at 31 weeks.

Poster 30 A Blur During a Progesterone Peak Divya Ganugapati1, Ketan Bulsara2, Lakshmi Leishangthem3 University of Connecticut School of Medicine, Farmington, Connecticut, USA, 2University of Connecticut, Division of Neurosurgery, Farmington, Connecticut, USA, 3University of Connecticut, Department of Neurology and Ophthalmology, Farmington, Connecticut, USA 1 Introduction: During pregnancy, tumors positive for estrogen or progesterone receptors undergo accelerated growth causing symptomatic aggravation. We present a rare and challenging case of a suprasellar meningioma with visual loss presenting at 31 weeks. Description of Case(s): 40-year-old female 31 weeks pregnant, with no significant medical history, presented with rapidly declining vision loss in her left eye(OS) for one month. Visual acuity(VA) on initial exam outside was 20/20 bilaterally, declining to 20/40 OS within three weeks. Initial neuro-ophthalmology exam at week 4 showed VA of 20/150 and relative afferent pupillary defect(APD) OS. Optic nerves and retinal nerve fiber layers were normal bilaterally. Humphrey visual field(HVF) showed severe inferonasal defect OS and mild nasal defect in the right eye. Urgent MRI without contrast revealed a 9x13x11.5mm suprasellar mass rising extrinsic to the pituitary gland, compressing the left optic nerve and chiasm. Given high risk of visual loss, patient agreed for cesarean section followed by MRI with contrast revealing a suprasellar diffuse homogenous enhancement region consistent with a possible meningioma. She underwent a left cranial-orbital osteotomy for tumor resection and pathology confirmed a WHO grade 1 transitional cell meningioma positive for progesterone receptor. Targeted exome sequencing revealed numerous missense variants (ERBB2, GEN1, ID1, MLH1, PRDM1). 3 months postoperatively, VA returned to 20/20 OS with resolution of her APD and a small residual inferonasal defect on HVF. Conclusions, including unique features of the case(s): Meningiomas diagnosed during pregnancy are more likely to occur on the skull base due to the presence of abundant arachnoid cap cells and perivenous sinuses. Surgical decision-making can be challenging before 37 weeks and special considerations are required to optimize care for the mother and child. This case uniquely presents a complicated localization process and despite some reports of HER2 expression in meningiomas during pregnancy, is the first report to document a variant in the ERRB2 gene in this population. References: Gurcay AG, Bozkurt I, Senturk S, Kazanci A, Gurcan O, et al. Diagnosis, treatment, and management strategy of meningioma during pregnancy, Asian Journal of Neurosurgery, 13(1), 86-89, 2018. Keywords: tumors, visual fields, neuroimaging, skull base Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: None provided. 94 | North American Neuro-Ophthalmology Society