Two Perplexing Cases of Craniopharyngioma

Case 1: A 19 yo man presented with sinus pressure, headaches, and neck stiffness not relieved by a course of antibiotics. He subsequently noticed bilateral loss of peripheral vision and binocular horizontal diplopia. Exam revealed a VA of 20/30 OD and 20/40 OS without relative afferent pupillary defect and with alternating esophoria on alternate cover test. Fundus exam showed bilateral disc edema. CT head revealed a suprasellar mass with calcified, solid enhancing, and cystic components. MRI head showed a suprasellar large cystic mass with peripheral enhancement resulting in obstructive hydrocephalus with transependymal CSF flow. He received intravenous dexamethasone and subsequent right frontal intracranial intraventricular endoscopy with cyst wall fenestration and aspiration. Pathology confirmed craniopharyngioma with acellular calcific or keratinous material. Case 2: A 9 yo girl presented with one month of severe vision loss, longstanding frontal HA and dizziness. Exam revealed a VA of 20/200 OU with no relative afferent pupillary defect and grade V optic disc edema OU on fundus exam. MRI revealed a large heterogeneously enhancing suprasellar mass with T1 hyperintense proteinaceous components on pre-contrast images with obstructive hydrocephalus. She received intravenous dexamethasone and an external ventricular drain followed by decompressive resection of the mass. Pathology showed an adamantinomatous craniopharyngioma. After discharge, she developed a large right subdural fluid collection with a midline shift and required subdural peritoneal shunt. Unfortunately, the patient had LP vision OU at follow up.

Poster 1 Two Perplexing Cases of Craniopharyngioma Jeffery Farmer1, Padmaja Sudhakar1, Flavius Raslau1 1 University of Kentucky, Lexington, Kentucky, USA Introduction: Craniopharyngioma are solid or mixed solid-cystic tumors arising from remnants of Rathke's pouch. We encountered two perplexing cases of craniopharyngioma that presented within weeks of each other. Description of Case(s): Case 1: A 19 yo man presented with sinus pressure, headaches, and neck stiffness not relieved by a course of antibiotics. He subsequently noticed bilateral loss of peripheral vision and binocular horizontal diplopia. Exam revealed a VA of 20/30 OD and 20/40 OS without relative afferent pupillary defect and with alternating esophoria on alternate cover test. Fundus exam showed bilateral disc edema. CT head revealed a suprasellar mass with calcified, solid enhancing, and cystic components. MRI head showed a suprasellar large cystic mass with peripheral enhancement resulting in obstructive hydrocephalus with transependymal CSF flow. He received intravenous dexamethasone and subsequent right frontal intracranial intraventricular endoscopy with cyst wall fenestration and aspiration. Pathology confirmed craniopharyngioma with acellular calcific or keratinous material. Case 2: A 9 yo girl presented with one month of severe vision loss, longstanding frontal HA and dizziness. Exam revealed a VA of 20/200 OU with no relative afferent pupillary defect and grade V optic disc edema OU on fundus exam. MRI revealed a large heterogeneously enhancing suprasellar mass with T1 hyperintense proteinaceous components on pre-contrast images with obstructive hydrocephalus. She received intravenous dexamethasone and an external ventricular drain followed by decompressive resection of the mass. Pathology showed an adamantinomatous craniopharyngioma. After discharge, she developed a large right subdural fluid collection with a midline shift and required subdural peritoneal shunt. Unfortunately, the patient had LP vision OU at follow up. Conclusions, including unique features of the case(s): Craniopharyngiomas occur in a bimodal distribution and represent 5-10% of all brain tumors in children. Malignant transformation is rare. Risk factors for visual decline include younger age at diagnosis, optic nerve edema at presentation, and tumor recurrence. References: 1. Bunin GR, Surawicz TS, Witman PA, et al. The descriptive epidemiology of craniopharyngioma. J Neurosurg 1998; 89:547. 2. Jane JA Jr, Laws ER. Craniopharyngioma. Pituitary 2006; 9:323. 3. Karavitaki N, Brufani C, Warner JT, et al. Craniopharyngiomas in children and adults: systematic analysis of 121 cases with long-term follow-up. Clin Endocrinol (Oxf) 2005; 62:397. 4. Lo AC, Howard AF, Nichol A, et al. Long-term outcomes and complications in patients with craniopharyngioma: the British Columbia Cancer Agency experience. Int J Radiat Oncol Biol Phys 2014; 88:1011. 5. Olsson DS, Andersson E, Bryngelsson IL, et al. Excess mortality and morbidity in patients with craniopharyngioma, especially in patients with childhood onset: a populationbased study in Sweden. J Clin Endocrinol Metab 2015; 100:467. Keywords: Tumors, Neuroimaging, High intracranial pressure/headache, Diagnostic tests (ERG, VER, OCT, HRT, mfERG, etc), Eyelid & adnexal disease, Visual fields Financial Disclosures: The authors had no disclosures. Grant Support: None. Contact Information: None provided. 2020 Annual Meeting Syllabus | 59