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Background: Craniopharyngioma has a bimodal age distribution with adults having more pronounced hormone deficiencies.1 Studies indicate hormone deficiencies in >80% of cases, most commonly growth hormone (GH) deficiency.2 Comparatively, acromegaly is due to excess GH primarily from a pituitary adenoma.3 Coexistence of a craniopharyngioma and a secretory pituitary adenoma is extremely rare,4 and here we present a case with both. Clinical Case: A 59-year-old woman presented with a two-month history of acutely worsening headache and a week of vision change. A computerized tomography (CT) head scan revealed a 2.2 cm partially solid and cystic sellar mass compressing the optic chiasm. The patient underwent emergent resection. Pre-operational biochemical studies demonstrated normal hypothalamus-pituitary axes except a mildly elevated insulin-like growth factor 1 (IGF-1). Pathology revealed an adamantinomatous craniopharyngioma. Although decreased, IGF-1 remained elevated post-operatively and magnetic resonance imaging (MRI) showed a small non-enhancing area within the pituitary. The patient recalled a gradual increase in shoe and glove size, coarser facial features, weight gain, bilateral carpal tunnel, and mild fluid retention in ankles over the past few years. The GH remained unsuppressed in a glucose suppression test. Conclusion: We present a case of pathology proven craniopharyngioma and concurrent GH excess. The patient presented with a sellar mass with mass effect prompting emergent pituitary resection. Although findings of combined solid and cystic with calcification on MRI point to craniopharyngioma diagnosis,1 it is difficult to discern a concurrent GH-secreting tumor as it tends to be smaller and hypointense on T2-weighted MRI.5 While most craniopharyngioma patients demonstrate hormone deficiency, comprehensive hormonal evaluation and close follow-up are warranted, as in rare cases, patients can have co-existing secretory pituitary adenoma and craniopharyngioma. Early diagnosis and intervention can help prevent detrimental complications related to excess growth hormone. References: 1. Müller HL. Craniopharyngioma. Handb Clin Neurol. 2014;124:235-53. 2. Zoicas F, Schöfl C. Craniopharyngioma in adults. Front Endocrinol. 2012;3:46. 3. Gadelha, M. et al. Complications of Acromegaly and the Impact of the Current Treatment Landscape: An Update. Endocrine Reviews. 2019;40(1):268-332. 4. Fountas A, Chai ST, Ayuk J, Gittoes N, Chavda S, Karavitaki N. A rare challenging case of co-existent craniopharyngioma, acromegaly and squamous cell lung cancer. Endocrinol Diabetes Metab Case Rep. 2018;2018:18-0018. 5. Potorac I, et al. Pituitary MRI characteristics in 297 acromegaly patients based on T2-weighted sequences. Endocrine Related Cancer. 2015;22:169-77. |
