Hoong-Wei Gan

Neuroendocrine Morbidity After Pediatric Optic Gliomas: A Longitudinal Analysis of 166 Children Over 30 Years

CONTEXT Fifty percent of pediatric low-grade gliomas affect the optic pathway, hypothalamus, and suprasellar areas (OP/HSGs), resulting in significant long-term neuroendocrinopathy. OBJECTIVE This study aimed to dissect tumor- from treatment-related risk factors for OP/HSG-associated neuroendocrinopathy. DESIGN This was a retrospective case notes analysis of 166 children with newly diagnosed OP/HSGs at our quaternary center between 1980 and 2010 by multivariate Cox, linear, and logistic regression. RESULTS Patients were of median (range) age 4.9 (0.2-15.4) years at diagnosis and followed up for 8.3 (0.04-26.8) years. Despite high 20-year overall survival (81.0%), progression-free and endocrine event-free survival (EEFS) were 47.2 and 20.8%, respectively. EEFS declined up to 15 years post-diagnosis, with hypothalamic involvement (P < .001) being implicated more than radiotherapy (P = .008) in earlier endocrinopathy; the reverse being true of its density (radiotherapy P < .001; hypothalamic involvement P = .006). GH deficiency (GHD) was most common (40.3%), followed by central precocious puberty (CPP, 26.0%), gonadotropin (GnD; 20.4%), TSH (13.3%), and ACTH (13.3%) deficiencies. GHD increased with later treatment eras (P < .01), but replacement did not increase progression. CPP was associated with future GnD (P < .05). Posterior pituitary dysfunction (PPD; 7.2%) occurred in 57.9% after only biopsies or shunt procedures, and was associated with 6/13 deaths; 50.2% became obese. Tumor extent, surgery, and increased endocrinopathy, rather than radiotherapy, predicted visuocognitive morbidity. CONCLUSIONS This first longitudinal OP/HSG-specific study demonstrates that hypothalamo-pituitary dysfunction evolves hierarchically over decades. Tumor location predicts its speed of onset and radiotherapy its density. GnD can evolve from previous CPP, whereas life-threatening PPD can occur after any surgery. Our data suggest that recent radiation-avoiding chemotherapeutic strategies have increased GHD without improving survival.

Congenital Epidermoid Cyst Presenting as Isolated Painful Trigeminal Neuropathy: Indications for Neuroimaging in the Diagnostic Process

This article reports a case of a cerebellopontine angle epidermoid cyst presenting as isolated painful trigeminal neuropathy. The indolent nature of these uncommon benign tumors leads to frequent delays in their presentation and diagnosis, with patients often initially undergoing dental procedures. This is illustrated in the present case reported here, which highlights the difficulties in identifying trigeminal neuralgia (TN), particularly in its early phases, and supports current recommendations for routine neuroimaging in suspected cases of painful trigeminal neuropathy, which, unlike classic TN, is caused by a disorder other than neurovascular compression (even in the absence of additional neurologic symptoms or signs) and is present particularly in younger patients with atypical features. Additionally, this case report offers a unique patient perspective of living with TN, with a detailed description by one of the authors of the nature of the pain and its impact.