SEARCHCASE_REPORTpediatric ophthalmology, neurology

case_report·pediatric ophthalmology, neurology, pediatrics, oncology, neuro-ophthalmology·PMC10529672

Diencephalic Syndrome: Misleading Clinical Onset of Low-Grade Glioma

Current Oncology · 10 authors, 2 centres

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TL;DR

This retrospective case series describes four pediatric patients whose low-grade gliomas first presented with diencephalic syndrome, causing diagnostic delays due to non-specific symptoms like failure to thrive.

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This retrospective monocentric study describes four pediatric patients with diencephalic syndrome (DS) as an early manifestation of low-grade glioma (LGG). The study analyzes clinical features, diagnostic delays, tumor sites, and outcomes. A key result was a mean diagnostic delay of 22.75 months between symptom onset and diagnosis, with patients initially misdiagnosed with malabsorption or eating disorders. Tumor locations varied, including diencephalic, leptomeningeal, and a rare bulbar site. All patients underwent surgical biopsy and received chemotherapy. Currently, all patients have stable disease but experience long-term sequelae like visual, endocrinological, and neuropsychological dysfunction.

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case_report

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pediatric ophthalmology

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ORIGINAL PAPER

PMC10529672

Diencephalic Syndrome: Misleading Clinical Onset of Low-Grade Glioma

Current Oncology

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ABSTRACT

Simple Summary Diencephalic Syndrome (DS) is a rare disorder secondary to an intracranial neoplasm typically located in the hypothalamic region or its vicinity. Lack of specific symptoms and latency of overt neurologic impairment are misleading and delay diagnosis. A multidisciplinary evaluation is essential to exclude the most frequent causes of failure to thrive, and it is essential to underline the need for pediatricians to focus their attention on this dangerous cause of poor weight gain or stunting in childhood in order to make an early diagnosis and improve prognosis and quality of life. Abstract Background: Diencephalic Syndrome is an atypical early manifestation of low-grade gliomas; so, it is important to detect it in patients that experience a failure to thrive despite adequate length growth and food intake. The purpose of this article is to focus attention on this rare but potentially dangerous cause of poor weight gain or stunting in childhood. Materials and Methods: We describe four patients with Diencephalic Syndrome and low-grade gliomas who were evaluated in our institution from January 2017 to December 2021. Case Description and Results: two patients presented with suspected malabsorption, and two presented with a suspected eating disorder. In all cases, neurological symptoms appeared late, explaining the reason for the diagnostic delay, which impacts negatively on prognosis and on quality of life. Currently, patients 1 and 2 have stable disease in second-line therapy, patient 3 has stable disease post end of second-line therapy, and patient 4 has stable disease in first-line therapy. Everyone is in psychophysical rehabilitation. Conclusions: A multidisciplinary evaluation is essential in order to make an early diagnosis and improve prognosis and quality of life.

CITATION EXPORT

Milena La Spina, Manuela Caruso, Carmela Gulizia et al.. Diencephalic Syndrome: Misleading Clinical Onset of Low-Grade Glioma. Current Oncology. (2023). https://doi.org/10.3390/curroncol30090610

AUTHORS

10 · 2 CENTRES

Milena La Spina, Manuela Caruso, Carmela Gulizia, Mattia Comella, Rachele Soma, Mariaclaudia Meli, Piera Samperi, Gregoria Bertuna, Andrea Di Cataldo, Giovanna Russo

1Pediatric Hematology and Oncology Unit, Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy; [email protected] (M.L.S.); [email protected] (C.G.); [email protected] (M.C.); [email protected] (R.S.); [email protected] (M.M.); [email protected] (P.S.); [email protected] (G.B.); [email protected] (A.D.C.)
2Pediatric Endocrinology and Diabetology Center, Department of Clinical and Experimental Medicine, University of Catania, 95123 Catania, Italy; [email protected]