ESPE Abstracts (2014) 82 P-D-3-1-909

Idiopathic Central Diabetes Insipidus: a Case of Pediatric Xhantogranuloma

Gabriella Pozzobon, Chiara Damia, Andrea Voto, Maria Piera Ferrarello, Gisella Garbetta, Sara Osimani, Giulia Pruccoli, Cristina Partenope & Giuseppe Chiumello

Department of Pediatrics, San Raffaele Scientific Institute, Milan, Italy

Background: Xhantogranuloma of the sellar region (XG) is a very rare brain tumor and is clinically and pathologically distinct from classical adamantinomatous craniopharyngioma, but the differential diagnosis is difficult because there are no typical neuroradiological signes. The characteristic and the outcome of XG in children remain still unclear.

Objective and hypotheses: We aimed to describe a case report of XG and multiple pituitary hormone deficiency.

Method: A 12-year-old boy presented with polyuria, polydipsia and nocturia with episodes of morning headaches and vomiting. No visual disturbancies. The physical exam was normal, with adeguate auxological parameters.

Results: The water balance documented polyuria (67 ml/kg per day) and polydipsia (54 ml/kg per day). Laboratory exams showed diabetes insipidus (diagnosed through fluid deprivation test) associated with central hypothyroidism and secondary adrenal insufficiency. Brain MRI evidenced a small hyperintense expansive sellar and suprasellar lesion with cystic aspect, without vascular abnormalities; no calcifications at TC control were found. The lesion was surgically removed with a transphenoidal approach. The histological examination showed a xanthogranulomatous hypophysitis with giant cell reaction, cholesterol crystal, and necrotic inflammatory material. A multiple pituitary hormone replacement therapy (hydrocotisone, desmopressin, and L-thyroxine) was started with good response.

Conclusion: XG of the sellar region is a very rare tumor and only few pediatric cases have been described in literature, moreover the nature and clinical course remains unclear. When a lesion of the sellar region is associated with a multiple pituitary hormone deficiency XG should be included in the differential diagnosis. Further studies are needed to understand the prognosis and the long-term outcomes of this tumor.

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