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Sellar Xanthogranulomatosis in a Two-Year-Old Girl: A Case Report
Laith A Ayasa1, Sara Rahhal2, Ala'a K Najjar3
1Faculty of Medicine, Al-Quds University, Jerusalem, PSE.
Insights
This case report details a rare sellar xanthogranuloma in a pediatric patient, emphasizing diagnostic challenges and successful surgical management. The study highlights the importance of multidisciplinary expertise for favorable outcomes in treating this uncommon intracranial lesion.
Area of Science:
- Neuro-oncology
- Pediatric Pathology
- Radiology
Background:
- Sellar xanthogranulomas are exceptionally rare intracranial tumors, posing significant diagnostic and therapeutic hurdles, especially in pediatric populations.
- This report focuses on a unique case of a two-year-old child diagnosed with this rare condition.
Observation:
- The patient presented with headache, left eye ptosis, and neurological deficits.
- Imaging studies (CT and MRI) identified a hypodense sellar lesion.
- Surgical resection via left pterional craniotomy was performed.
Findings:
- Histopathology revealed characteristic features of xanthogranuloma, including foamy macrophages and giant cells.
- Immunohistochemistry (CD1a, langerin) confirmed the diagnosis and excluded Langerhans cell histiocytosis (LCH).
- Complete surgical resection resulted in symptom resolution and neurological recovery.
Implications:
- This case underscores the diagnostic complexities and rarity of sellar xanthogranulomas in children.
- Successful management highlights the critical role of accurate diagnosis and surgical intervention.
- The findings contribute valuable insights into the clinical presentation and treatment of this uncommon intracranial pathology.
Abstract:
Sellar xanthogranulomas are extremely rare intracranial lesions, particularly in pediatric patients, and their diagnostic and therapeutic challenges prompt thorough investigation. We describe a case of a two-year-old toddler diagnosed with sellar xanthogranuloma, highlighting the challenges encountered in its diagnosis and management. The child presented with symptoms, including headache, ptosis of the left eye, and neurological deficits. Brain computed tomography (CT) and magnetic resonance imaging (MRI) revealed a hypodense sellar lesion. The patient underwent a left pterional craniotomy for resection of the mass. Histopathological examination suggested the diagnosis of sellar xanthogranuloma, characterized by foamy macrophages, giant cells, lymphocytic infiltrates, fibrous proliferation, necrotic detritus, and hemosiderin deposits. Further diagnostic precision was achieved through immunohistochemical staining, including CD1a and langerin, which successfully ruled out the possibility of Langerhans cell histiocytosis (LCH), reinforcing the diagnosis of sellar xanthogranuloma. The successful surgical resection of the lesion led to a favorable outcome, evidenced by the significant alleviation of symptoms as well as the restoration of normal neurological function. Post-operative assessments demonstrated a marked improvement in the patient's quality of life, and there were no observed complications or recurrence of the lesion during the follow-up period. In summary, our case report not only highlights the rarity and diagnostic challenges of sellar xanthogranulomas but also emphasizes the importance of collaborative medical expertise in achieving accurate diagnosis and successful therapeutic outcomes in pediatric patients. The successful management of this case offers valuable insights into the clinical presentation, diagnostic complexities, and treatment strategy of sellar xanthogranulomas, further enriching our understanding of this uncommon intracranial pathology.
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