Juvenile xanthogranuloma of supra-sellar region: a rare presentation

2015 ◽  
Vol 34 (11) ◽  
pp. 368-370 ◽  
Author(s):  
Katelyn O. Stepan ◽  
Aseem Sharma ◽  
Michael R. Chicoine ◽  
Ravindra Uppaluri ◽  
Sonika Dahiya
Keyword(s):  
Juvenile Xanthogranuloma ◽  
Sellar Region ◽  
Rare Presentation

Juvenile Xanthogranuloma of the Sellar Region with a 5-Year Medical History: Case Report and Literature Review

2021 ◽  
pp. 1-8
Author(s):  
Dekui Cheng ◽  
Fengyu Yang ◽  
Ziji Li ◽  
Fan Qv ◽  
Wei Liu
Keyword(s):  
Medical History ◽  
Pediatric Population ◽  
Benign Lesion ◽  
Giant Cells ◽  
Juvenile Xanthogranuloma ◽  
Epithelial Tissue ◽  
Sellar Region ◽  
Optic Chiasma ◽  
Suprasellar Region ◽  
Field Disturbance

<b><i>Introduction:</i></b> Xanthogranuloma of the sellar region is a rare benign lesion, and there are few cases reported in children. Its histogenesis is controversial, and it is difficult to strictly differentiate it from craniopharyngioma (CP), Rathke’s cleft cyst, or pituitary adenoma. <b><i>Case Presentation:</i></b> A 16-year-old boy presented with a rare xanthogranuloma of the sellar region after complaining of retardation of growth 5 years previously. The ophthalmologic evaluation revealed no visual field disturbance. Endocrinological examination revealed hypopituitarism. Magnetic resonance imaging showed an intrasellar mass extending into the suprasellar region and compressing the optic chiasma, which appeared mixed signals on T1-weighted images. Endonasal transsphenoidal resection of the tumor was performed. Histological analysis of the tumor sections demonstrated granulomatous tissue with cholesterol clefts, hemosiderin deposits, fibrous tissues, multinucleated giant cells, and lymphocyte. Thus, the tumor was pathologically diagnosed as xanthogranuloma of the sellar region, which is different from adamantinomatous CP. There was no epithelial tissue in any part of the tumor including tumor capsule but have focal necrosis and calcification. His endocrinological dysfunction did not recover, so a hormonal replacement was continuously required. <b><i>Conclusion:</i></b> Xanthogranuloma of the sellar region is a rare entity but must be considered in the differential diagnosis of lesions of the sellar region, even in pediatric population. We should think about this disease when dealing with children with stunted growth accompanied by a long medical history. Our case demonstrates the natural progression of the disease, suggesting that xanthogranuloma of the sellar region without epithelial components may be an independent disease.

scholarly journals Rare presentation of juvenile xanthogranuloma in the posterior fossa of a toddler

2021 ◽  
Vol 14 (3) ◽  
pp. e241411
Author(s):  
Jennifer Yang ◽  
Robert Newbury ◽  
Michael Levy ◽  
John Ross Crawford
Keyword(s):  
Posterior Fossa ◽  
Juvenile Xanthogranuloma ◽  
Rare Presentation

scholarly journals SAT-LB312 Central Diabetes Insipidus as Presenting Manifestation of Suprasellar Epidermoid Cyst

2020 ◽  
Vol 4 (Supplement_1) ◽  
Author(s):  
Samia Abdullah Bokhari
Keyword(s):  
Diabetes Insipidus ◽  
Epidermoid Cyst ◽  
Mass Effect ◽  
Cystic Mass ◽  
Central Diabetes Insipidus ◽  
Neural Tube Closure ◽  
Visual Field Defects ◽  
Sellar Region ◽  
Rare Presentation ◽  
Surrounding Areas

Abstract Back Ground: Epidermoid cysts (ECs) result from the inclusion of squamous epithelial elements during neural tube closure. ECs are tumors constituting 02-1.8% of all brain tumors. ECs are typically found in cerebellopontine angle, but occasionally develop in sellar region. ECs are usually clinically silent, but may produce signs of mass effect as headaches, visual field defects. ECs presenting with Central Diabetes insipidus is reported but rare. Only two cases were reported in literature (Ref: 1).Here we report a case of sellar Epidermoid cyst presenting with Diabetes insipidus. Case Description: 49-year male presented with one-month history of polyuria, polydipsia and weight loss. The initial work up identified normal blood glucose, serum calcium and renal function. The water deprivation test confirmed the diagnosis of central Diabetes insipidus. Further pituitary hormonal assessment revealed panhypopituitarism along with diabetes insipidus. The MRI of brain showed evidence of large sellar supracellar cystic mass with a differential diagnosis of craniopharyngioma, Rathkeys cyst. Surgery performed in order to remove the tumor. The pathological report confirmed the tumor as epidermoid cyst. He did well through hospital stay. DI and along with panhypopituitarism persisted post operatively and treated with hormonal replacement. Conclusion: ECs of sellar region vary in presentation depending upon their location, and extension into surrounding areas producing mass effects. Diabetes Insipidus is a rare presentation in these rare tumors. References: 1: CW huo, C Caputo,YY Wang: Supracellar keratinous cyst: A case report and review on its radiological features and treatment: Surgical Neurology International 2018,9;15

Rare presentation of juvenile xanthogranuloma in the thoracic spine of an adult patient: case report and literature review

2011 ◽  
Vol 153 (9) ◽  
pp. 1813-1818 ◽  
Author(s):  
Ayushi Jain ◽  
Kusum Mathur ◽  
Suman Khatri ◽  
Shweta Kasana ◽  
S. K. Jain
Keyword(s):  
Case Report ◽  
Literature Review ◽  
Adult Patient ◽  
Thoracic Spine ◽  
Juvenile Xanthogranuloma ◽  
Patient Case ◽  
Rare Presentation

Abstract #842 Partial Hypopituitarism Following Miscarriage- A Rare Presentation

2018 ◽  
Vol 24 ◽  
pp. 195
Author(s):  
Monisha Priyadarshini Kumar ◽  
Irtsam Shahid ◽  
Daniela Ciltea
Keyword(s):  
Rare Presentation

Abstract #255: Kenny-Caffey Syndrome: A Rare Presentation of a Rare Disease

2006 ◽  
Vol 12 ◽  
pp. 93-94
Author(s):  
Khurshid Ahmad Khan ◽  
Stephen A. Brietzke
Keyword(s):  
Rare Disease ◽  
Rare Presentation
Sign in / Sign up

Export Citation Format

Share Document