Xanthomatous Hypophysitis Presenting in An Adolescent Girl: A Long-Term Follow-Up of A Rare Case and Review of The Literature

Wong, Jeanne Sze Lyn; Nasruddin, Azraai; Selveindran, Nalini M; Latif, Kartikasalwah Abd; Kassim, Fauziah; Nair, Sukanya Banerjee; Hong, Jinkuk

doi:10.1016/j.aace.2021.01.008

Cited by 4 publications

(4 citation statements)

References 16 publications

(87 reference statements)

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“…Prevalence of compression symptoms reported in other studies was variable. In study by Yang et al, which included 14 patients, headache was present in 85%, visual disturbances in 71.4% compared to the study with headache as the rst symptom in 30.4% and neuro-ophthalmological disorders in 26.1% of patients [9,13]. Amano et al reported 7 patients with XG, 6 secondary to RCC, with high prevalence of headache 86%, visual disturbances 71% and hypopituitarism 86% [10].…”

Section: Discussionmentioning

confidence: 93%

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Xantho-Granulomatous Hypophysitis: Analysis Of Clinical, Biochemical, Radiological And Etiological Characteristics

Miljić

Pekić

Doknic

et al. 2022

Preprint

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Introduction: Xanthogranulomas (XG) are chronic inflammatory lesions caused by accumulation of lipid laden macrophages with granuloma formation, usually secondary to hemorrhage, infarction or necrosis upon existing neoplasm or cyst. Primary sellar xanthogranulomas are rare and may represent a stage in xanthomatous hypophysitis (XH) evolution.Aim: To analyze clinical presentation, hormonal, radiological and histological features of patients diagnosed with pituitary xanthomatous and xhantogranulomatous lesions in a tertiary clinical center. Material and methods: Data collected from electronic medical histories of patients diagnosed with xanthomatous hypophysistis (XH) and xanthogranulomatous (XG) lesions after pituitary surgery at University Clinical Center of Serbia in Belgrade were analyzed in retrospective manner (2015-2021).Results: Cohort included three pediatric and eleven adult patients (9 male and 5 female), average age 14.6±4.7 years in the pediatric, and 55.3±14.4 years in the adult subgroup. At presentation panhypopituitarism was diagnosed in 8 (57.1%) (7 male, 1 female), hyperprolactinemia in 3 (21.4%) patients, polyuria-polydipsia in only one patient (7.1%). Nine patients (64.3%) presented with headache, 5 (37.5%) with neuro-ophthalmic disorders, 3 (21.4%) with neurological symptoms. On magnetic resonance imaging (MRI) lesions were described as cystic in 3 (21.4 %) or predominantly solid in 11 (78.6%) patients. Secondary forms were associated with Ratke's cleft cyst (RCC) in 3 (21.4%) and pituitary adenoma (PA) in 6 (42.8%) cases. “Pure” sellar XG, independent of preexisting lesions, were found in 5 (35.7%) exclusively male patients. Only one patient was diagnosed with XH, majority with pituitary XG.Conclusion: Our cohort was unique for male preponderance and high prevalence of panhypopituitarism in male patients. XG pituitary lesions were more common than XH. PA followed by RCC were the leading causes of secondary XG lesions. Majority of patients presented with predominantly solid lesions on MRI. The most frequent presenting symptoms were headache and hypopituitarism, followed by neuro-ophtalmic deficits. Polyuria-polydipsia and neurological symptoms were rare at presentation.

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Section: Discussionmentioning

confidence: 93%

“…In our study only one patient was diagnosed with XH while majority had pituitary XG (5 primary, 8 secondary). So far 36 case reports of XH have been published in the literature [12,13], the largest case series included 12 patients with XH [4].…”

Section: Discussionmentioning

confidence: 99%

Xantho-Granulomatous Hypophysitis: Analysis Of Clinical, Biochemical, Radiological And Etiological Characteristics

Miljić

Pekić

Doknic

et al. 2022

Preprint

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“… 4 , 17 , 21 In one case observed by Deodhare et al, 18 corticosteroid therapy temporarily shrunk an XH lesion; however, the lesion significantly expanded following discontinuation of the therapy. In another report by Wong et al, 15 a 14-year-old female with XH experienced gradually increasing size of her sellar mass for 2 years following partial resection, at which point she was treated with high-dose prednisolone. A significant reduction in the mass size was demonstrated 6 months later as a response to the steroid therapy.…”

Section: Discussionmentioning

confidence: 99%

“…Observations Xanthomatous lesions of the pituitary gland are a rare category of hypophysitis that have been described in several reports. [3][4][5][6][7][8][9][10][11][12][13][14][15][16][17][18] Traditionally, these lesions have been divided into two distinct categories: XH and XG; however, there is growing evidence of clinicopathologic overlap between them. 3 XH is histologically characterized by the presence of foamy histiocytes and lymphoplasmacytic infiltrates within the pituitary gland or sellar region with little to no hemosiderin pigment.…”

Section: Discussionmentioning

confidence: 99%

Successful immunomodulatory treatment for recurrent xanthogranulomatous hypophysitis in an adolescent: illustrative case

DeCou

Recinos

Prayson

et al. 2022

Journal of Neurosurgery: Case Lessons

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BACKGROUND Xanthomatous lesions of the pituitary have been linked to ruptured or hemorrhagic Rathke’s cleft cysts. Most cases are reported to resolve following radical resection. When recurrence does occur, there is no established treatment regimen. High-dose glucocorticoids have been reported to be beneficial in several published cases; however, their effects are often not sustained once therapy is discontinued. OBSERVATIONS The authors report the case of an adolescent male who developed recurrent xanthogranulomatous hypophysitis associated with a Rathke’s cleft cyst despite two surgical interventions. He was treated with a short course of dexamethasone followed by a maintenance course of celecoxib and mycophenolate mofetil. This regimen proved to be safe and well-tolerated, and it successfully prevented another recurrence of his xanthogranulomatous hypophysitis. LESSONS This case demonstrates a novel nonsurgical approach to the management of recurrent xanthogranulomatous hypophysitis. It suggests a potential application of a combined corticosteroid-sparing immunosuppressive and anti-inflammatory regimen in other cases of refractory xanthogranulomatous hypophysitis.

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A novel approach to hypophysitis: outcomes using non-glucocorticoid immunosuppressive therapy

Vakharia,

Muhammed,

Remba-Shapiro

et al. 2023

European Journal of Endocrinology

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Objective To determine pituitary function before and after non-glucocorticoid immunosuppressive therapy (NGIT) in subjects with hypophysitis and evaluate their clinical and radiologic outcomes. Design Retrospective, longitudinal study. Methods We reviewed a large database, selected subjects with hypophysitis treated with NGIT, and collected information on the duration of therapy, and clinical, hormonal, and radiologic outcomes. Results Twelve subjects met inclusion criteria. Five subjects had primary hypophysitis (PH), while seven had secondary hypophysitis (SH) due to an underlying systemic inflammatory disease. Mean age ± SD was 48.0±15.7 years and 40.9±13.0 years, for PH and SH, respectively. The majority were female (PH 60% and SH 86%). BMI ± SD at presentation was 25.2±2.5 kg/m2 and 26.8±6.7 kg/m2 for PH and SH, respectively. The most common symptom at presentation was fatigue (75%). All PH subjects (100%) and 2 (28.6%) SH subjects had polyuria/polydipsia. There was a significant decrease in mean pituitary stalk thickness after NGIT (p=0.0051) (mean duration 16.5±4.8 months). New hormone loss or recovery occurred rarely. Mycophenolate mofetil was the most used NGIT: adverse effects prompted discontinuation in two out of seven subjects. Conclusions Subjects with hypophysitis receiving NGIT had stable or improved brain/pituitary MRI findings with significant decrease in pituitary stalk thickness. NGITs did not improve anterior pituitary function. Our findings suggest that NGIT may be considered as an alternative therapy for patients with hypophysitis who require immunosuppression.

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Xanthomatous Hypophysitis Presenting in An Adolescent Girl: A Long-Term Follow-Up of A Rare Case and Review of The Literature

Cited by 4 publications

References 16 publications

Xantho-Granulomatous Hypophysitis: Analysis Of Clinical, Biochemical, Radiological And Etiological Characteristics

Xantho-Granulomatous Hypophysitis: Analysis Of Clinical, Biochemical, Radiological And Etiological Characteristics

Successful immunomodulatory treatment for recurrent xanthogranulomatous hypophysitis in an adolescent: illustrative case

A novel approach to hypophysitis: outcomes using non-glucocorticoid immunosuppressive therapy

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