Sellar Xanthogranuloma: A Quest Based on Nine Cases Assessed with an Anterior Pituitary Provocation Test

Shimizu, Fujio; Takajo, Tomoko; Kinoshita, Yasuyuki; Hanaya, Ryosuke; Arimura, Hiroshi; Sugata, Jun; Sugata, Sei; Bohara, Manoj; Hiraki, Takao; Yoshimoto, Koji; Arita, Kazunori

doi:10.1016/j.wneu.2019.06.021

Cited by 10 publications

(15 citation statements)

References 21 publications

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“…Although calcifications are rare in xanthogranuloma, they have been reported in the literature. [ 1 , 3 ] In the present case, the sellar lesion showed high T1WI and T2WI signals with no areas of calcification.…”

Section: Discussionsupporting

confidence: 51%

Sellar xanthogranuloma: A diagnostic challenge

Alharbi

Alkhaibary

Alaglan

et al. 2022

Surgical Neurology International

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Background: Sellar xanthogranulomas are rare intracranial lesions comprising <1% of all sellar lesions. They were described as a separate entity by the World Health Organization in 2000. Because of the paucity of sellar xanthogranuloma cases reported in the literature, they remain a diagnostic challenge with indefinite origin, clinical course, and outcome. The present study reports a case of sellar xanthogranuloma describing the clinical presentation, radiological/pathological characteristics, and outcome. Case Description: A 43-year-old female, known to have diabetes, hypothyroidism, and polycystic ovarian syndrome, presented with a 2-week history of sudden right-sided facial deviation, periorbital pain, and moderate-intensity headache. The patient also reported amenorrhea not improving with polycystic ovarian syndrome treatment. Neurologic examination showed bilateral visual field defects and impaired visual acuity. Computed tomography scan, without contrast, revealed a hypodense sellar lesion with areas of hyperdensity. Magnetic resonance imaging showed a well-defined sellar lesion, exhibiting high signals on T1-weighted and T2-weighted images. The patient underwent microscopic trans-nasal trans-sphenoidal excision of the lesion. Histological sections of the sellar lesion revealed fibrous connective tissue with chronic inflammatory cells and cholesterol clefts, suggestive of xanthogranuloma. The patient is currently followed up at neurosurgery, endocrinology, and ophthalmology clinics with periodic laboratory/radiological investigations. Conclusion: Sellar xanthogranulomas remain rare intracranial lesions with few cases reported in the literature. Patients mostly present with severe hypopituitarism and visual dysfunction. They show no characteristic radiological features. The diagnosis is confirmed histopathologically, and the prognosis is generally favorable.

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Section: Discussionsupporting

confidence: 51%

Sellar xanthogranuloma: A diagnostic challenge

Alharbi

Alkhaibary

Alaglan

et al. 2022

Surgical Neurology International

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“…Of eight patients with intracranial lesions, four had pituitary dysfunction (posterior dysfunction in one, anterior dysfunction in one, and both dysfunctions in two), three had visual impairment, two had epilepsy, and one had mental retardation. There are few case reports describing endocrine dysfunction and/or visual impairment in patients with JXG of the sellar region 6,24,25 . All pituitary dysfunctions in the four patients in our series were present at the time of JXG diagnosis.…”

Section: Discussionmentioning

confidence: 56%

“…There are few case reports describing endocrine dysfunction and/or visual impairment in patients with JXG of the sellar region. 6,24,25 All pituitary dysfunctions in the four patients in our series were present at the time of JXG diagnosis. In patients with LCH, CDI usually develops at two or three years after the diagnosis of LCH, followed by anterior pituitary dysfunction.…”

Section: Discussionmentioning

confidence: 70%

Long‐term outcomes of children with extracutaneous juvenile xanthogranulomas in Japan

Maeda

Morimoto

Shibata

et al. 2020

Pediatric Blood & Cancer

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Background: Juvenile xanthogranuloma (JXG) is the most common non-Langerhans cell histiocytosis in children. The mortality and morbidity of JXG with extracutaneous lesions remain unclear. Methods: Data of patients aged < 18 years who were diagnosed with JXG between 2001 and 2010 were retrospectively collected through a nationwide survey.Results: Twenty patients (11 male and nine female) had extracutaneous lesions. The median observation time was 10 years (range, 0-17). Six patients presented with symptoms at birth. The median age at diagnosis was 8.5 months (range, 0 month-13 years). Fifteen patients underwent treatment for JXG, including chemotherapy (n = 11), and five did not receive treatment. All patients except one survived; 17 were disease-free and two survived with disease. One newborn-onset patient with liver, spleen, and bone marrow involvement died of the disease. Permanent sequelae included central diabetes insipidus, growth hormone deficiency, and panhypopituitarism detected at diagnosis in three, one, and two patients, respectively. Four patients had visual impairment (optic nerve compression and intraocular invasion in two each), three had epilepsy, one had mental retardation, and one had a skin scar. Eight patients who had intracranial lesions were older at diagnosis, and had a higher frequency of disease-related comorbidities and permanent sequelae than those without intracranial involvement. Conclusions:Patients with extracutaneous JXG had good outcomes, although those with intracranial lesions had serious permanent sequelae. Effective and safe treatment regimens for patients with intracranial JXG need to be developed.

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“…XGs are generally present in the second and fourth decades of life [ 39 ]. In the largest cohorts, a slight male predominance has been identified [ 3 , 5 , 25 , 40 ]; however, smaller studies have opposite findings [ 17 , 18 , 22 , 41 ].…”

Section: Demographics and Clinical Presentationmentioning

confidence: 99%

Xanthogranuloma of the Sellar Region: A Comprehensive Review of Neuroimaging in a Rare Inflammatory Entity

Lozovanu

Georgescu

Florescu³

et al. 2022

JPM

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Xanthogranuloma of the sellar region is a rare chronic inflammatory lesion resulting from secondary hemorrhage, inflammation, infarction, and necrosis of an existing Rathke’s cleft cyst, craniopharyngioma, or pituitary adenoma. Sellar xanthogranulomas are challenging to differentiate from other cystic lesions preoperatively due to the lack of characteristic imaging features. We performed a literature overview of the clinical and paraclinical features, treatment options, and long-term outcomes of patients with sellar xanthogranuloma, focusing on the preoperative radiological diagnosis. The hyperintense signal in both T1- and T2-weighted sequences, cystic or partially cystic morphology, ovoid shape, sellar epicenter, intra- and suprasellar location, intratumoral calcifications, linear rim contrast enhancement, and the absence of cavernous sinus invasion suggest xanthogranuloma in the preoperative differential diagnosis. An endoscopic endonasal gross total resection without radiotherapy is the preferred first-line treatment. Given the low rate of recurrence rate and low chance of endocrinological recovery, a mass reduction with decompression of the optic apparatus may represent an appropriate surgical goal. Identifying the xanthogranulomas’ mutational profile could complement histopathological diagnosis and give insight into their histo-pathogenesis. A better preoperative neuroimagistic diagnosis of sellar xanthogranulomas and differentiation from lesions with a poorer prognosis, such as craniopharyngioma, would result in an optimal personalized surgical approach.

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Sellar Xanthogranuloma: A Quest Based on Nine Cases Assessed with an Anterior Pituitary Provocation Test

Cited by 10 publications

References 21 publications

Sellar xanthogranuloma: A diagnostic challenge

Sellar xanthogranuloma: A diagnostic challenge

Long‐term outcomes of children with extracutaneous juvenile xanthogranulomas in Japan

Xanthogranuloma of the Sellar Region: A Comprehensive Review of Neuroimaging in a Rare Inflammatory Entity

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