XG = xanthogranuloma

  • 文章类型: Case Reports
    背景:垂体黄色瘤病变与Rathke裂囊肿破裂或出血性有关。据报道,大多数病例在根治性切除术后消退。当复发发生时,没有既定的治疗方案。据报道,在一些已发表的病例中,大剂量糖皮质激素是有益的;然而,一旦停止治疗,它们的效果通常不会持续。
    方法:作者报告了一例青少年男性,尽管进行了两次手术干预,但还是出现了与Rathke囊肿相关的复发性黄色肉芽肿性垂体炎。他接受了短期地塞米松治疗,然后接受了塞来昔布和霉酚酸酯的维持治疗。该方案被证明是安全和耐受性良好的,它成功地阻止了他的黄色肉芽肿性垂体炎的另一次复发。
    结论:本病例证明了一种治疗复发性黄色肉芽肿性垂体炎的新的非手术方法。这表明保留皮质类固醇的免疫抑制和抗炎联合方案在其他难治性黄色肉芽肿性垂体炎病例中的潜在应用。
    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.
    METHODS: 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.
    CONCLUSIONS: 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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  • 文章类型: Case Reports
    The authors report the case of a large left occipital mass lesion in an 8-month-old boy who presented with seizure. Neuroimaging demonstrated an approximately 5-cm extraaxial tumor, and the patient underwent partial resection. The tumor was strongly attached to the tentorium and falx. In the postoperative course the residual lesion regressed spontaneously, and after 5 years only a slight residual tumor remained along the tentorium. Histopathological examination of the tumor revealed non-Langerhans cell histiocytosis (non-LCH). However, the tumor was not diagnosed as juvenile xanthogranuloma (JXG) because it lacked Touton giant cells. Hence, the authors described this lesion as a fibroxanthogranuloma. Most intracraniospinal non-LCHs have been reported as JXG; however, several cases of xanthomatous tumors with histopathological features resembling those of JXG have been described as fibrous xanthoma, xanthoma, fibroxanthoma, and xanthogranuloma. Among JXG and the xanthomatous tumors, a review of the literature revealed several cases of dural-based tumors; these dural-based tumors have had favorable courses, including the case described in this report. In addition, the patient in the present case experienced spontaneous regression of the residual tumor. The authors report this unique case and review the literature on isolated intracraniospinal non-LCHs, especially in cases of dural-based lesion.
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