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鞍区黄色肉芽肿:一种罕见炎症性病变的神经影像学综合综述

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

作者信息

Lozovanu Vera, Georgescu Carmen Emanuela, Florescu Lavinia Maria, Georgiu Carmen, Silaghi Horatiu, Fratea Andrian, Silaghi Cristina Alina

机构信息

Department of Endocrinology, "Iuliu Hatieganu" University of Medicine and Pharmacy Cluj-Napoca, 8 Victor Babes Street, 400012 Cluj-Napoca, Romania.

County Clinical Emergency Hospital Cluj, 3-5 Clinicilor Street, 400006 Cluj-Napoca, Romania.

出版信息

J Pers Med. 2022 Jun 8;12(6):943. doi: 10.3390/jpm12060943.

DOI:10.3390/jpm12060943
PMID:35743728
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9225214/
Abstract

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.

摘要

鞍区黄色肉芽肿是一种罕见的慢性炎症性病变,由现有的拉克氏裂囊肿、颅咽管瘤或垂体腺瘤继发出血、炎症、梗死和坏死引起。由于缺乏特征性影像学表现,鞍区黄色肉芽肿术前与其他囊性病变的鉴别具有挑战性。我们对鞍区黄色肉芽肿患者的临床和副临床特征、治疗选择及长期预后进行了文献综述,重点关注术前影像学诊断。T1加权和T2加权序列均呈高信号、囊性或部分囊性形态、卵圆形、鞍区中心、鞍内和鞍上位置、瘤内钙化、线性边缘强化以及无海绵窦侵犯,提示术前鉴别诊断为黄色肉芽肿。首选的一线治疗是内镜下经鼻全切除且不进行放疗。鉴于复发率低且内分泌恢复机会少,减轻肿块并对视器减压可能是合适的手术目标。确定黄色肉芽肿的突变谱可补充组织病理学诊断并深入了解其组织发病机制。对鞍区黄色肉芽肿进行更好的术前神经影像学诊断并与预后较差的病变(如颅咽管瘤)相鉴别,将有助于制定最佳的个性化手术方案。

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