关键词: Pituitary Pituitary neuroendocrine tumor Rathke’s cleft cyst Xanthogranuloma

Mesh : Female Humans Male Young Adult Adult Middle Aged Aged Pituitary Neoplasms / complications diagnostic imaging epidemiology Pituitary Diseases / epidemiology Pituitary Gland / diagnostic imaging pathology Magnetic Resonance Imaging Central Nervous System Cysts / complications Cysts / pathology Granuloma / complications pathology Xanthomatosis / epidemiology pathology

来  源:   DOI:10.1507/endocrj.EJ23-0398

Abstract:
Pituitary xanthogranulomatomas (XG) are a rare pathological entity caused by accumulation of lipid laden macrophages and reactive granuloma formation usually triggered by cystic fluid leakage or hemorrhage. Our aim was to compare clinical characteristics and presenting features of patients with secondary etiology of XG and those with no identifiable founding lesion (primary -\"pure\" XG) in order to gain new insights into this rare pituitary pathology. In a retrospective review of 714 patients operated for sellar masses, at tertiary center, we identified 16 (2.24%) with histologically confirmed diagnosis of pituitary XG over the period of 7 years (2015-2021). Patients were further analyzed according to XG etiology: \"pure\"- XG (n = 8) with no identifiable founding lesion were compared to those with histological elements of pituitary tumor or cyst - secondary XG (n = 8). We identified 16 patients (11 male), mean age 44.8 ± 22.3 years, diagnosed with pituitary XG. Secondary forms were associated with Ratke\'s cleft cyst (RCC, n = 2) and pituitary adenoma (PA, n = 6). The most common presenting features in both groups were hypopituitarism (75%), headache (68.5%) and visual disturbances (37.5%). Predominance of male sex was noted (males 68.75%, females 31.25%), especially in patients with primary forms. Patients with primary pituitary XG were all males (p = 0.0256) and more frequently affected by panhypopituitarism (87.5% vs. 25%, p = 0.0406) compared to patients with secondary causes. Hyperprolactinemia was noted in pituitary tumor group with secondary etiology only (p = 0.0769). Majority of lesions were solid on magnetic resonance imaging - MRI (81.25%). Distinct clinical phenotype was observed dependent on the etiology of XG.
摘要:
垂体黄色肉芽肿瘤(XG)是一种罕见的病理实体,由脂质聚集的巨噬细胞和反应性肉芽肿形成引起,通常由囊性液体渗漏或出血引发。我们的目的是比较具有XG继发性病因的患者和没有可识别的基础病变(原发性-“纯”XG)的患者的临床特征和呈现特征,以获得对这种罕见的垂体病理学的新见解。在对714例鞍区肿块手术患者的回顾性研究中,在三级中心,在7年(2015-2021年)的时间里,我们发现16例(2.24%)经组织学证实诊断为垂体XG.根据XG病因对患者进行了进一步分析:将没有可识别的基本病变的“纯”-XG(n=8)与具有垂体瘤或囊肿的组织学成分-继发性XG(n=8)进行比较。我们确定了16名患者(11名男性),平均年龄44.8±22.3岁,诊断为垂体XG。次要形式与Ratke裂隙囊肿(RCC,n=2)和垂体腺瘤(PA,n=6)。两组中最常见的表现特征是垂体功能减退(75%),头痛(68.5%)和视力障碍(37.5%)。男性占主导地位(男性68.75%,女性31.25%),尤其是在原发性患者中。原发性垂体XG患者均为男性(p=0.0256),更常受全垂体功能减退的影响(87.5%vs.25%,p=0.0406)与具有次要原因的患者相比。仅在继发病因的垂体肿瘤组中观察到高泌乳素血症(p=0.0769)。大多数病变在磁共振成像-MRI上为固体(81.25%)。根据XG的病因观察到不同的临床表型。
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