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观察具有神经元分化特征的垂体腺瘤的临床病理特征。

Observation of Clinicopathologic Features of Pituitary Adenoma With Neuronal Differentiation.

机构信息

Department of Pathology, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.

Department of Neurosurgery, The First Affiliated Hospital of Fujian Medical University, Fuzhou, China.

出版信息

Front Endocrinol (Lausanne). 2022 Mar 15;13:848762. doi: 10.3389/fendo.2022.848762. eCollection 2022.

Abstract

OBJECTIVE

To investigate the clinicopathologic features of pituitary adenoma with neuronal differentiation.

METHODS

Four patients with mixed gangliocytoma-pituitary adenomas between January 2011 and January 2021 and 111 new-onset patients with adenomas between January 2019 and June 2021 who attended the First Affiliated Hospital of Fujian Medical University were included in the study. The histological and immunohistochemical findings were analyzed. Neuronal differentiation marker staining was performed on new-onset adenomas, and the related literature was reviewed.

RESULTS

Altogether, more than 100 mixed gangliocytoma-pituitary adenoma cases have been reported in the literature until now, of which pituitary-specific POU-class homeodomain transcription 1 (PIT1) positive adenomas are more frequently observed. In the present study, all 4 patients we described were female, aged 29 to 53 years (mean 39 years). Clinically, 3/4 patients presented with acromegaly, and 1/2 patients presented with headache. Histologically, the tumor was composed of two distinct mixed components. The one was a population of neoplastic ganglionic cells with large nuclei, prominent nucleoli, and abundant basophilic cytoplasm embedded in a fibrillary background. Stains of chromograninA (CgA), synaptophysin (Syn), Calretinin (CR) were positive. Axotomy-like expression was observed in neurofilament (NF) staining. PIT1 was expressed in partial ganglionic cells in all cases. The other component was a population of small uniform cells with round nuclei and acidophilic cytoplasm. Prolactin (PRL) and growth hormone (GH) were positive in all 4 cases. PIT1 was positive in the nuclei of adenomas. Although adenomas and ganglionic regions varied in histology, there was a population of cells with neuronal differentiation expressing PIT1. Additionally, axotomy-like expression of NF staining could be seen in a distant area of adenoma regions. A total of 111 cases of adenomas without ganglionic cells were included in this study, including 7 cases with neuronal differentiation. Among them, 4 cases were prolactinomas, 2 cases were somatotroph adenomas, and 1 case was corticotroph adenoma. 6/7 cases were PIT1-positive adenomas. And the remaining one case is T-PIT-positive adenoma.

CONCLUSIONS

Mixed gangliocytoma-pituitary adenomas are rare tumors with neuronal differentiation. The majority of MGAs are associated with endocrinopathies, mainly acromegaly. Our results suggest that PIT1-positive pituitary adenomas may have neural differentiation potential, which may not be unusual. This indication supports the possibility that the neuronal transdifferentiation of adenomatous cells is a possible mechanism, and the underlying mechanism requires further elucidation.

摘要

目的

研究具有神经元分化的垂体腺瘤的临床病理特征。

方法

纳入 2011 年 1 月至 2021 年 1 月期间的 4 例混合性神经节细胞瘤-垂体腺瘤患者和 2019 年 1 月至 2021 年 6 月期间就诊于福建医科大学附属第一医院的 111 例新发垂体腺瘤患者。分析其组织学和免疫组织化学表现。对新发腺瘤进行神经元分化标志物染色,并复习相关文献。

结果

迄今为止,文献中已报道了 100 多例混合性神经节细胞瘤-垂体腺瘤病例,其中更常观察到垂体特异性 POU 类同源域转录因子 1(PIT1)阳性腺瘤。在本研究中,我们描述的 4 例患者均为女性,年龄 29 至 53 岁(平均 39 岁)。临床上,4 例患者中有 3 例表现为肢端肥大症,2 例患者表现为头痛。组织学上,肿瘤由两种截然不同的混合成分组成。一种是由大细胞核、明显核仁、丰富嗜碱性细胞质组成的成群的肿瘤性神经节细胞,嵌入纤维状背景中。嗜铬粒蛋白 A(CgA)、突触素(Syn)、钙视网膜蛋白(CR)染色阳性。神经丝(NF)染色可见轴突样表达。所有病例中 PIT1 在部分神经节细胞中表达。另一种成分是一群具有圆形核和嗜酸性细胞质的小而均匀的细胞。4 例均显示催乳素(PRL)和生长激素(GH)阳性。PIT1 在腺瘤细胞核中呈阳性。尽管腺瘤和神经节区域的组织学表现不同,但存在一群表达 PIT1 的具有神经元分化的细胞。此外,还可以在腺瘤区域的远处观察到 NF 染色的轴突样表达。本研究共纳入 111 例无神经节细胞的腺瘤病例,其中 7 例具有神经元分化。其中,4 例为催乳素瘤,2 例为生长激素腺瘤,1 例为促肾上腺皮质激素腺瘤。7 例中 PIT1 阳性的腺瘤有 6 例。另 1 例为 T-PIT 阳性腺瘤。

结论

混合性神经节细胞瘤-垂体腺瘤是一种罕见的具有神经元分化的肿瘤。大多数 MGA 与内分泌疾病有关,主要是肢端肥大症。我们的结果表明,PIT1 阳性的垂体腺瘤可能具有神经分化潜能,这可能并不罕见。这一结果提示腺瘤细胞的神经转分化可能是一种潜在的机制,其潜在机制尚需进一步阐明。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/86a9/8965364/18f9acdac96f/fendo-13-848762-g001.jpg

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