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异位侵袭性 ACTH 分泌垂体腺瘤酷似脊索瘤:病例报告及文献复习。

Ectopic invasive ACTH-secreting pituitary adenoma mimicking chordoma: a case report and literature review.

机构信息

Department of Radiology, the Second Affiliated Hospital of Nanjing Medical University, No. 121, Jiangjiayuan Road, 210011, Nan Jing, P.R. China.

Department of Neurosurgery, the Second Affiliated Hospital of Nanjing Medical University, Nan Jing, P.R. China.

出版信息

BMC Neurol. 2023 Feb 23;23(1):81. doi: 10.1186/s12883-023-03124-7.

DOI:10.1186/s12883-023-03124-7
PMID:36814261
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC9948490/
Abstract

BACKGROUND

Ectopic pituitary adenoma (EPA) is defined as a special type of pituitary adenoma that originates outside of the sellar region, is extra- or intra-cranially located, and without connection to normal pituitary tissue. EPA is extremely rare, with most cases presented as case reports or small case series. Due to nonspecific symptoms and laboratory indicators, the preoperative diagnosis, treatment and management for EPA remain challenging.

CASE PRESENTATION

Here, we report the imaging phenotype and pathological findings of a case of invasive EPA in a 47-year-old woman. A preoperative non-contrast CT scan revealed a 5.8 × 3.6 × 3.7 cm soft tissue mass located in the sphenoid sinus and clivus. MRI showed an ill-defined solid mass with heterogeneous signals on T1-weighted and T2-weighted images. The mass displayed infiltrative growth pattern, destroying bone of the skull base, invading adjacent muscles and encasing vessels. The patient underwent partial tumor resection via transsphenoidal endoscopic surgery. Pathological examination led to diagnosis of ectopic ACTH-secreting pituitary adenoma. Post-surgery, the patient received external beam radiotherapy.

CONCLUSION

EPA with invasive growth pattern has rarely been reported. The imaging phenotype displays its relationship to the pituitary tissue and surrounding structures. Immunohistochemical examination acts as a crucial role in differentiating EPA from other skull base tumors. This case report adds to the literature on EPA by summarizing its characteristics alongside a review of the literature.

摘要

背景

异位垂体腺瘤(Ectopic Pituitary Adenoma,EPA)是指起源于鞍区以外、位于颅内外且与正常垂体组织无联系的特殊类型垂体腺瘤。EPA 极为罕见,大多数病例以个案报告或小病例系列形式呈现。由于症状和实验室指标不具特异性,EPA 的术前诊断、治疗和管理仍然具有挑战性。

病例报告

本研究报告了一例 47 岁女性侵袭性 EPA 的影像学表型和病理发现。术前非增强 CT 扫描显示蝶窦和斜坡区有一个 5.8×3.6×3.7cm 的软组织肿块。MRI 显示 T1 加权和 T2 加权图像上信号不均匀的边界不清的实性肿块。肿块呈浸润性生长方式,破坏颅底骨,侵犯邻近肌肉并包裹血管。患者接受了经蝶窦内镜手术的部分肿瘤切除术。病理检查提示异位 ACTH 分泌性垂体腺瘤。术后,患者接受了外照射放疗。

结论

侵袭性生长方式的 EPA 很少见。影像学表型显示其与垂体组织和周围结构的关系。免疫组织化学检查在鉴别 EPA 与其他颅底肿瘤方面起着至关重要的作用。本病例报告通过总结其特征并回顾文献,为 EPA 的文献增添了新内容。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/6439246275a0/12883_2023_3124_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/036524995208/12883_2023_3124_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/147cc9bb007f/12883_2023_3124_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/6439246275a0/12883_2023_3124_Fig3_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/036524995208/12883_2023_3124_Fig1_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/147cc9bb007f/12883_2023_3124_Fig2_HTML.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8673/9948490/6439246275a0/12883_2023_3124_Fig3_HTML.jpg

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Front Endocrinol (Lausanne). 2021 Mar 11;12:619161. doi: 10.3389/fendo.2021.619161. eCollection 2021.
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