Steinmetz Maximilien, Lebeau Julie, Bouchain Olivier, Moïse Martin, Bianchi Elettra, Andris Cécile, Chapelle Anne-Catherine, Pintiaux Axelle, Reuter Gilles
Department of Neurosurgery, CHU Liège, Avenue de L'hôpital, Liège, Belgium.
Department of Otorhinolaryngology, CHU Liège, Avenue de L'hôpital, Liège, Belgium.
Surg Neurol Int. 2025 Apr 25;16:151. doi: 10.25259/SNI_947_2024. eCollection 2025.
Meningioma is the most frequent primary benign intracranial tumor, with a higher incidence in women. Treatment with progesterone acetates, including cyproterone, nomegestrol, chlormadinone, promegestone, medrogestone, and medroxyprogesterone acetate, has been identified as a risk factor of meningioma, particularly in the anterior and middle cranial fossae. Discontinuation of these treatments often leads to volume stabilization or regression of the tumor.
A 42-year-old woman undergoing treatment with nomegestrol acetate (NA) presented with headaches and visual loss in her right eye. She was diagnosed with a large spheno-orbital meningioma invading the sphenoid and ethmoid sinuses, associated with hyperostosis of the sphenoid wing. An initial resection was performed using an extended endonasal approach. Immunohistochemistry confirmed a chondroid meningioma, Grade II, with progestin receptor in 100% of the tumor cell nuclei and a Ki-67 proliferation index of 3-5%. NA was immediately stopped on diagnosis. Despite the cessation of the NA, the intraosseous sphenoidal part of the tumor continued to grow, leading to optic nerve compression. A second surgery was performed using a right fronto-temporo-orbito-zygomatic approach. Examination of the dura of the middle fossa showed subtle tumoral infiltration, while the Ki-67% index was estimated at 1%. Examination of the sphenoid bone demonstrated reactive hyperostosis with minimal to no tumor infiltration.
This case illustrates that the proliferative activity of the progestin-associated meningioma does not account for intraosseous progression within the sphenoid bone following cessation of progestin therapy. Our observations suggest an upregulation of osteogenesis in infiltrated bone, even as the dural part of the meningioma regresses.
脑膜瘤是最常见的原发性颅内良性肿瘤，女性发病率更高。已确定使用醋酸孕酮类药物治疗，包括环丙孕酮、诺美孕酮、氯地孕酮、普美孕酮、甲羟孕酮和醋酸甲羟孕酮，是脑膜瘤的一个危险因素，尤其是在颅前窝和颅中窝。停止这些治疗通常会导致肿瘤体积稳定或缩小。
一名正在接受醋酸诺美孕酮（NA）治疗的42岁女性出现头痛和右眼视力丧失。她被诊断为侵犯蝶窦和筛窦的大型蝶眶脑膜瘤，伴有蝶骨翼骨质增生。最初采用扩大经鼻入路进行手术切除。免疫组织化学证实为II级软骨样脑膜瘤，100%的肿瘤细胞核中有孕激素受体，Ki-67增殖指数为3-5%。诊断后立即停用NA。尽管停用了NA，但肿瘤的蝶骨骨内部分仍继续生长，导致视神经受压。第二次手术采用右额颞眶颧入路。中颅窝硬脑膜检查显示有细微的肿瘤浸润，而Ki-67%指数估计为1%。蝶骨检查显示有反应性骨质增生，肿瘤浸润极少或无浸润。
该病例表明，孕激素相关脑膜瘤的增殖活性并不能解释孕激素治疗停止后蝶骨内的骨内进展情况。我们的观察结果表明，即使脑膜瘤的硬脑膜部分缩小，浸润骨中的成骨作用仍会上调。
