Abstract：
Calcified pseudoneoplasms of the neuraxis (CAPNONs) are rare, fibro-osseous lesions with an unknown cause that may present anywhere along the neuroaxis. Little is known about how intracranial CAPNONs present and about patients\' long-term outcomes.
A retrospective institutional review of intracranial pathology-confirmed CAPNONs was performed. Presenting clinical features, management, and clinical outcomes are highlighted. A literature review of intracranial CAPNON lesions was also performed to build on our series.
Ten patients were identified who met the inclusion criteria. Most patients presented with headaches (n = 6; 60%), seizures (n = 5; 50.0%), and neck and facial pain (n = 3; 30.0%). Most lesions were supratentorial (n = 7; 70.0%), with 3 infratentorial origins. Surgical resection was the most common initial management undertaken (n = 7; 70.0%). No new permanent postoperative neurologic deficits were identified. The median clinical and/or radiographic follow-up for all patients was 6.8 years (range, 0.7-23.3 years), with no recurrence of disease for 5 patients who underwent gross total resection. Four of 5 patients with residual or nonresectable lesions showed no interval growth on radiographic follow-up; 1 patient showed progression and worsening of presenting symptoms 2 months after resection. Resection substantially improved seizures and headaches in patients presenting with these symptoms (80% and 83.3%, respectively).
Intracranial CAPNONs may present with a wide variety of symptoms characteristic of the site of origin. The outcomes of these symptoms regarding survival and disease control are generally favorable, although resection does not always yield complete resolution of presenting deficits in certain patients, particularly those presenting with headaches or neck/facial pain.
A retrospective institutional review of intracranial pathology-confirmed CAPNONs was performed. Presenting clinical features, management, and clinical outcomes are highlighted. A literature review of intracranial CAPNON lesions was also performed to build on our series.
Ten patients were identified who met the inclusion criteria. Most patients presented with headaches (n = 6; 60%), seizures (n = 5; 50.0%), and neck and facial pain (n = 3; 30.0%). Most lesions were supratentorial (n = 7; 70.0%), with 3 infratentorial origins. Surgical resection was the most common initial management undertaken (n = 7; 70.0%). No new permanent postoperative neurologic deficits were identified. The median clinical and/or radiographic follow-up for all patients was 6.8 years (range, 0.7-23.3 years), with no recurrence of disease for 5 patients who underwent gross total resection. Four of 5 patients with residual or nonresectable lesions showed no interval growth on radiographic follow-up; 1 patient showed progression and worsening of presenting symptoms 2 months after resection. Resection substantially improved seizures and headaches in patients presenting with these symptoms (80% and 83.3%, respectively).
Intracranial CAPNONs may present with a wide variety of symptoms characteristic of the site of origin. The outcomes of these symptoms regarding survival and disease control are generally favorable, although resection does not always yield complete resolution of presenting deficits in certain patients, particularly those presenting with headaches or neck/facial pain.
摘要：
背景：钙化的神经轴假核质（CAPNONs）很少见，病因不明的纤维骨性病变，可能出现在神经轴上的任何地方。关于颅内CAPNON的表现和患者的长期结局知之甚少。
方法：对颅内病理证实的CAPNON进行回顾性机构审查。呈现临床特征，管理,并强调了临床结果。在我们系列的基础上，还对颅内CAPNON病变进行了文献综述。
结果：确定了10例符合纳入标准的患者。大多数患者出现头痛（n=6，60％），癫痫发作（n=5，50.0%），颈部和面部疼痛（n=3，30.0％）。大多数病变是幕上，（n=7，70.0%），有三个起源。手术切除是最常见的初始管理（n=7，70.0％）。没有发现新的永久性术后神经功能缺损。所有患者的中位临床和/或影像学随访为6.8年（范围0.7-23.3年），5例接受大体全切除术的患者无疾病复发。在影像学随访中，五名残留或不可切除病变的患者中有四名没有间隔增长；一名患者在切除后两个月表现出进展和症状恶化。切除可显著改善出现这些症状的患者的癫痫发作和头痛（80%和83.3%，分别)。
结论：颅内CAPNONs可能表现出多种以起源部位为特征的症状。这些关于生存和疾病控制的结果通常是有利的，虽然切除并不总是能完全解决某些患者的表现缺陷，尤其是那些出现头痛或颈部/面部疼痛的人。
方法：对颅内病理证实的CAPNON进行回顾性机构审查。呈现临床特征，管理,并强调了临床结果。在我们系列的基础上，还对颅内CAPNON病变进行了文献综述。
结果：确定了10例符合纳入标准的患者。大多数患者出现头痛（n=6，60％），癫痫发作（n=5，50.0%），颈部和面部疼痛（n=3，30.0％）。大多数病变是幕上，（n=7，70.0%），有三个起源。手术切除是最常见的初始管理（n=7，70.0％）。没有发现新的永久性术后神经功能缺损。所有患者的中位临床和/或影像学随访为6.8年（范围0.7-23.3年），5例接受大体全切除术的患者无疾病复发。在影像学随访中，五名残留或不可切除病变的患者中有四名没有间隔增长；一名患者在切除后两个月表现出进展和症状恶化。切除可显著改善出现这些症状的患者的癫痫发作和头痛（80%和83.3%，分别)。
结论：颅内CAPNONs可能表现出多种以起源部位为特征的症状。这些关于生存和疾病控制的结果通常是有利的，虽然切除并不总是能完全解决某些患者的表现缺陷，尤其是那些出现头痛或颈部/面部疼痛的人。
