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神经轴索钙化性假肿瘤(CAPNON):解析一种罕见的非肿瘤性中枢神经系统钙化病变。

Calcifying pseudoneoplasm of the neuraxis (CAPNON): Unraveling a rare non-neoplastic calcified central nervous system lesion.

作者信息

Sassi Farah, Zehani Alia, Ghedira Khalil, Chelly Ines, Bellil Khedija, Haouet Slim

机构信息

Pathology Department, Rabta Hospital, Tunisia Faculty of Medicine, University of Tunis el MANAR, Tunis, Tunisia.

Pathology Department, Rabta Hospital, Tunisia Faculty of Medicine, University of Tunis el MANAR, Tunis, Tunisia.

出版信息

Int J Surg Case Rep. 2023 Aug;109:108588. doi: 10.1016/j.ijscr.2023.108588. Epub 2023 Aug 3.

Abstract

INTRODUCTION AND IMPORTANCE

Calcifying pseudoneoplasms of the neuraxis (CAPNON) are infrequent benign brain tumors. They demonstrate slow growth and are characterized by calcium deposits within the tumor tissue. CAPNONs are observed in the supratentorial compartment of the brain. Due to their rarity, increasing knowledge of CAPNONs is essential for accurate diagnosis and effective management of affected patients. This report describes a case of CAPNON diagnosed in a 56-year-old female and discusses the clinical, imaging, and histopathological findings of this rare entity.

CASE PRESENTATION

A 56-year-old female presented with a history of recurrent holocranial headache and dizziness, progressively worsening over the last month. Physical and neurological examinations revealed no evident abnormalities. Brain magnetic resonance imaging revealed a calcified and cystic mass, measuring 40 × 32 mm in the right frontal lobe. Complete excision of the mass was done. Histologically, the lesion was composed of glial tissue with abundant amorphous lamellar calcification and a myxoid matrix in the background. Concentric circular calcifications were observed with osseous metaplasia present in some areas. Palisading spindle to epithelioid cells was noted around the lesion. The final diagnosis was CAPNON. The postoperative course was uneventful, and one year of follow-up revealed no signs of recurrence.

DISCUSSION

CAPNON typically occurs in middle-aged adults and can present with variable symptoms depending on its location within the brain, including seizures, headaches, or neurological deficits. Surgical resection is considered the optimal treatment for CAPNON. Raising awareness and understanding of this rare entity is necessary for accurate diagnosis and management of patients affected by this condition.

摘要

引言与重要性

神经轴索钙化性假瘤(CAPNON)是一种罕见的良性脑肿瘤。它们生长缓慢,其特征是肿瘤组织内有钙沉积。CAPNON见于脑幕上腔。由于其罕见性,增加对CAPNON的了解对于准确诊断和有效治疗受影响患者至关重要。本报告描述了一例在一名56岁女性中诊断出的CAPNON病例,并讨论了这种罕见疾病的临床、影像学和组织病理学表现。

病例介绍

一名56岁女性有全颅反复头痛和头晕病史,在过去一个月中逐渐加重。体格检查和神经系统检查未发现明显异常。脑磁共振成像显示右侧额叶有一个40×32毫米的钙化囊性肿块。对该肿块进行了完整切除。组织学上,病变由胶质组织组成,背景中有大量无定形层状钙化和黏液样基质。观察到同心圆形钙化,部分区域有骨化生。病变周围可见栅栏状梭形至上皮样细胞。最终诊断为CAPNON。术后过程顺利,一年的随访未发现复发迹象。

讨论

CAPNON通常发生于中年成年人,根据其在脑内的位置不同可表现出不同症状,包括癫痫发作、头痛或神经功能缺损。手术切除被认为是CAPNON的最佳治疗方法。提高对这种罕见疾病的认识和理解对于准确诊断和治疗受此疾病影响的患者是必要的。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/d9ec/10428139/78f68f68b18f/gr1.jpg

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