Duchesne Mathilde, Nguyen Quoc Dat, Guyot Anne, Pommepuy Isabelle, Caire François, Labrousse François
Service d'anatomie pathologique, CHU Dupuytren, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France.
Service de neurochirurgie, CHU Dupuytren, 2, avenue Martin-Luther-King, 87042 Limoges cedex, France.
Ann Pathol. 2018 Dec;38(6):391-394. doi: 10.1016/j.annpat.2018.04.006. Epub 2018 May 30.
Calcifying pseudoneoplasms of the neuraxis (CAPNON) are rare lesions of the central nervous system. To date, about 60 cases have been reported in literature. We present a case that had the peculiarity to occur in a pregnant woman. At 32 weeks of gestation, a 26-year-old woman was hospitalized to explore nocturnal epigastralgia. During the hospitalisation, the patient presented generalised seizures. As an eclampsia had been suspected, a caesarean delivery was performed. Post-operatively, the patient harboured memory disorders and neuro-imaging explorations were done. They showed an intracerebral calcified mass located in the left frontal lobe and surrounded by an oedema. A complete surgical resection was performed. Histological examination of the surgical specimen showed a calcified tissue containing a fibrillary or granular material. A dense and hyalinised eosinophilic material focally surrounded the calcifications and contained regular fusiform cells of fibroblastic type. Foci of lipomatous and osseous metaplasia were present. Immunohistochemical staining for EMA and STAT6 was negative. There was no associated meningioangiomatosis nor tumour proliferation. Forty-five months after surgery, the patient did not present any seizures and had no sequelae. CAPNON are rare lesions occurring at any age. Their location in the central nervous system is ubiquitous and they can be intra or extra axial. The treatment is surgical and the prognosis excellent. CAPNON must be recognized and distinguished from the other calcified lesions, tumoural or non-tumoural, to avoid an inadequate and potentially harmful treatment.
神经轴索钙化性假瘤(CAPNON)是中枢神经系统的罕见病变。迄今为止,文献中已报道约60例。我们报告一例发生在孕妇身上的特殊病例。妊娠32周时,一名26岁女性因夜间上腹部疼痛入院检查。住院期间,患者出现全身性癫痫发作。由于怀疑子痫,遂行剖宫产。术后,患者存在记忆障碍,并进行了神经影像学检查。检查显示左额叶有一脑内钙化肿块,周围有水肿。进行了完整的手术切除。手术标本的组织学检查显示钙化组织含有纤维状或颗粒状物质。致密且玻璃样变的嗜酸性物质局灶性地围绕钙化灶,并含有成纤维细胞类型的规则梭形细胞。存在脂肪化生和骨化生灶。EMA和STAT6免疫组化染色均为阴性。无相关的脑膜血管外皮细胞瘤或肿瘤增殖。术后45个月,患者未再出现癫痫发作,也无后遗症。CAPNON是任何年龄均可发生的罕见病变。它们在中枢神经系统中的位置广泛,可位于轴内或轴外。治疗方法为手术,预后良好。必须识别CAPNON并将其与其他钙化性病变(肿瘤性或非肿瘤性)区分开来,以避免不适当且可能有害的治疗。