Department of Legal Medicine, Faculty of Medicine, University of Toyama, Toyama, Japan.
Department of Pathology, Toyama Prefectural Central Hospital, Toyama, Japan.
Neuropathology. 2022 Dec;42(6):548-554. doi: 10.1111/neup.12849. Epub 2022 Oct 9.
A 53-year-old man with a history of an untreated brain mass was taken to Toyama Prefectural Central Hospital by emergency transport. Computed tomography revealed an intracranial hypo-attenuated lesion exhibiting mass effect. Several calcified foci were observed around the lesion. His radiographical diagnosis was meningioma with calcification and edema. He suddenly showed tonic seizure after admission; therefore an emergency craniotomy was performed. However, he unfortunately died due to advanced cerebral edema. Microscopic findings of the surgically obtained materials were consistent with neurenteric cyst (NC). Intracranial hard masses were found adjacent to NCs, and the masses were composed of fibrous cartilage-like matrix with extensive linear calcification and the presence of surrounding round-to-oval epithelioid cells. Thus, calcifying pseudoneoplasm of the neuraxis (CAPNON) associated with NC was considered the most appropriate diagnosis of the present case. To the best of our knowledge, this is the first report of such a case. The present case suggests that delay of treatment might cause a poor outcome, at least in CAPNON associated with NC. Careful investigations, including the underlying pathology, may be essential when considering the etiology of CAPNON and its treatment strategies.
一位 53 岁男性，曾患有未经治疗的脑肿块，由急救车送往富山县中央医院。计算机断层扫描显示颅内低衰减病变，有肿块效应。病变周围观察到几个钙化灶。影像学诊断为伴有钙化和水肿的脑膜瘤。入院后他突然出现强直阵挛发作，因此进行了紧急开颅手术。但不幸的是，他因严重脑水肿死亡。从手术获得的标本的显微镜检查结果与神经肠囊肿（NC）一致。在 NC 附近发现颅内硬肿块，肿块由纤维软骨样基质组成，广泛线性钙化，并存在周围圆形至椭圆形上皮样细胞。因此，考虑到与 NC 相关的中枢神经系统钙化假性肿瘤（CAPNON）是最适合本病例的诊断。据我们所知，这是首例此类病例报告。本病例提示，至少在与 NC 相关的 CAPNON 中，治疗延迟可能导致不良预后。在考虑 CAPNON 的病因及其治疗策略时，仔细的调查，包括潜在的病理学，可能是必要的。
