Huang Jiahua, Ghent Finn, Rodriguez Michael, Davies Mark
Department of Neurosurgery, St George Public Hospital, Gray Street, Australia.
Department of Kogarah, Pathology, Douglass Hanly Moir Pathology, Macquarie Medical Centre, Macquarie Park, New South Wales, Australia.
Surg Neurol Int. 2020 Aug 15;11:249. doi: 10.25259/SNI_386_2020. eCollection 2020.
Calcifying pseudoneoplasm of the neuraxis (CAPNON) is a rare entity which can occur at intracranial and spinal locations. Clinical presentation is due to local mass effect rather than tissue infiltration. Lesions causing significant symptoms or are showing radiological progression require surgical resection. Maximal surgical resection is considered curative for this non-neoplastic entity with only two cases of recurrence reported in the literature. Cranial nerve involvement is extremely rare and the presenting neurological deficit is unlikely to improve even with surgical intervention.
We describe a case of CAPNON at the right posterior clinoid process with involvement of the right oculomotor nerve in a 38-year-old male. Computed tomography demonstrated an amorphous mass which had intermediate to low T1 and T2 signal on magnetic resonance imaging. The oculomotor nerve was compressed with sign of atrophy. The patient underwent maximal surgical debulking for progressive symptoms of worsening pain and ophthalmoplegia. Postoperatively, the patient's symptoms were stable but did not improve.
Preoperative diagnosis of CAPNON is difficult due to its rarity and nonspecific clinical and radiological findings. Surgical resection is considered in cases with worsening symptoms, progression on serial imaging, or uncertain diagnosis. Relatively inaccessible lesions with little or no clinical symptoms can be observed.
神经轴钙化性假瘤（CAPNON）是一种罕见的病变，可发生于颅内和脊柱部位。临床表现是由于局部占位效应而非组织浸润。引起明显症状或显示影像学进展的病变需要手术切除。对于这种非肿瘤性病变，最大程度的手术切除被认为是治愈性的，文献中仅报道了2例复发病例。颅神经受累极为罕见，即使进行手术干预，出现的神经功能缺损也不太可能改善。
我们描述了一例38岁男性患者，其右侧后床突出现CAPNON并累及右侧动眼神经。计算机断层扫描显示一个无定形肿块，在磁共振成像上T1和T2信号为中等至低信号。动眼神经受压并有萎缩迹象。该患者因疼痛加重和眼肌麻痹的进行性症状接受了最大程度的手术减瘤。术后，患者症状稳定但未改善。
由于CAPNON罕见且临床和影像学表现不具特异性，术前诊断困难。对于症状恶化、系列影像学检查有进展或诊断不确定的病例，考虑手术切除。对于相对难以接近且几乎没有临床症状的病变，可以进行观察。
