Domecq Laplace Lucila, Ruella Mauro, Caffaratti Guido, Villamil Facundo, Monsalve Martin, Alcorta Santiago Condomi, Cervio Andres
Department of Neurosurgery, Fleni, Buenos Aires, Argentina.
Department of Neurosurgery, Fleni, Buenos Aires, Argentina.
World Neurosurg. 2022 Nov;167:e423-e431. doi: 10.1016/j.wneu.2022.08.022. Epub 2022 Aug 11.
Calcifying pseudoneoplasm of the neuraxis (CAPNON) is an extremely rare entity with fewer than 150 cases reported in the literature and mostly with a supratentorial or spinal location. Posterior fossa CAPNON has been reported scarcely, and association with perilesional edema is a topic not yet approached which might play a significant role in treatment decision and clinical progression. Our objective is to report, to our knowledge, the first series of 3 posterior fossa CAPNON surgically treated in a single institution and assess features that help provide a systematic approach to diagnosis and timely treatment.
This was a monocentric, retrospective study of surgical patients diagnosed with a posterior fossa CAPNON in the last 5 years. A thorough bibliographic research was conducted.
Three patients were included. Locations involved IV ventricle, right cerebellopontine angle with extension to foramen magnum, and cerebellar vermis. Two of them presented with symptoms linked to acute hydrocephalus, and the other one presented with progressive cranial nerve palsy and brainstem compression signs. The 3 of them showed radiological signs of perilesional edema on their preoperative magnetic resonance imaging. Gross total resection was accomplished in one case, with near and subtotal resections in the others. There were no complications. The outcome was favorable in all cases.
It is essential to contemplate this infrequent diagnosis in cases of calcified lesions involving the posterior fossa. When symptoms manifest, surgery should be considered. Perilesional edema could be associated with symptomatic progression and hence a sign suggesting the need for surgical treatment.
神经轴索钙化性假瘤（CAPNON）是一种极为罕见的疾病，文献报道的病例不足150例，且大多位于幕上或脊髓。后颅窝CAPNON的报道极为罕见，其与病灶周围水肿的关系尚未有研究，而这可能在治疗决策和临床进展中起重要作用。我们的目的是据我们所知报告在单一机构手术治疗的首例3例后颅窝CAPNON病例系列，并评估有助于提供系统诊断方法和及时治疗的特征。
这是一项对过去5年中诊断为后颅窝CAPNON的手术患者进行的单中心回顾性研究。进行了全面的文献检索。
纳入3例患者。病变部位包括第四脑室、延伸至枕骨大孔的右侧小脑脑桥角以及小脑蚓部。其中2例出现与急性脑积水相关的症状，另一例出现进行性颅神经麻痹和脑干受压体征。3例患者术前磁共振成像均显示病灶周围水肿的影像学征象。1例实现了全切除，其他患者为近全切除和次全切除。无并发症发生。所有病例预后良好。
对于累及后颅窝的钙化性病变病例，必须考虑到这种罕见的诊断。当出现症状时，应考虑手术治疗。病灶周围水肿可能与症状进展相关，因此是提示需要手术治疗的一个征象。
