Johnson Gabrielle W, Mian Ali Y, Dahiya Sonika, Rich Keith M, Chicoine Michael R, Limbrick David D
Departments of1Neurosurgery.
2Radiology, and.
J Neurosurg Case Lessons. 2022 Nov 14;4(20). doi: 10.3171/CASE22360.
Choroid plexus papillomas are benign tumors of the choroid plexus. Although typically focal, they can metastasize. Rarely, patients may present with numerous cystic lesions throughout the craniospinal axis.
The authors present three cases of pathologically confirmed fourth ventricular World Health Organization (WHO) grade 1 choroid plexus papillomas presenting with numerous cystic lesions throughout the craniospinal axis. Two cases were treated with only resection of the fourth ventricular mass; one was treated with a partial cyst fenestration. During follow-up, there was only mild interval growth of the cystic lesions over time, and all patients remained asymptomatic from their cystic lesions. The authors summarize five additional cases of cystic dissemination in the published literature and discuss hypotheses for the pathophysiology of this rare presentation.
Choroid plexus papillomas may present with numerous, widely disseminated cystic lesions within the craniospinal axis. Thus, the authors recommend preoperative and routine imaging of the entire neuroaxis in patients with choroid plexus tumors, regardless of WHO grade. Although the role of adjuvant therapy and cyst fenestration in the treatment of these lesions remains unclear, watchful waiting may be indicated, especially in asymptomatic patients, because the lesions often demonstrate slow, if any, growth over time.
脉络丛乳头状瘤是脉络丛的良性肿瘤。虽然通常为局限性，但它们可发生转移。罕见情况下，患者可能在整个颅脊髓轴出现大量囊性病变。
作者报告了3例经病理证实的世界卫生组织（WHO）1级第四脑室脉络丛乳头状瘤，这些病例在整个颅脊髓轴均出现大量囊性病变。2例仅接受了第四脑室肿块切除术；1例接受了部分囊肿开窗术。在随访期间，囊性病变随时间仅有轻度进展，所有患者的囊性病变均无症状。作者总结了已发表文献中另外5例囊性播散的病例，并讨论了这种罕见表现的病理生理学假说。
脉络丛乳头状瘤可能在颅脊髓轴内出现大量广泛播散的囊性病变。因此，作者建议，对于脉络丛肿瘤患者，无论WHO分级如何，术前均应对整个神经轴进行常规影像学检查。虽然辅助治疗和囊肿开窗术在这些病变治疗中的作用仍不明确，但可能需要密切观察，尤其是对于无症状患者，因为这些病变通常生长缓慢，甚至无生长。
