Jayawardena Dinali, Yates Joseph, Clarke Enrico, Sinclair Georges
Department of Oncology, University Hospital Southampton, Southampton, United Kingdom.
Department of Histopathology, University Hospital Southampton, Southampton, United Kingdom.
Surg Neurol Int. 2025 May 16;16:182. doi: 10.25259/SNI_190_2025. eCollection 2025.
Biologically and morphologically distinct from other ependymomas, myxopapillary ependymomas (MPEs) are rare, slow-growing glial tumors originating predominantly from the conus medullaris, cauda equina, or filum terminale. Gross total resection is the standard of care for primary MPE. Nevertheless, despite maximal resection, the risk of recurrence, usually within the neural axis, remains high. However, extra-neural metastases can also occur. Due to the rarity of the entity, there is a lack of consensus on the management of recurrences and extra-neural metastatic disease. We present a case report and literature review of this rare ependymal tumor.
We describe a case of a male patient with MPE who developed multiple recurrences, treated with numerous surgical resections, radiotherapy, and salvage chemotherapy before eventually developing extra-neural metastatic disease to lungs, abdomen, and lymph nodes 37 years after initial diagnosis. A biopsy of an axillary lymph node confirmed histomorphology comparable to the primary histology.
To our knowledge, there are <30 cases of extra-craniospinal metastatic MPE reported since 1955. Consequently, there is no major consensus on the treatment of extra-neural metastatic MPE. Case reports and series remain of utter importance to share experience and help customize management. From this angle, surgery, and radiotherapy are still used in the face of central nervous system recurrence and "limited" extra-neural spread, depending on the patterns of invasion. Chemotherapy has shown a modest effect so far; however, positive outcomes from targeted agents and immunotherapy (alone or combined) have been reported, which warrants further exploration.
黏液乳头型室管膜瘤（MPE）在生物学和形态学上与其他室管膜瘤不同，是一种罕见的、生长缓慢的神经胶质瘤，主要起源于脊髓圆锥、马尾或终丝。根治性手术切除是原发性MPE的标准治疗方法。然而，尽管进行了最大程度的切除，复发风险仍然很高，通常发生在神经轴内。此外，也可能发生神经外转移。由于该疾病罕见，对于复发和神经外转移疾病的管理缺乏共识。我们报告一例这种罕见的室管膜瘤病例并进行文献综述。
我们描述了一名患有MPE的男性患者，该患者多次复发，在初次诊断37年后最终发生肺、腹部和淋巴结的神经外转移之前，接受了多次手术切除、放疗和挽救性化疗。腋窝淋巴结活检证实组织形态学与原发组织学相似。
据我们所知，自1955年以来，报告的颅外脊髓转移性MPE病例<30例。因此，对于神经外转移性MPE的治疗没有主要共识。病例报告和系列研究对于分享经验和帮助定制治疗方案仍然非常重要。从这个角度来看，面对中枢神经系统复发和“有限”的神经外扩散，根据侵袭模式，手术和放疗仍然被使用。化疗迄今为止显示出一定效果；然而，已经报道了靶向药物和免疫疗法（单独或联合使用）的阳性结果，这值得进一步探索。
