Delayed Manifestation of Sacral Clear Cell Meningioma Distent Metastasis or Multifocal Disease?

Abstract

Study design: The authors report on a clear cell meningioma (CCM) of the sacrum revealed 17 years after a removal of a spinal lesion. A review of 29 spinal CCM previously reported was done. Objective: To focus on clinical and biological behaviour of CCM. Summary of background data: CCM is a rare subtype characterrized by its inordinately aggressive clinical course despite its benign appearance. The tumour shows proclivity for the cerebellopontine angle and cauda equine region. Recurrence and metastasis have been commonly reported. Method: A 26 year-old man presented with low back pain related to a lytic lesion of the sacrum. He had a history of an intradural extramedullary meningioma of the cervical spine removed when he was 9 year-old. CT scan revealed a sacral osteolytic lesion with anterior and lateral extension. Open biopsy revealed a clear cell meningioma. That was the same pattern of the tumour removed 17 years before. Results: Our patient was managed conservatively by palliative radiation therapy. At 2 years follow-up, he experienced improvement of pain and walking. Conclusion: CCM is an aggressive tumour with a high risk of metastases through cerebrospinal fluid. A follow up with spinal MRI should be performed.