Extraspinal ependymomas are rare. The majority occur in the sacrococcygeal region. The subcutaneous variety accounts for approximately two thirds of cases, which are commonly misdiagnosed as a pilonidal cyst or sinus. Treatment is complete surgical resection. The role of coccygectomy is controversial. Adjuvant radiotherapy is of benefit to those with an incompletely excised tumour. Up to 20% metastasise, chiefly to the inguinal lymph glands, but pulmonary metastases are also reported. Palliative chemotherapy has not been shown to be of any benefit. Long term follow-up is important as metastases can occur up to 20 years after initial presentation. We report a 37-year-old woman with a subcutaneous sacrococcygeal ependymoma with iliac lymph nodal metastasis at presentation.

Lumbosacral ependymomas: a review of the management of intradural and extradural tumors. Neurosurg Focus. 2003 Nov 15;15(5):E13.

OBJECT: The goal of this study was to review the management of intra- and extradural ependymomas. Spinal ependymomas most commonly occur as intramedullary tumors throughout the spinal axis. In the lumbosacral region, ependymomas are most commonly associated with the conus medullaris and cauda equina, but can also occur extradurally in the sacrum, presacral tissues, or subcutaneous tissues over the sacrum. These two tumor locations produce different management concerns. Intradural ependymomas, especially those in the lumbosacral region, are now recognized for their potential to spread throughout the central nervous system (CNS), whereas extradural tumors elicit more concern for their association with extraneural metastases. METHODS: The authors have reviewed the literature regarding both of these distinct tumors and have summarized recommendations for the management of intra- and extradural lumbosacral ependymomas. For both tumors, it appears that gross-total resection is the treatment of choice when feasible. The role of radiation therapy has not been adequately studied for either tumor location, but most clinicians use this modality in patients with subtotal resection of intradural ependymomas, local recurrence, or CNS dissemination. Data supporting the use of radiation therapy for extradural ependymomas are lacking. There does not appear to be a significant role for chemotherapy in either tumor location. CONCLUSIONS: Despite the risk for local recurrence and CNS dissemination, the prognosis for intradural lumbosacral ependymomas is good, with a greater than 90% 10-year patient survival in most series. The prognosis for extradural ependymomas does not appear to be as good. Much depends on extradural tumor location, however; the outlook is better for dorsal sacral tumors than presacral tumors.