Chondrosarcoma of the spine: a narrative review.

Chondrosarcoma is an uncommon primary bone tumor with an estimated incidence of 0.5 per 100,000 patient-years. Primary chondrosarcoma of the mobile spine and sacrum cumulatively account for less than 20% of all cases, most .commonly causing patients to present with focal pain with or without radiculopathy, or myelopathy secondary to neural element compression. Because of the rarity, patients benefit from multidisciplinary care at academic tertiary-care centers. Current standard-of-care consists of en bloc surgical resection with negative margins; for high grade lesions adjuvant focused radiation with ≥60 gray equivalents is taking an increased role in improving local control. Prognosis is dictated by lesion grade at the time of resection. Several groups have put forth survival calculators and epidemiological evidence suggests prognosis is quite good for lesions receiving R0 resection. Future efforts will be focused on identifying potential chemotherapeutic adjuvants and refining radiation treatments as a means of improving local control.

Chordoma of the sacrum and mobile spine: a narrative review.

Chordoma is a notochord-derived primary tumor of the skull base and vertebral column known to affect 0.08 to 0.5 per 100,000 persons worldwide. Patients commonly present with mechanical, midline pain with or without radicular features secondary to nerve root compression. Management of these lesions has classically revolved around oncologic resection, defined by en bloc resection of the lesion with negative margins as this was found to significantly improve both local control and overall survival. With advancement in radiation modalities, namely the increased availability of focused photon therapy and proton beam radiation, high-dose (>50 Gy) neoadjuvant or adjuvant radiotherapy is also becoming a standard of care. At present chemotherapy does not appear to have a role, but ongoing investigations into the ontogeny and molecular pathophysiology of chordoma promise to identify therapeutic targets that may further alter this paradigm. In this narrative review we describe the epidemiology, histopathology, diagnosis, and treatment of chordoma.

Recurrent Extradural Myxopapillary Ependymoma With Oligometastatic Spread.

Myxopapillary ependymomas are a slow-growing, grade I type glial tumor in the lumbosacral region. More rarely, they can present as extradural, subcutaneous sacrococcygeal, or perisacral masses, and it is under these circumstances that they are more likely to spread. Here, we report the presentation of a sacrococcygeal mass in patient that was initially resected confirming extradural myxopapillary ependymoma. At initial resection, multiple small pulmonary nodules were detected. This mass recurred 2 years later at the resection site with an interval increase in the previously imaged pulmonary nodules. Resection of both the post-sacral mass and largest lung metastasis confirmed recurrent myxopapillary ependymoma with oligometastatic spread. Because these tumors are rare, with extradural presentation being even more infrequent, to this date there are no definitive therapeutic guidelines for initial treatment and continued surveillance. For myxopapillary ependymoma, current standard of care is first-line maximal surgical resection with or without postoperative radiotherapy depending on the extent of disease and extent of resection. However, there remains insufficient evidence on the role of radiotherapy to oligometastatic foci in providing any further survival benefit or extending time to recurrence. Thus, prospective studies assessing the role of upfront treatment of oligometastases with local resection and adjuvant radiotherapy are needed for improved understanding of extradural myxopapillary ependymoma.