Primary Intracranial Myxoid Chondrosarcoma: Report of a Case and Review of the Literature

Abstract

The authors present a case of primary intracranial extraosseous myxoid chondrosarcoma without any attachment to the cranium or the meninges. The clinical and radiological findings of the primary intraparenchymal tumor are described with a review of the literature concerning cranial and intracranial myxoid chondrosarcoma.

Intracranial Extraskeletal Myxoid Chondrosarcoma : Case Report and Literature Review

“…In six of the eight patients diagnosed with this tumor to date, including our current patient, the tumors were completely removed by surgical excision2,5,8,13,17). Fortunately, these tumors had good dissection margins from the surrounding tissue, and tumor adhesions were not severe13).…”

“…Intracranial extraskeletal myxoid chondrosarcomas are extremely rare, with only seven cases previously reported to date2,5,8,13-15,17). A summary of these previous patients, including imaging results, the surgical extent of the tumor, postoperative radiation treatment, and patient outcomes is shown in Table 1.…”

“…Since its first description in 1972, only seven intracranial extraskeletal myxoid chondrosarcoma tumors have been reported2,4,5,8,13-15,17). We describe a patient with an intracranial myxoid chondrosarcoma originating from the choroid plexus of the left lateral ventricle.…”

Primary intracranial extraskeletal myxoid chondrosarcoma

“…The manuscripts were reviewed and their bib liographies scanned for additional articles. Nine previously reported cases of intracranial extraskeletal myxoid chondrosarcoma were found [6][7][8][10][11][12][13][14][15]. Their features are reported in Table 2.…”

“…Ultrastructural and molecular studies suggested that skeletal myxoid chondrosarcomas and EMC are two separate entities rather than the same entity arising in two different locations: detection of a EWS-CHN gene fusion RNA resulting from the t (9;22) mutation would be highly specific to EMC [5,6]. EMC is thought to be a tumour of intermediate malignancy, but with a supposed better prognosis than other types of chondrosarcomas [7,8]. Tumour size, cellular density, presence of anaplasia or rhabdoid features, high mitotic activity, and high Ki67 have been reported to be adverse pathological prognostic factors, but the rarity of intracranial locations does not allow for validation of those factors as significant prognostic factors for survival (Table 1) [9].…”