Chordoma and chondrosarcoma: Similar, but quite different, skull base tumors

Abstract

BACKGROUND. Chordoma and chondrosarcoma of the skull base are frequently amalgamated because of similar anatomic location, clinical presentation, and radiologic findings. The chondroid chordoma variant has been reported to carry a better prognosis. The objective of the current study was to investigate the distinctions between these 3 entities. METHODS.The data concerning 109 patients with chordoma, chondroid chordoma, and chondrosarcoma who were treated by a single surgeon with maximum surgical resection and frequently by adjunct proton beam radiotherapy between 1990 and 2006 were analyzed retrospectively. Pathologic distinction was established by cytokeratin and epithelial membrane antigen staining. Clinical, radiologic, pathologic, and cytogenetic studies were analyzed in relation to disease recurrence and death.RESULTS. The average follow-up was 48 AE 37.5 months (range, 1-191 months).There were no reliable distinguishing clinical or radiologic features noted between the groups. Chondrosarcoma patients had a significantly better outcome compared with chordoma patients with regard to survival and recurrence-free survival (P 5 .028 and P < .001, respectively), whereas patients with chondroid chordoma had a poor outcome similar to chordoma patients with regard to survival and recurrence-free survival (P 5 .337 and P 5 .906, respectively). CONCLUSIONS.Chordoma and chondrosarcoma differ with regard to their origin and histology, and differ markedly with regard to outcome. Chondroid chordomas behave in a manner that is clinically similar to chordomas, with the same prognosis. Both chordoma types demonstrate an aggressive clinical course and poor outcome after disease recurrence. The optimal treatment for all groups of patients involves radical surgical resection followed by high-dose radiotherapy in patients with chordomas. Radiotherapy may not be necessary in patients with low-grade chondrosarcoma.

Chordoma

“…Because of the clinical and radiologic features, location, means of treatment and sometimes even the histologically masquerading appearance, skull base chordoma is confused with low-grade chondrosarcoma, and to date the two are frequently lumped together. However, they are totally different disease entities with disparate outcomes [4]. I believe that the diagnosis of chordoma should only be accepted with positive immunohistochemical markers demonstrating its epithelial origin (cytokeratin, EMA and Brachyury) [2].…”

Synchronous Infrasellar and Cervical Chondroid Chordoma: A Case Report and Review of the Literature

“…This improves the survival rates and decreases rate of recurrences to a 25% [1,19,20]. Chemotherapy is not effective [9,20,21].…”

Surgical management of skull base tumors

“…Local bony destruction and invasion can make oncologic excision very difficult. 41 Because these tumors are relatively insensitive to radiation and chemotherapy, surgery is the primary treatment modality.…”

“…43 Chondrosarcomas are much less aggressive and these patients have a higher rate of recurrence-free survival. 41 …”