Chordomas and chondrosarcomas of the skull base: treatment and outcome analysis in a consecutive case series of 24 patients

Abstract

Background We present our 9-year consecutive case series of skull base chordomas and chondrosarcomas from a UK tertiary referral centre, discussing treatments offered and outcomes. This was carried out to improve understanding around current treatment and to better inform the management of future patients. Methods Consecutive case series over a 9-year period (2007–2016). Retrospective data analysis from the electronic skull base multidisciplinary team database and the digital patient records at a UK tertiary referral centre Results Twenty-four patients were identified (11 chordomas, 13 chondrosarcomas, mean age 52). Nineteen had proton beam therapy (PBT) postoperatively; two had intensity-modulated radiotherapy; two had no further treatment. One patient was lost to follow-up. All chordomas were resected via a transnasal endoscopic approach. Of the 19 patients undergoing resection with PBT, 13 were disease free at latest follow-up, and six patients had local recurrence, of which two died (mean follow up 7.4 years). Of the three patients treated with surgery then IMRT/TomoTherapy, one died 4 years post-treatment, and the other two are alive after 4 and 5 years of follow-up respectively. Of the two patients treated with surgery alone, one was lost to follow-up, and the other is alive after more than 8 years. Chondrosarcoma 5-year survival was 91.6%, and chordoma 4-year survival was 75%. Conclusion Skull base chordomas and chondrosarcomas can be challenging to resect, and most cases require adjuvant therapy to achieve control. Where complete resection is not possible, it is critical to undertake sufficient resection to permit high-dose radiation.

Risk Factors for Radiation Necrosis and Local Recurrence after Proton Beam Therapy for Skull Base Chordoma or Chondrosarcoma

“…For three-fraction SRS for brain metastases, normal brain tissue V18 < 30 cc and V23 < 7 cc were associated with <10% risk of brain necrosis. Finally, we evaluate the results of this study based on the latest reports [14,[21][22][23]31,[46][47][48][49][50][51][52][53][54][55][56][57][58][59][60][61]. According to the latest reports, the 5-year local recurrence rate for chordoma was about 15 to 35%, while for chondrosarcoma, it was less than 10%.…”

Clinical Characteristics and Prognostic Risk Factors of Parasellar Chondrosarcoma

“…It has also been reported that the cavernous carotid artery [ 14 ], the internal carotid artery [ 40 ], and the cavernous carotid artery [ 14 ] could be surrounded by parasellar chondrosarcoma. Generally, intracranial chondrosarcoma is difficult to resect completely and often requires adjuvant treatment for complete control [ 41 ]. Tumors with these preoperative characteristics greatly increase the difficulty of total resection of the parasellar chondrosarcoma.…”

Challenges in Diagnosing Chordoma (Skull Base Tumors)

“…Chordoma and chondroid chordoma both are immune positive for epithelial markers cytokeratin (CK) and epithelial membrane antigen (EMA), though chondrosarcoma is negative for both [28]. Chordoma show up comparable to fetal notochord on both light and electron microscopy and are immune-histochemically and ultrastructurally similar.…”