Skull Base Chordomas and Chondrosarcomas

Abstract

Skull base chordomas account for less than 0.2% and chondrosarcomas for less than 0.15% of all intracranial tumors. Although their clinical and imaging presentations are similar, they derive from different origins. Chordomas arise from embryonic remnants of the primitive notochord and chondrosarcomas from primitive mesenchymal cells or from the embryonic rest of the cranial cartilaginous matrix. Both entities are characterized by infiltration and destruction of the surrounding bone and soft tissue and a high locoregional recurrence rate. Chondrosarcomas, when treated with similar complex strategies, display a much better prognosis than chordomas. The overall survival is approximately 65% for chordomas and 80% for chondrosarcomas at 5 years and 30 and 50%, respectively, at 10 years. Chordomas are divided into the following 3 histological types: classical (conventional), chondroid, and dedifferentiated. Chondrosarcomas have conventional, mesenchymal, clear cell, and dedifferentiated subgroups. Both tumor entities often present with nonspe-cific symptoms, and headaches are the most reported initial symptom. Computed tomography and magnetic resonance imaging are required to determine the tumor localization and the extent of tumor growth. The treatment philosophy is to maximize tumor resection, minimize morbidity, and preserve function. Neurosurgical approaches commonly used for the resection of intracranial chordomas and chondrosarcomas are transsphenoidal, transbasal, cranio-orbitozygomatic, transzygomatic extended middle fossa, transcondylar, and transmaxillary approaches. Chordomas and chondrosarcomas are not sensitive to chemotherapy and there are no approved drugs for their treatment. The present treatment concept is a combination of surgical resection with a maximal excision and preserving patients' quality of life by adjuvant radiotherapy for both chordomas and chondrosarcomas.

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

“…It is well established that complete resection of skull base chordomas and chondrosarcomas is difficult, and, even in cases with good macroscopic clearance, there may be microscopic tumour cells left behind. This is demonstrated by the high recurrence rate in our series, particularly in the chordoma cohort, and this is consistent with current literature [17]. As a result, many patients can be offered higher dose adjuvant radiotherapy which provided the best chance of local control.…”

Primary Skull Base Chondrosarcomas: A Systematic Review

“…As described by Feuvret et al [ 36 ], primary SBCs may also invade the anterior fossa, mostly causing vision impairment and hypopituitarism by directly compressing the optic apparatus or the pituitary stalk. However, such symptoms are non-specific and may also occur in patients with skull base chordomas and other sarcomas [ 8 , 56 , 57 ]. Likewise, radiological features of chordomas and SBCs may overlap, with similar localization and bone destructive patterns [ 58 ].…”

“…While low-grade SBCs may be indolent and slow-growing, poorly differentiated neoplasms are highly invasive, destructive, and may metastasize. Symptoms derive from the direct compression of cranial nerves and neurovascular structures at the base of the skull, with headache and diplopia being the most frequent [ 5 , 8 ]. Computer tomography (CT) scans can evaluate bone invasion and destruction, but magnetic resonance imaging (MRI) provides better delineation of soft tissue involvement in skull base chondromas [ 8 , 9 , 10 ].…”

“…Symptoms derive from the direct compression of cranial nerves and neurovascular structures at the base of the skull, with headache and diplopia being the most frequent [ 5 , 8 ]. Computer tomography (CT) scans can evaluate bone invasion and destruction, but magnetic resonance imaging (MRI) provides better delineation of soft tissue involvement in skull base chondromas [ 8 , 9 , 10 ]. The main goals of surgery are tissue diagnosis and maximal safe resection, in order to prevent neurological deterioration, optimize conditions for dose-escalated adjuvant radiotherapy, and improve survival.…”

Higher cMET dependence of sacral compared to clival chordoma cells: contributing to a better understanding of cMET in chordoma

“…Chordomas are rare, malignant, locally destructive, slowly growing bone tumors that show an invasive growth behavior; prognosis is poor with a median survival of seven years after diagnosis. Current treatment concepts include surgical en-bloc resection with negative margins and high-dose radiation therapy as well as chemotherapy for the extremely rare dedifferentiated chordomas 1 , 2 . Several authors have suggested an interaction between hepatocyte growth factor (HGF)/cMET and epidermal growth factor receptor (EGFR) signalling 3 , 4 .…”