Intracranial chondrosarcoma: review of the literature and report of 15 cases

Abstract

The available data in the literature (177 cases), two current clinical patients, and cases which occurred in The Netherlands (13) were reviewed concerning the clinical presentation, pathological features, radiological data, and treatment options of chondrosarcoma of the cranial base. The mean age of patients was 37 years, the male/female ratio 1: 1.1. The most frequent complaints were diplopia with oculomotor disorders (51%), headache (31%), and decreased hearing, dizziness, and tinnitus with statoacusticus dysfunction (21%). The mean duration of symptoms before diagnosis was 27 months. The chondrosarcomas were located in the petrosal bone in 37% (47 cases), in the occipital bone and clivus in 23% (30 cases), in the sphenoïd bone in 20% (25 cases) and to a lesser extent in frontal, ethmoïdal, and parietal bones (14%). In 6% (eight cases) the primary location was in dural tissue. Radiological examinations showed bone destruction and variable calcification (CT), involvement of neuronal and vascular structures (MRI), and mostly hypovascularity on angiography. On histological examination 51% of tumours were classified as grade I, 11% grade II, 30% mesenchymal, and 8% myxoïd. The mesenchymal type was the most malignant as illustrated by a strong tendency to intradural and cerebral growth and possibly occurrence in younger age groups. The treatment of choice until recently was surgery because of the critical location and local aggressive nature. Regrowth of tumour after surgery occurred in 53% of the patients (average after 32 months). Charged particle irradiation gave a five year survival of 83-94% and a local control rate of 78%-91%. Both in surgery and radiotherapy there is treatment related morbidity and mortality that should be considered when oVering these therapies. Recent promising results imply that charged particle radiotherapy, in combination with surgery, may be the therapeutical choice of the future. (J Neurol Neurosurg Psychiatry 1998;65:88-92)

Classical intracranial chondrosarcoma: A case report

“…Intracranial chondrosarcoma typically affects patients in the fourth and fifth decades of life, with no gender preference (7). The clinical presentation of chondrosarcomas has been extensively reported in the literature (8,9). Generally, patients present with an extensive history of headaches and symptoms associated with increased intracranial pressure.…”

“…In a review of 192 chondrosarcoma cases by Chandler et al (12), 62% were of the classical subtype, while the mesenchymal and myxoid types accounted for 30 and 8% of cases, respectively. Korten et al (8) reviewed 192 cases of chondrosarcoma and reported that in general, the mesenchymal type is malignant and occasionally spreads to distant areas, while the classical subtype is the most benign of the three subtypes. In this study, a case of classical type intracranial chondrosarcoma that occurred in the left frontal region of the skull was presented, which has rarely been reported in the literature to date.…”

The diagnosis and management of parasellar tumours of the pituitary

“…Chondrosarcomas arise off the midline at the suture line, unlike chordomas that arise from the clivus in the midline. Radiological examination almost always reveals bone destruction and variable degrees of calcification on CT, involvement of the neural and vascular structures on MRI, and mostly hypovascularity on angiography (Korten et al 1998). Occasionally, cyst-like hypodense centres secondary to necrosis can be found (Cohen-Gadol et al 2003).…”

“…6). These are slowly growing tumours presenting with visual complaints, mainly diplopia, whereas a third of patients complain of headache (Korten et al 1998). Less common presentations include dizziness, tinnitus, facial sensory deficits, ataxia and hemiparesis.…”

“…Less than 200 cases of intracranial chondrosarcomas have been described (Korten et al 1998). These malignant tumours (WHO grades II-III) occur more commonly in the axial part of the skeleton, representing less than 5% of skull base tumours with w75% arising in the parasellar region (Kunanandam & Gooding 1995).…”