Indolent but invasive chondroid malignancies that can form in the skull base.
The most common sites for chondrosarcoma to grow are the pelvis and shoulder, along with the superior metaphysial and diaphysial regions of the arms and legs.
However, chondrosarcoma may occur in any bone, and are sometimes found in the skull, particularly at its base.
Standard management of chondrosarcoma involves surgical resection and adjuvant radiation therapy.
Endonasal endoscopic approach for resection of petroclival chondrosarcomas between 2010 and 2014, 8 patients (4 men and 4 women) underwent endonasal endoscopic operations to resect petroclival chondrosarcomas at 2 institutions. The patients' mean age was 44.8 years (range 30-64 years). One of the patients had previously undergone radiation therapy and another a staged craniotomy. Using volumetric software, an independent neuroradiologist assessed the extent of the resections on MRI scans taken immediately after surgery and at the 3-month follow-up. Immediate complications and control of symptoms were also recorded. In addition, the authors reviewed the current literature on surgical treatment of chondrosarcoma.
The mean preoperative tumor diameter and volume were 3.4 cm and 9.8 cm(3), respectively. Six patients presented with cranial neuropathies. Endonasal endoscopic surgery achieved > 95% resection in 5 of the 8 patients and < 95% resection in the remaining 3 patients. One of the 6 neuropathies resolved, and the remaining 5 partially improved. One instance of postoperative cerebrospinal fluid leakage required a reoperation for repair; no other complications associated with these operations were observed. All of the patients underwent adjuvant radiotherapy.
According to the experience of Moussazadeh et al. the endoscopic endonasal route is a safe and effective approach for the resection of appropriately selected petroclival chondrosarcomas 1).
Parafalcine chondrosarcoma is extremely rare, and may be difficult to differentiate preoperatively from falx meningioma. An 18-year-old woman presented with a parafalcine chondrosarcoma incidentally detected as a small lesion 2 years before admission, suggesting falx meningioma. Brain computed tomography and magnetic resonance imaging just before admission revealed the parafalcine lesion had increased by about nine times in volume during the last 2 years. Single-photon emission computed tomography (SPECT) after intravenous administration of both thallium-201 chloride 2).
The potential malignant progression of enchondroma into a secondary chondrosarcoma is a well-known fact. Nevertheless, chondrosarcoma located at the skull base in patients with Maffuci syndrome is a very rare condition, with only 18 cases reported in the literature.
Beer-Furlan et al. report 2 other cases successfully treated through an expanded endoscopic endonasal approach and discuss the condition based on the literature review.
Skull base chondrosarcoma associated with Maffucci syndrome is a rare condition. The disease cannot be cured, therefore surgical treatment should be performed in symptomatic patients aiming for maximal tumor resection with function preservation. The endoscopic endonasal approach is a safe and reliable alternative for the management of these tumors 3).