Chondrosarcoma (CHS) is a malignant neoplasm characterized by the formation of cartilaginous matrix by neoplastic cells, with a high propensity for local recurrences. Head and neck CHS is rare, accounting for less than 12% of all cases of CHS, usually affecting the maxilla. The majority of affected patients are in the fourth decade of life, with a slight predilection for male patients. A painless swelling is commonly the most frequent complaint. Surgery with wide en-bloc resection is the preferred treatment for CHS; radiotherapy and chemotherapy are usually palliative options. Owing to its rarity, there are few clinical series evaluating the biological behaviour of head and neck CHS. The aim of this study is to analyse the clinicopathological characteristics of head and neck CHS by reporting 3 new cases of this neoplasia affecting the jaw bones and reviewing the clinical series previously published in the English literature.
Chondrosarcoma (CHS) is classified by the World Health Organization as a malignant tumour characterized by the formation of cartilage, but not of bone, by tumour cells . CHS accounts for 10–20% of all primary malignant bone tumours and, excluding multiple myeloma, it represents the second most common primary bone malignancy after osteosarcoma .
Head and neck CHS is unusual, ranging from 5% to 12% of all cases of CHS and despite the fact that virtually all anatomical location of the craniofacial skeleton can give rise to the neoplasia, excluding the larynx, it has a predilection for the anterior portion of the maxilla and sinonasal structures . The mandibular symphysis, coronoid, and condylar processes are the most affected sites in the mandible, which may be explained partly by embryological issues, once these mandibular regions tend to ossify endochondrally .
Owing to the rarity of head and neck CHS, few sizeable clinical series describing its biological behaviour have been published. The aim of this study is to analyse the clinicopathological characteristics of head and neck CHS by reporting 3 new cases of this neoplasia affecting the jaw bones and reviewing all the previously published clinical series in the English literature.
Case report 1
A 44-year-old female patient referred herself to a dentist presenting a small, firm swelling with normally coloured mucosa between the left lateral incisor and left canine in the maxilla ( Fig. 1 A ). Panoramic and periapical radiographs revealed displacement of the dental roots, a hazy radiopaque area in the anterior region of the maxilla and widening of the periodontal ligament space between the teeth involved ( Fig. 1 B). An incisional biopsy was performed and the diagnosis of fibrous dysplasia was made, leading to an erroneous osteoplasty.