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FREDERIC B.ASKINMDWILLIAM H.WESTRAMD
First described in 1959, mesenchymal chondrosarcoma is a rare subtype of chondrosarcomas, making up only 3% to 9% of all chondrosarcomas.1 It arises from both skeletal and extraskeletal sites but has a predilection for the head and neck.1,2 Skeletal tumors outnumber extraskeletal tumors by a ratio of 2:1. Within the head and neck, skeletal sites most commonly include the maxilla and mandible; extraskeletal sites include the orbits and meninges. Most patients are in their second or third decade of life when the diagnosis is made; however, cases have been reported in infants and the elderly. There is a slight male predilection, with a ratio of 4:3. Mesenchymal chondrosarcoma of the maxilla has an aggressive local behavior as well as a high metastatic potential. There has recently been an increase in the number of reports of mesenchymal chondrosarcoma, presumably because of an increased knowledge of the tumor among clinicians and pathologists.3-6
Presenting symptoms of mesenchymal chondrosarcoma of the maxilla include swelling, pain, nasal obstruction, tooth mobility or loss, epistaxis, and diplopia. Symptoms relating to nerve compression are also common, being seen in approximately 25% of cases at presentation. Presenting symptoms last, on average, 54 weeks, with a range of 3 weeks to 10 years before diagnosis is made.7
Radiographically, the lesions are usually radiopaque when they are located in the maxilla, particularly when they extend into the maxillary antrum. When they involve the mandible, they are usually radiolucent, with indistinct, ragged borders. They also may show multiple areas of fine and coarse calcifications. On T1-weighted magnetic resonance imaging scans, the tumors show lower signal intensity compared with brain. On T2-weighted images, they have relatively high signal intensity. There is usually no tumor blush on angiography.5,8,9
Mesenchymal chondrosarcomas are biphasic tumors composed of abundant sheaths or clusters of undifferentiated rounded or spindled hyperchromatic cells interspersed with small nests of variably differentiated cartilaginous tissue in which calcification and areas of metaplastic bone formation may be observed. Immunoreactivity to vimentin and S100 protein is seen in areas with chondroid differentiation. Areas with hemangiopericytic architecture, as seen in this case, are characteristic and can lead to an incorrect diagnosis of hemangiopericytoma. Unlike mesenchymal chondrosarcomas, hemangiopericytomas do not demonstrate cartilaginous differentiation. Other tumor types that may enter the differential diagnosis include osteosarcoma, osteochondroma, atypical chondroma, fibrosarcoma, Ewing sarcoma, rhabdomyosarcoma, and synovial sarcoma. In order to demonstrate the presence of cartilaginous differentiation, multiple sections of the lesion may have to be examined.4,7,10-12
Mesenchymal chondrosarcoma is regarded as a high-grade tumor. Therapy for mesenchymal chondrosarcoma of the head and neck is derived from experience with relatively few cases. Radical surgical excision of the tumor is the treatment of choice. If any involvement of the orbit is suspected, orbital exenteration is also indicated. Chemotherapy, radiation therapy, or both can be used in an adjuvant role. Their efficacy in the treatment of these tumors, however, is unclear, as there is little long-term data on how these modalities can best be used. Chemotherapy and radiation therapy have been used to treat tumors that are not amenable to excision, recurrences, and metastases.3,7
Huvos et al6 divided a group of 13 patients with mesenchymal chondrosarcomas in different sites into those with well-differentiated hemangiopericytomatoid tumors (n = 8) and those with undifferentiated small-cell variants (n = 5). Five of the 8 patients with well-differentiated tumors had no evidence of disease after 8 to 49 months of follow-up. Although the treatment was not uniform, 4 of the 8 patients had received neoadjuvant and adjuvant chemotherapy. The two patients in the undifferentiated tumor group who were still alive had been treated with preoperative radiation and chemotherapy and postoperative chemotherapy.
The prognosis for mesenchymal chondrosarcoma is poor; however, a long clinical course is not unexpected. Nakashima et al5 reviewed the long-term follow-up of 23 patients with mesenchymal chondrosarcoma in any location. Seventeen of the patients had died of their disease 6 months to 23 years after presentation. The mean survival for this group was 6.7 years. Huvos and Marcove8 demonstrated a 5-year survival rate of 42% and a 10-year rate of 28% in a group of 35 patients with mesenchymal chondrosarcoma in any site. In a review by Takahashi et al,7 8 (42%) of 19 patients with mesenchymal chondrosarcoma of the maxilla died of the tumor, with an average survival time of 90 months.5,7,8
The 23 patients described had no distant metastases or unknown status at the time of presentation; however, one third of them had locoregional recurrence that developed up to 9 years after initial treatment. Metastases also developed in one third of cases up to 23 years after initial treatment. Approximately 80% of patients with locoregional recurrence later developed metastases. Only 1 patient with distant metastases did not have detectable locoregional recurrence at any time. In view of these numbers, it is obvious that long-term follow-up is necessary.3-5,8
Diagnosis Pathology Quiz Case 1. Arch Otolaryngol Head Neck Surg. 2002;128(10):1211–1212. doi:
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