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Background. Chordomas are uncommon primary malignant tumors of bone that typically occur in the axial skeleton including the sacrum, vertebrae, and skull base. The base of skull tumors usually are not amenable to complete surgical resection, and most require post-operative radiotherapy. The natural history of skull base chordoma is typified by slow locally invasive tumor progression and eventual death, although few parameters are known that allow stratification of patients into prognostic groups. Methods. Sixty-two patients with skull base chordomas treated at the Massachusetts General Hospital by proton beam irradiation therapy with at least 2 years of follow-up information were reviewed in an attempt to identify clinical and pathologic parameters that predicted outcome. Results. Female sex, tumor necrosis in preradiation treatment biopsy, and tumor volume in excess of 70 ml were each independent predictors of shortened overall survival after radiation therapy for skull base chordoma. Conclusions. Stratification of patients with skull base chordoma into poor and good outcome groups can be performed using the three parameters identified in our study. In addition, the striking difference in survival between the sexes suggests that further investigations of these tumors should include determination of their hormonal receptor status and consideration of hormonal manipulation in their management. Cancer 1994;74:2261-7.

Background. Chordomas are rare neoplasms that show a proclivity for the spheno-occiput and sacral regions. A 'chondroid' variant involving the spheno-occiput has been associated with improved survival. 'Classic' or nonchondroid chordomas are uniformly immuno-reactive for keratins. Chondroid chordomas are said to be immunonegative for epithelial markers, a feature used to support the concept that they represent chondro-sarcomas. Methods. The authors performed immunohistochemical studies on 25 patients with chondroid chordoma (mean age, 40.0 years) and on 16 patients with classic chordomas (mean age, 44.2 years) to establish tumor subsets based upon immunophenotype, specifically reactivity for epithelial markers. Kaplan-Meier survival curves were then constructed for each group with age as an added variable. Results. All classic chordomas reacted for keratins as did 8 (32%) of the 25 chondroid chordomas. Forty-four percent of classic and 85% of chondroid chordomas were positive for S-100 protein. At 5 years, all patients younger than 40 years of age were alive in both the classic and chondroid groups. In contrast, of patients older than 40 years of age, only 22% with classic chordomas and 38% with chondroid chordomas were alive. Conclusions. Regardless of tumor subtype, age is the single most important variable in determining survival; patients younger than 40 years of age do better than older patients. There are no significant survival differences between patients with cartilage-containing tumors that are keratin immunopositive ('true' chondroid chordoma) or negative (chondrosarcoma). Immunostaining for keratins is of no prognostic value in assessing chondroid lesions of the spheno-occiput.

Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma that has been reported to have a relatively good prognosis. The authors report ten patients with EMC on whom there was a minimum follow-up of 10 years. The patients' ages ranged from 31 to 72 years (mean, 57 years); there were six men and four women (seven white, three black). The tumor locations included the knee area and thigh (three patients each), the shoulder (two patients), and the leg and foot (one patient each). The tumors ranged from 3.5 to 18 cm in greatest dimension (median, 11.5 cm). All cases had typical histologic features. Lung metastases developed in all patients but one, and three patients had extrathoracic metastases (one in regional lymph nodes; one in subcutis; and one widespread). Four of the patients who had metastases also had local recurrence, as did the only patient without known metastases. Seven patients died of tumor at 4, 5, 8, 10, 15, 16, and 17 years, respectively, and the three remaining patients were alive with metastatic disease at latest follow-up of 13, 14, and 16 years. The authors' results are distinctly different from those previously reported in series with shorter follow-up times. The authors conclude that extraskeletal myxoid chondrosarcomas are indolent but resilient and capricious tumors with a high potential for metastasis, especially to the lungs, over the long term. Cancer 1992; 70:2827-30.

Chondrosarcomas are cartilaginous tumors, and chondrosarcomas of bone occur primarily in adults. The condition can occur in children, however, and in some reports in the medical literature the condition is said to have a poorer prognosis in children than in adults. But this was not supported by the current study of 47 chondrosarcomas in patients under the age of 17. Only one of the chondrosarcomas was of high grade. The low-grade histological appearance of the other tumors was reflected in the long-term follow-up of the patients for an average of eight years, during which time metastatic spread of the tumors was not observed. The treatment of chondrosarcoma is surgical, and although the prognosis is favorable, local recurrence and the necessity of further surgery is not uncommon. The difference between the outcome of chondrosarcoma in the present report and that in previous reports may be due to differences in diagnostic criteria used for defining chondrosarcoma. In the current study, the authors did not consider cases of the more malignant chondroblastic osteosarcoma, although cases of this tumor would be classified with chondrosarcoma by others. (Consumer Summary produced by Reliance Medical Information, Inc.)