Dahlin's Bone Tumors. General Aspects and Data on 11,087 Cases
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TL;DR: Osteosarcoma, which is the most common primary bone tumor, occurs most frequently in adolescents, but there is a second incidence peak among individuals aged >60 years, and direct comparisons among patients of all ages and ethnicities are not available.
Abstract: Background
Osteosarcoma, the most common primary bone tumor, occurs most frequently in adolescents, but a second incidence peak among individuals over age 60 exists. Most osteosarcoma epidemiology studies have been embedded in large analyses of all bone tumors, or focused on cases occurring in adolescence. Detailed descriptions of osteosarcoma incidence and survival specifically, with direct comparisons among subjects of all ages and ethnicities, are not available.
1,723Â citations
TL;DR: Overall, worldwide osteosarcoma incidence rates were quite similar in the younger age groups, but the greatest variation in incidence rates was observed in the elderly.
Abstract: Osteosarcoma incidence rates in the United States peak in adolescence and in the elderly. The international patterns of osteosarcoma incidence in children have been described, whereas those for young, middle age or elderly adults have not. Using the Cancer Incidence in Five Continents, International Agency for Cancer Research database we compared incidence rates for children and adolescents (age 0-24 years), the middle age group (25-59 years) and elderly (>or=60 years) persons by world regions and individual countries. Overall, worldwide osteosarcoma incidence rates were quite similar in the younger age groups. The greatest variation in incidence rates was observed in the elderly.
527Â citations
TL;DR: Understanding and recognizing the spectrum of appearances of the various types of primary chondrosarcoma allow improved patient assessment and are vital for optimal clinical management including diagnosis, biopsy, staging, treatment, and prognosis.
Abstract: Chondrosarcoma is a malignant tumor that produces cartilage matrix, and lesions that arise de novo are called primary. Primary chondrosarcoma is the third most common primary malignant tumor of bone, constituting 20%-27% of all primary malignant osseous neoplasms. There are numerous types of primary chondrosarcomas, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. The conventional intramedullary chondrosarcoma is the most frequent type, and it most commonly involves the long bones or pelvis in up to 65% of cases. Although the pathologic appearance varies with specific lesion type, chondrosarcomas grow with lobular type architecture, and these hyaline cartilage nodules demonstrate high water content and peripheral enchondral ossification. Imaging features directly reflect this pathologic appearance, and the various subtypes often show distinctive features. Radiographic findings often suggest the diagnosis of chondrosarcoma because of identification of typical "ring-and-arc" chondroid matrix mineralization (representing the enchondral ossification) and aggressive features of deep endosteal scalloping and soft-tissue extension. These latter features are usually best assessed, as is lesion staging, with computed tomography (CT) or magnetic resonance (MR) imaging. CT is optimal to detect the matrix mineralization, particularly when it is subtle or when the lesion is located in anatomically complex areas. Both CT and MR imaging depict the high water content of these lesions as low attenuation and very high signal intensity with T2-weighting, respectively. Understanding and recognizing the spectrum of appearances of the various types of primary chondrosarcoma allow improved patient assessment and are vital for optimal clinical management including diagnosis, biopsy, staging, treatment, and prognosis.
403Â citations
TL;DR: Meta-analysis in patients with localised high-grade osteosarcoma shows that 3-drug regimens, for example MAP are the most efficacious drug regimens and Salvage of poor responders by changing drugs, or intensifying treatment postoperatively has not proven to be useful.
355Â citations
TL;DR: A wide surgical margin is the most important predictor of survival and of local recurrence in patients with sacrococcygeal chordoma and a combined anteroposterior approach increases the likelihood of obtaining a wide margin.
Abstract: Background: Sacrococcygeal chordoma presents a difficult diagnostic and therapeutic problem, with a high rate of local recurrence. The purpose of this report is to define the importance of adequate surgical treatment for optimum outcome and survival.
Methods: Fifty-two patients underwent surgical treatment for sacrococcygeal chordoma between 1980 and 2001. The series included eighteen female patients and thirty-four male patients, with an average age of fifty-six years (range, thirteen to seventy-six years) at the time of the diagnosis. The surgical approach depended on the level and extent of the lesion, with a posterior approach performed in twenty-two patients and a combined anteroposterior approach used in thirty. A wide surgical margin was achieved in twenty-one patients.
Results: At an average of 7.8 years (range, 2.1 to twenty-three years) postoperatively, twenty-three patients were alive with no evidence of disease. Twenty-three patients (44%) had local recurrence. The rate of recurrence-free survival was 59% at five years and 46% at ten years. The overall survival rates were 74%, 52%, and 47% at five years, ten years, and fifteen years, respectively. The most important predictor of survival was a wide margin. All patients with a wide margin survived, and this survival rate was significantly different from that for patients who had had either marginal or intralesional excision (p = 0.0001). Of the twenty-one patients with a wide margin, seventeen (81%) had undergone a combined anteroposterior approach and only four had been treated with a posterior approach.
Conclusions: A wide surgical margin is the most important predictor of survival and of local recurrence in patients with sacrococcygeal chordoma. Use of a combined anteroposterior approach increases the likelihood of obtaining a wide margin.
Level of Evidence: Therapeutic Level IV. See Instructions to Authors for a complete description of levels of evidence.
316Â citations