This review provides an overview of the histopathology, classification, diagnostic procedures, and therapy of skeletal chondrosarcoma. Chondrosarcomas that arise de novoareprimarychondrosarcomas,whereaschondrosarcomas developing superimposed on pre-existing benign cartilage neoplasms such as enchondromas or osteochondromas are referred to as secondary chondrosarcomas. Conventional chondrosarcomas can be categorized according to their location in bone into central, peripheral, and juxtacortical chondrosarcomas. Histological grading is related to prognosis; however, it is also subject to interobserver variability. Rare subtypes of chondrosarcoma, including dedifferentiated, mesenchymal, and clear cell chondrosarcoma, are discussed as well. Magnetic resonanceimagingisnecessarytodelineatetheextentoftheintraosseous and soft tissue involvement preoperatively. Computed tomography is especially recommended in the pelvis and other flat bones where it may be difficult to discern the pattern of bone destruction and the presence of matrix mineralization. Wide, en-bloc excision is the preferred surgical treatment in intermediate- and high-grade chondrosarcoma. In low-grade chondrosarcoma confined to the bone, extensive intralesional curettage followed by local adjuvant treatment and filling the cavity with bone grafthaspromisinglong-termclinicalresultsandsatisfactory local control. Chondrosarcomas are relatively radiotherapy resistant; therefore, doses >60 Gy are needed in attempts to achieve local control after incomplete resection. Irradiation with protons or other charged particles seems beneficial in this curative situation. Chemotherapy is only possibly effective in mesenchymal chondrosarcoma, and is of uncertain value in dedifferentiated chondrosarcoma. Potential new systemic treatment targets are being discussed. The Oncologist 2008;13:320–329

https://theoncologist.onlinelibrary.wiley.com/doi/pdf/10.1634/theoncologist.2007-0237erratum

The Clinical Approach Towards Chondrosarcoma

We assessed the clinical results and complications associated with a new endoprosthetic replacement system (Mutars®) used in 250 patients with a malignant bone or soft tissue tumor. The key features of the system are its cementless, hexagonal-shaped stem (titanium alloy), the possibility of torsion

Endoprosthetic reconstruction in 250 patients with sarcoma.

We studied 153 patients with non-metastatic chondrosarcoma of bone to determine the risk factors for survival and local tumour control. The minimum follow-up was for five years; 52 patients had axial and 101 appendicular tumours. Surgical treatment was by amputation in 27 and limb-preserving surgery in 126. The cumulative rate of survival of all patients, at 10 and 15 years, was 70% and 63%, respectively; 40 patients developed a local recurrence between 3 and 87 months after surgery and 49 developed metastases. Local recurrence was associated with poor survival in patients with concomitant metastases but not in those without. On multivariate analysis independent risk factors for rates of survival include extracompartmental spread, development of local recurrence and high histological grade. Independent risk factors for local recurrence include inadequate surgical margins and tumour size greater than 10 cm. Location within the body, the type of surgery and the duration of symptoms are of no prognostic significance. Surgical excision with an oncologically wide margin provides the best prospect both for cure and local control in these patients.

Risk factors for survival and local control in chondrosarcoma of bone

As a group, cartilage tumours are the most common primary bone lesions They range from benign lesions, such as enchondromas and osteochondromas, to malignant chondrosarcoma The benign lesions result from the deregulation of the hedgehog signalling pathway, which is involved in normal bone development These lesions can be the precursors of malignant chondrosarcomas, which are notoriously resistant to conventional chemotherapy and radiotherapy Cytogenetic studies and mouse models are beginning to identify genes and signalling pathways that have roles in tumour progression, such as hedgehog, p53, insulin-like growth factor, cyclin-dependent kinase 4, hypoxia-inducible factor, matrix metalloproteinases, SRC and AKT, suggesting potential new therapeutic approaches

Cartilage tumours and bone development: molecular pathology and possible therapeutic targets

Background. Enchondromatosis is characterized by the presence of multiple benign cartilage lesions in bone. While Ollier disease is typified by multiple enchondromas, in Maffucci syndrome these are associated with hemangiomas. Studies evaluating the predictive value of clinical symptoms for development of secondary chondrosarcoma and prognosis are lacking. This multi-institute study evaluates the clinical characteristics of patients, to get better insight on behavior and prognosis of these diseases. Method.Aretrospectivestudywasconductedusingclinical dataof144Ollierand17Maffuccipatientsfrom13European centers and one national databank supplied by members of the European Musculoskeletal Oncology Society. Results. Patients had multiple enchondromas in the hands and feet only (group I, 18%), in long bones including scapula and pelvis only (group II, 39%), and in both small and long/flat bones (group III, 43%), respectively. The overall incidence of chondrosarcoma thus far is 40%. In group I, only 4 patients (15%) developed chondrosarcoma, in contrast to 27 patients (43%) in group II and 26 patients (46%) in group III, respectively. The risk of developing chondrosarcoma is increased when enchondromas are located in the pelvis (odds ratio, 3.8; p 0.00l). Conclusions. Overall incidence of development of chondrosarcoma is 40%, but may, due to age-dependency, increase when considered as a lifelong risk. Patients with

/pdf/incidence-predictive-factors-and-prognosis-of-chondrosarcoma-2nck3rv47i.pdf

Incidence, Predictive Factors, and Prognosis of Chondrosarcoma in Patients with Ollier Disease and Maffucci Syndrome: An International Multicenter Study of 161 Patients

We carried out extensible endoprosthetic replacement of the proximal or total humerus in 18 children aged between six and 12 years, after resection of primary bone tumours mainly for osteosarcoma and Ewing’s sarcoma. In 11 patients we performed 44 lengthening procedures, with an average of two per child annually and a mean total extension of 29.9 mm per patient. We were able to achieve lengthening of the operated limb with few complications and a mean functional rating of 79.3% according to the Enneking system. Progressive lengthening of these prostheses does not adversely affect the overall function of the arm, and superior subluxation of the head of the prosthesis has not been a problem.

Extensible endoprostheses of the humerus after resection of bone tumours

Ten patients underwent endoprosthetic replacement of the distal humerus for bone tumours over a period of 30 years. There were eight primary and two secondary tumours in four men and six women with a mean age of 47.5 years (15 to 76). The mean follow-up was eight years (9 months to 31 years). Four patients required further surgery, three having revision for aseptic loosening; two of these and one other later needing a rebushing. There were no cases of postoperative nerve palsy, infection, local recurrence or mechanical failure of the implant. Four patients died from their disease, all with the prosthesis functioning satisfactorily. At follow-up the mean flexion deformity of the elbow was 15° (0 to 35) and the mean range of flexion was 115° (110 to 135). The functional results showed a mean Toronto extremity salvage score of 73% of normal. Endoprosthetic replacement of the distal humerus and elbow is a satisfactory method of treating these rare tumours.

/pdf/the-results-of-endoprosthetic-replacement-for-tumours-of-the-34u7huhp2l.pdf

The results of endoprosthetic replacement for tumours of the distal humerus

Only 1% of all primary bone tumours are situated in the distal humerus. Destruction of the distal humerus by tumour is rare and reconstruction of the distal humerus is challenging. Because of the amount of bone loss following tumour excision, excision arthroplasty or arthrodesis is impossible and hence some form of reconstruction is usually required. Allograft reconstruction and hemiarthroplasty are uncommon and lead to an unpredictable outcome. Ten patients underwent endoprosthetic replacement of the distal humerus for bone tumours over a thirty one-year period. There were 8 primary and 2 secondary tumours and male to female ratio was 2:3. Average age of the patients was 47.5 years (15–76 years). Mean follow up was 8 years (9 months - 31 years). Four patients required further surgery, three having revision for asceptic loosening and two of these and one other later needing a rebushing. There were no permanent nerve palsies, infections, local recurrences or mechanical failures of the implant. Four patients died of their disease between 12 and 71 months after operation, all with their prosthesis working normally. Average flexion deformity was 15 degrees (0–35) and average flexion of these patients was 115 degrees (110–135). The average TES Score for these patients was 73% (29% to 93%). The activities which the patients found to be no problem (TES score more than 4.5 out of 5) were: brushing hair, drinking from a glass, putting on make up or shaving, picking up small items, turning a key in a lock, doing light household chores and socialising with friends, whilst activities that proved difficult (TES score less than 3 out of 5) were: gardening and lifting a box to an overhead shelf. Pain was not a problem and only 1 of the surviving patients reported ever having to use regular analgesics. Conclusions: Endoprosthetic replacement of the distal humerus and elbow joint is a satisfactory method of dealing with these unusual tumours in the long term.

The results of endoprosthetic replacements for tumours of the distal humerus

To The Editor: 

We read with interest "Chondrosarcoma of Bone: An Assessment of Outcome," by Lee et al. (81-A: 326-338, March 1999). Their experience extending over twenty-two years is impressive and largely parallels our own experience, which we are currently reviewing. We completely agree with their comments about the difficulty in grading chondroid tumors, and we note that some of the low-grade tumors in their study would have certainly been considered to be of borderline malignancy. We fully appreciate the difficulty in grading this group of tumors, and we have, for the past five years, designated them as CLUMPs, which stands for "cartilaginous lesions of unknown malignant potential." This categorization will help us to get some idea in the future about how these borderline lesions behave, but at the present time the follow-up is insufficient for us to be able to comment with any certainty.

We believe that in order for a tumor to be designated as a central chondrosarcoma, it must demonstrate permeative growth on either the biopsy specimen or the resection specimen. We define permeative growth as the presence of tumor around three sides of a trabecula normal medullary bone. If this feature is present, then the tumor is defined as a chondrosarcoma. This leaves us with a sizable portion of low-grade cartilaginous tumors that may be modestly cellular, focally myxoid, show mild nuclear …

Chondrosarcoma of bone.

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