is a common complication after treatment for breast cancer. Factors associated with increased risk of lymphedema include extent of axillary surgery, axillary radiation, infection, and patient obesity. 2,3 The panel recommends educating the patients on lymphedema, monitoring for lymphedema, and referring for lymphedema management as needed.”

NCCN Clinical Practice Guidelines in Oncology (NCCN Guidelines

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

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.

https://pubs.rsna.org/doi/pdf/10.1148/rg.235035134

From the archives of the AFIP: imaging of primary chondrosarcoma: radiologic-pathologic correlation.

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

Dedifferentiated chondrosarcoma is a rare, highly malignant variant of chondrosarcoma in which a high-grade spindle-cell sarcoma coexists with a lower-grade chondroid tumour. We have reviewed our experience with this neoplasm in 22 patients, all of whom were treated using modern oncological principles of planned resection and chemotherapy. Despite this the median survival was under nine months and only 18% were alive at five years. Those patients who received chemotherapy, and in whom wide margins of excision were achieved at operation, did best. It is essential to have an accurate preoperative diagnosis in order to plan treatment which may offer a better prospect of cure.

Experience in the treatment of dedifferentiated chondrosarcoma

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

Purpose. Clear cell chondrosarcoma is a rare variant of chondrosarcoma. Six cases are herein reported.

Subjects. We have treated six patients with clear cell chondrosarcoma in the past 28 years, representing 1.6% of all chondrosarcomas seen in this time period.

Results and Discussion. Half the patients had been initially underdiagnosed and inappropriately treated.

Conclusions. Our results and our review of the literature highlight the fact that inadequate initial treatment leads to a high rate of both local recurrence and metastasis whilst wide initial excision is usually curative.

/pdf/clear-cell-chondrosarcoma-of-bone-3gjyev7cmw.pdf

Clear cell chondrosarcoma of bone.

Synovial sarcoma is a relatively common soft tissue sarcoma particularly in the adolescent and young adult. We report an unusual case of a synovial sarcoma arising within a popliteal cyst in a 13-year-old female presenting with bilateral popliteal cysts. MR imaging demonstrated the cyst with evidence of subacute haemorrhage and a discrete nodule of tumour.

Synovial sarcoma arising in association with a popliteal cyst

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