Dedifferentiated Chondrosarcoma




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Location: Proximal femur, pelvis, and proximal humerus.

Clinical: On a history of long duration with moderate symptoms, a rapid progression of pain and swelling occurs. Pathologic fractures frequent.

Imaging: On x-ray: (1) old cartilaginous lesion: moderate expansion of the bone with thickened, scalloped cortex, with many calcifications; (2) new lesion: destroying the cortex, involving the soft tissues, and dissolving the calcifications, with ill-defined margins. Three different features are as follows: (a) pathologic fracture in a new aggressive lytic lesion canceling preexisting calcifications of an intensely radiopaque old cartilaginous lesion, (b) usual chondrosarcoma aspect with a small high-grade lytic lesion, (c) typical high-grade sarcoma feature with small remnants of chondrosarcoma. On CT, two lesions of different density, matrix, and enhancement. On MRI, two different signal intensities on T1 and T2.

Histopathology: Two types of tissues: (1) low-grade cartilaginous tumor and (2) high-grade malignancy like UPS, OS, FS. The transition is sharp.

Course and Staging: Fast growth, high risk of recurrence with inadequate surgery, and high rate of metastases, often observed at diagnosis. Usually, stage IIB or III.

Treatment: Wide or radical resection. Chemotherapy protocols of OS have been used with effectiveness. Often, an amputation is necessary to obtain adequate margins. Poor prognosis.


Key Points













Clinical

Symptoms rapidly increasing. Pathologic fracture possible

Radiological

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Feb 19, 2017 | Posted by in MUSCULOSKELETAL MEDICINE | Comments Off on Dedifferentiated Chondrosarcoma

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