extraskelettales Chondrosarkom

Extraskeletal
myxoid chondrosarcoma: tumor response to sunitinib. Computed Tomography scan (CT) tumor assessment. In panels A, B, C (white arrows) CT, venous phase, after contrast medium, shows a peritoneal metastasis at baseline, after 1 and 4 months of sunitinib with evidence of 30% decrease in tumor size and contrast uptake. Panel D shows an interval tumor progression at 6 months from baseline, i.e. after 2 months from sunitinib interruption, while panel E shows a new response at 7 months, 4 weeks after restoring sunitinib.
clinicalsarcomaresearch.biomedcentral.com • CC-by-2.0
Extraskeletal
myxoid chondrosarcoma: tumor response to sunitinib. Computed Tomography scan (CT) and [18F]fluorodeoxyglucose–positron emission tomography scan (PET) abscess evaluation. CT showed a new mass in the right gluteus muscles characterized by fluid content with rim enhancement (panel C) which was not evident at baseline (panel A). That area became positive on PET (panel D) compared to baseline (panel B). CT (panel E) and PET (panel F) confirmed the resolution of the infection, three months later after sunitinib interruption and treatment with antibiotics.
clinicalsarcomaresearch.biomedcentral.com • CC-by-2.0
Anthracycline-based
chemotherapy in extraskeletal myxoid chondrosarcoma: a retrospective study. Response to chemotherapy with epirubicin and ifosfamide. CT scan (arterial phase after contrast medium). Thigh primary extraskeletal myxoid chondrosarcoma (Panel A1) with concomitant intra-abdominal lymphonodes involvement (Panel A2) at baseline, and after 6 cycles of treatment with epirubicin and ifosfamide (Panel B1 and B2, respectively). The response is marked by a >30% decrease in tumor size thus classifying for a partial response by RECIST.
clinicalsarcomaresearch.biomedcentral.com • CC-by-2.0
Radiopaedia • CC-by-nc-sa 3.0 • de

Extraskeletal chondrosarcomas make up only 2% of soft-tissue sarcomas  and only 1% of all chondrosarcomas.

Pathology 

They tend to be of higher grade than run-of-the-mill conventional intramedullary chondrosarcomas, with the majority being of the myxoid (most common) or mesenchymal varieties .

Location

Extraskeletal myxoid chondrosarcomas typically occur in the extremities, with the thigh being most common. They occur at all ages but typically around the age of 50 .

Extraskeletal mesenchymal chondrosarcomas on the other hand tend to occur in young adults. They are seen also in the thigh, but also have a predilection for the head and neck, occurring in the meninges, the orbit and even in the brain.

Chondrosarcomas have been reported in most other parts of the body, although with less frequency, including:

  • meninges of the brain and spinal cord (most common)
  • lower limb (particularly thigh)
  • soft tissues of the head and neck
    • orbit
    • larynx
    • sinonasal cavity
  • lung - primary pulmonary chondrosarcoma - very rare 
  • solid organs
    • pancreas

Radiographic features

Their appearance is the same as that of chondrosarcomas elsewhere (see generic chondrosarcoma article for radiographic features).

Differential diagnoses

Siehe auch:
und weiter:
Assoziationen und Differentialdiagnosen zu extraskelettales Chondrosarkom:
Chondrosarkom
 
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