9240/3 Mesenchymal chondrosarcoma


Definitions

Bone
ICD-O-3 topography code: C40-C41
ICD10: C40-C41
           

Mesenchymal chondrosarcoma is a rare malignant variant of chondrosarcoma and most commonly affects the craniofacial bones (especially the jawbones), the ribs, the ilium, and the vertebrae. The tumours are usually well defined, circumscribed masses varying from 3 to 30 cm in diameter. They are primarily lytic and destructive with poor margins, not significantly differing from ordinary chondrosarcoma in most cases. Expansion of the bone is frequent, and cortical destruction or cortical breakthrough with extraosseous extension of soft tissue is common.
Mesenchymal chondrosarcoma is histologically composed of highly undifferentiated small round cells and islands of well differentiated hyaline cartilage 1
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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The tumour occurs at any age, with a peak incidence in the second and the third decades. It is highly malignant, with a strong tendency toward local recurrence and distant metastasis which are observed even after a delay of more than 20 years. The clinical course is frequently protracted and relentless, making longterm follow up mandatory
2
 
Fletcher CDM, Unni KK, Mertens F (Eds.)
World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone.
3rd Edition
IARC Press: Lyon 2002



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Connective, subcutaneous and other soft tissues
ICD-O-3 topography code: C49
ICD10: C49
           

A rare morphologic variant of chondrosarcoma. It is characterized by the presence of malignant small round cells, biphasic growth pattern, and well differentiated hyaline cartilage. Clinical presentation includes pain and swelling. The clinical course is aggressive, with local recurrences and distant metastases.



Nasal cavity and Sinuses
ICD-O-3 topography code: C30-C31
ICD10: C30-C31
           

A rare morphologic variant of chondrosarcoma. It is characterized by the presence of malignant small round cells, biphasic growth pattern, and well differentiated hyaline cartilage. Clinical presentation includes pain and swelling. The clinical course is aggressive, with local recurrences and distant metastases.