mesenchymal chondrosarcoma


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mesenchymal chondrosarcoma

A rare high-grade chondrosarcoma consisting of undifferentiated mesenchymal cells admixed with cartilage.
 
Sites
Femur, ribs, jaw, skull, and spine are most common, though it may occur in any bone.

Clinical findings
Pain, swelling. Two-thirds arise in bone—femur, ribs, spine, maxilla, mandible, pelvis and other tubular bones.

Demographics
Comprise 2–10% of chondrosarcomas. Most common age 20 to 40; slight male predominance.

Clinical findings
1–40-cm mass, pain, headache with intracranial lesions. Base of skull tumours present with brainstem symptoms and cranial nerve dysfunction.

Imaging
Lytic lesions with stippled density characteristic of cartilaginous tumours; margin is ill-defined, but may be sharp if sclerosis is present. Expansile lesions are osteolytic. A lobulated (calcified “popcorn” lesion) extraosseous mass in soft tissues (e.g., skin, joint tissues, viscera, or meninges) may develop over time.

Metastatic sites
Lung, bone, viscera, lymph nodes.

Management
Radical aggressive surgery. Complete excision yields a 27% survival; incomplete, 0%. Chemotherapy as for Ewing sarcoma.

Prognosis
Poor compared to other chondrosarcomas. Chemotherapy + complete surgical remission yields a 10-year disease-free survival of 72%, compared to 17% for surgery alone. They often recur and metastasise late > 10 years.
References in periodicals archive ?
Extraskeletal mesenchymal chondrosarcoma of the mediastinum.
Cytomorphology of intraparenchymal mesenchymal chondrosarcoma in frontal lobe: report of a case.
Reappraisal of mesenchymal chondrosarcoma: novel morphologic observations of the hyaline cartilage and endochondral ossification and beta-catenin, Sox9, and osteocalcin immunostaining of 22 cases.
Our case demonstrated an unusual and rare extraskeletal location of mesenchymal chondrosarcoma: extramedullary, intradural within the cervical spine.
Aigner, "Mesenchymal chondrosarcoma: An immunohistochemical study of 10 cases examining prognostic significance of proliferative activity and cellular differentiation," Pathology, vol.
More recently, Wehrli and colleagues reported on the utility of Sox9, a transcription factor thought to be a master regulator of chondrogenesis, as helpful in distinguishing mesenchymal chondrosarcoma from other small round blue cell tumors (5).
CONCLUSION: Mesenchymal chondrosarcoma though rare, should be considered in the differential diagnosis of soft tissue lesions, as one third of the cases occur extraskeletally.
Radiographically, it may be impossible to differentiate mesenchymal chondrosarcoma from --.
The consultation records of a senior bone pathologist (H.D.D.) spanning 45 years (1964-2009) were reviewed, yielding 20 patients from local, national, and international institutions who carried the diagnosis of mesenchymal chondrosarcoma. Information reviewed included clinical presentation, radiographs, and microscopic sections of the primary tumor stained with hematoxylin-eosin.
The review of current literature was performed by a PubMed query for articles with "Mesenchymal Chondrosarcoma" in the title or abstract from 1959 to the present.
Mesenchymal chondrosarcoma in the young: a clinicopathological study of 19 patients with explanation of histogenesis.
Tumor-induced osteomalacia and symptomatic looser zones secondary to mesenchymal chondrosarcoma. J Surg Oncol.