mesenchymal chondrosarcoma


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mesenchymal chondrosarcoma

[meseng′kəməl]
Etymology: Gk, mesos, middle, enchyma, infusion, chondros, cartilage, sarx, flesh, oma, tumor
a rare malignant tumor of soft tissue that develops in many sites. The tumors are highly vascular.

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 ?
Despite being a recognized entity for more than 50 years,1 mesenchymal chondrosarcoma continues to present substantial diagnostic, prognostic, and management challenges, in large part because of its rarity.
Font et al (12) correlated computed tomography appearance with histopathology of orbital mesenchymal chondrosarcoma, noting that hemangiopericytomatous areas are enhancing and chondrosarcoma-like areas appear nonenhancing upon gadolinium administration.
The histology of mesenchymal chondrosarcoma is typically characterized by a unique, biphasic pattern, composed of mesenchymal and chondrocytic components.
CD99, although immunoreactive in all 11 mesenchymal chondrosarcoma tumors in one study, (28) is also a marker for ES and for primitive neuroectodermal tumors, further clouding the distinction between these tumors.
In an attempt to improve diagnostic specificity, Wehrli et al (30) compared immunohistochemistry profiles of 12 mesenchymal chondrosarcoma samples with several other small, round, blue-cell tumors and demonstrated expression of Sox9 in both cartilaginous and mesenchymal regions in 21 of 22 mesenchymal chondrosarcoma tumors (95.
10,13,35-41) Findings in mesenchymal chondrosarcoma that are related to ES include the reciprocal translocation (11; 22) (q24; q12) (40) and trisomy 8.
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.
MRI of mesenchymal chondrosarcoma of the orbit: case report and review of literature.
Cell differentiation and matrix gene expression in mesenchymal chondrosarcoma.
Translocation der(13;21)(q10;q10) in skeletal and extraskeletal mesenchymal chondrosarcoma.
Mesenchymal chondrosarcoma of a child with reciprocal translocation (11;22)(q24;q12), Cancer Genet Cytogenet.