Humans ; Chondrosarcoma, Mesenchymal/pathology* ; Chondrosarcoma/surgery* ; Muscle, Skeletal/pathology* ; Bone Neoplasms/pathology*

Bone Neoplasms ; pathology ; Chondrosarcoma, Mesenchymal ; pathology ; Dura Mater ; pathology ; Humans ; Meninges ; pathology

Chondroblastoma ; pathology ; Chondrosarcoma ; diagnostic imaging ; pathology ; Chondrosarcoma, Mesenchymal ; pathology ; Diagnosis, Differential ; Female ; Hamartoma ; pathology ; Humans ; Lung Neoplasms ; diagnostic imaging ; pathology ; Middle Aged ; Radiography

Child ; Chondrosarcoma, Mesenchymal ; diagnosis ; drug therapy ; pathology ; Diagnosis, Differential ; Humans ; Male ; Pelvic Neoplasms ; diagnosis ; drug therapy ; pathology ; Tomography, X-Ray Computed ; Treatment Outcome

12E7 Antigen ; Adult ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Hemangiopericytoma ; pathology ; Humans ; Lymphoma ; pathology ; Male ; Nasal Cavity ; Neuroectodermal Tumors, Primitive ; pathology ; Nose Neoplasms ; metabolism ; pathology ; surgery ; Vimentin ; metabolism ; Young Adult

OBJECTIVETo study the clinicopathologic and radiologic features of dedifferentiated chondrosarcoma, focusing on its diagnosis and differential diagnosis.

METHODClinical, radiological and pathologic findings of 14 cases of dedifferentiated chondrosarcoma (including biopsy and surgical specimens) were analyzed by hematoxylin and eosin stained sections and immunohistochemistry.

RESULTSThe mean age of patients was 52 years. The male-to-female ratio was 9:5. The most common sites of involvement were pelvis, femur and humerus, similar to the conventional chondrosarcoma. Radiologically, they were malignant tumors with dimorphic pattern. Grossly, central chondrosarcomas were more common than those of the peripheral. An essential histological feature of dedifferentiated chondrosarcoma was an abrupt interface between the low-grade cartilaginous tumor and high-grade anaplastic sarcoma. The most common dedifferentiated components were osteosarcoma, malignant fibrous histocytoma and fibrosarcoma. False negative diagnosis and erroneous diagnosis were frequent when only one-time biopsy was available.

CONCLUSIONSDedifferentiated chondrosarcoma is a rare subtype of chondrosarcoma with poor prognosis, which has different features of clinical manifestation, imaging features and pathological characteristics, compared to conventional chondrosarcoma and chondroblastic osteosarcoma.

Adult ; Aged ; Bone Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Cell Differentiation ; Chondrosarcoma ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Chondrosarcoma, Mesenchymal ; pathology ; Diagnosis, Differential ; Female ; Femoral Neoplasms ; diagnostic imaging ; drug therapy ; pathology ; surgery ; Follow-Up Studies ; Humans ; Humerus ; pathology ; Male ; Middle Aged ; Neoplasm Recurrence, Local ; Osteosarcoma ; pathology ; Pelvic Bones ; pathology ; Radiography ; Vimentin ; metabolism ; Young Adult

Adolescent ; Antigens, CD34 ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; Diagnosis, Differential ; Hemangiopericytoma ; diagnostic imaging ; metabolism ; pathology ; Humans ; Male ; Platelet Endothelial Cell Adhesion Molecule-1 ; metabolism ; Sarcoma, Synovial ; metabolism ; pathology ; Tomography, X-Ray Computed ; Vimentin ; metabolism

12E7 Antigen ; Antigens, CD ; metabolism ; Bone Neoplasms ; metabolism ; pathology ; surgery ; Cell Adhesion Molecules ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Hemangiopericytoma ; pathology ; Humans ; Lung Neoplasms ; metabolism ; pathology ; surgery ; Male ; Middle Aged ; Pneumonectomy ; methods

Small round cell tumors (SRCTs) are a heterogeneous group of neoplasms composed of small, primitive, and undifferentiated cells sharing similar histology under light microscopy. SRCTs include Ewing sarcoma/peripheral neuroectodermal tumor family tumors, neuroblastoma, desmoplastic SRCT, rhabdomyosarcoma, poorly differentiated round cell synovial sarcoma, mesenchymal chondrosarcoma, small cell osteosarcoma, small cell malignant peripheral nerve sheath tumor, and small cell schwannoma. Non-Hodgkin\'s malignant lymphoma, myeloid sarcoma, malignant melanoma, and gastrointestinal stromal tumor may also present as SRCT. The current shift towards immunohistochemistry and cytogenetic molecular techniques for SRCT may be inappropriate because of antigenic overlapping or inconclusive molecular results due to the lack of differentiation of primitive cells and unavailable genetic service or limited moleculocytogenetic experience. Although usage has declined, electron microscopy (EM) remains very useful and shows salient features for the diagnosis of SRCTs. Although EM is not always required, it provides reliability and validity in the diagnosis of SRCT. Here, the ultrastructural characteristics of SRCTs are reviewed and we suggest that EM would be utilized as one of the reliable modalities for the diagnosis of undifferentiated and poorly differentiated SRCTs.
Chondrosarcoma, Mesenchymal ; Cytogenetics ; Diagnosis ; Gastrointestinal Stromal Tumors ; Genetic Services ; Humans ; Immunohistochemistry ; Lymphoma ; Melanoma ; Microscopy ; Microscopy, Electron ; Microscopy, Electron, Transmission* ; Neurilemmoma ; Neuroblastoma ; Neuroectodermal Tumors ; Osteosarcoma ; Pathology ; Peripheral Nerves ; Reproducibility of Results ; Rhabdomyosarcoma ; Sarcoma, Myeloid ; Sarcoma, Synovial

Adult ; Aged ; Antigens, CD34 ; metabolism ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; Diagnosis, Differential ; Female ; Follow-Up Studies ; Hemangiopericytoma ; metabolism ; pathology ; radiotherapy ; surgery ; Humans ; Male ; Meningeal Neoplasms ; metabolism ; pathology ; radiotherapy ; surgery ; Meningioma ; metabolism ; pathology ; Middle Aged ; Neoplasm Recurrence, Local ; Proto-Oncogene Proteins c-bcl-2 ; metabolism ; Solitary Fibrous Tumors ; metabolism ; pathology ; Vimentin ; metabolism ; Young Adult