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

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

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

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

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

OBJECTIVETo study the clinicopathologic and immunohistochemical features of mesenchymal chondrosarcoma.

METHODSThe clinical and histologic features of 23 cases of mesenchymal chondrosarcoma were analyzed. Immunohistochemical study was also performed in 14 of the cases.

RESULTSThe age of patients ranged from 12 to 47 years. Fourteen of them occurred in males. Thirteen cases involved the bony skeleton and 5 cases affected the soft tissue. The patients presented with pain and/or swelling. Histologically, the tumor consisted of a mixture of undifferentiated small round cells and hyaline cartilage. Transition between the two components was demonstrated and growth plate-like cartilage was observed. Immunohistochemical study showed that the small round cells were positive for Sox9 (14/14), CD99 (12/14), vimentin (6/14), CD56 (4/14), CD57 (4/14), neuron-specific enolase (3/14) and desmin(1/14). They were negative for Coll-II, S-100 protein, epithelial membrane antigen, pan-cytokeratin, synaptophysin, chromogranin A, CD34 and c-erbB2.

CONCLUSIONSMesenchymal chondrosarcoma is a rare malignant tumor. Thorough histologic examination, when coupled with immunohistochemical findings, is helpful in arriving at a correct diagnosis.

12E7 Antigen ; Adolescent ; Adult ; Antigens, CD ; metabolism ; Bone Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Cell Adhesion Molecules ; metabolism ; Child ; Chondrosarcoma, Mesenchymal ; diagnostic imaging ; metabolism ; pathology ; secondary ; surgery ; Female ; Follow-Up Studies ; Humans ; Immunohistochemistry ; Lung Neoplasms ; secondary ; Male ; Mediastinal Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Middle Aged ; Neoplasm Recurrence, Local ; Orbital Neoplasms ; diagnostic imaging ; metabolism ; pathology ; surgery ; Radiography ; SOX9 Transcription Factor ; metabolism ; Vimentin ; metabolism ; Young Adult

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

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

We report here on a case of primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas. A 41-year-old man was evaluated by CT to find the cause of his abdominal pain. The CT scans showed a heterogeneously enhancing necrotic mass with numerous areas of coarse calcification, and this was located in the left side of the retroperitoneal space and involved the body and tail of the pancreas. Portal venography via the celiac axis also showed invasion of the splenic vein. Following excision of the mass, it was pathologically confirmed to be primary extraskeletal mesenchymal chondrosarcoma that arose from the pancreas.
Abdominal Pain/etiology ; Adult ; Chondrosarcoma, Mesenchymal/complications/*diagnosis/surgery ; Contrast Media/administration & dosage ; Diagnosis, Differential ; Humans ; Iohexol/analogs & derivatives/diagnostic use ; Male ; Necrosis ; Pancreas/pathology/radiography ; Pancreatic Neoplasms/complications/*diagnosis/surgery ; Portal Vein/radiography ; Radiographic Image Enhancement/methods ; Rare Diseases ; Retroperitoneal Space/radiography ; Splenic Vein/radiography ; Tomography, X-Ray Computed/methods