Objective: To investigate the immunohistochemical expression of NKX3.1 and NKX2.2 in mesenchymal chondrosarcoma (MC), and to explore the differential diagnostic value of NKX3.1 and NKX2.2 in MC and other types of small round cell malignant tumors. Methods: A total of 12 cases of MC and 97 other small round cell malignant tumors diagnosed in Jinling Hospital, Nanjing University School of Medicine from 2001 to 2020 were collected for NKX3.1 and NKX2.2 immunohistochemical detection. Among them, two kinds of NKX3.1 antibodies [rabbit polyclonal antibody and rabbit monoclonal antibody (EP356)] were used for detection in 12 cases of MC, and one NKX3.1 antibody (rabbit polyclonal antibody) was detected in 97 cases of other small round cell malignant tumors, and the relevant literature was reviewed. Results: The 12 MC patients included 7 females and 5 males, with a mean age of 33 years (14-54 years). Nine cases were from bone and three from soft tissue. Among the 12 MC patients, 8 patients had postoperative recurrence or metastasis, and 3 of them died of tumor recurrence or metastasis. Histologically, 12 cases of MC showed typical bidirectional differentiation.The positive rate of both NKX3.1 antibodies in MC was 12/12, NKX3.1 was focal weakly positive in only one of 12 chondrosarcomas (grade 3), 5 alveolar rhabdomyosarcomas, 5 embryonal rhabdomyosarcomas, and 5 solitary fibrous tumors, respectively. The remaining 70 cases of other small round cell malignant tumors were negative. The positive rates of NKX2.2 in MC, Ewing sarcoma and olfactory neuroblastoma were 12/12, 15/15 and 4/5, respectively. In 12 cases of chondrosarcoma (grade 3), 5 cases of poorly differentiated synovial sarcoma, 5 cases of alveolar rhabdomyosarcoma, and 5 cases of solitary fibrous tumor, NKX2.2 was focally and weakly positive in only one case, respectively, and all the remaining 50 cases of other small round cell malignant tumors were negative. Conclusions: The expression of NKX3.1 and NKX2.2 proteins are significant indicators in the diagnosis of MC, and the combined detection of NKX3.1 and NKX2.2 can help distinguish MC from most other small round cell malignant tumors.
Biomarkers, Tumor ; Chondrosarcoma, Mesenchymal/diagnosis* ; Diagnosis, Differential ; Female ; Homeodomain Proteins ; Humans ; Immunohistochemistry ; Male ; Nuclear Proteins

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

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

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 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

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

Mesenchymal chondrosarcoma is a rare cartilaginous neoplasm of an extraskeletal origin, and this predominately occurs in the head and neck, and also in the lower extremities. Fewer than twenty cases of cardiac mesenchymal chondrosarcoma have so far been reported on. For the most part, the results of treatment for patients with this condition have been dismal. In this study, we describe a case of cardiac mesenchymal chondrosarcoma that responded to chemotherapy following surgical biopsy. A 46-year-old man was referred for evaluation of his pleural effusions in both lungs. Chest computed tomography revealed an ovoid-shaped mass in the posterior wall of the patient's left atrium. The echocardiogram revealed a large ovoid-shaped immobile mass (11 x 6 cm2) in the pericardiac space, which was attached to the posterior wall of the left atrium. Emergency pericardiostomy with closure thoracostomy was performed. Seven days later, a thoracotomy was performed for reduction and diagnosis of the cardiac mass. The pathological diagnosis was extraskeletal mesenchymal chondrosarcoma of the heart.. Postoperative chemotherapy was performed for the huge remaining mass with a combined regimen of etoposide, ifosfamide and cisplatin. After 6 cycles, the patient showed a partial response without symptoms. Although cardiac mesenchymal chondrosarcoma has been reported to be chemotherapy- resistant with a short survival duration, chemotherapy may prove to be an effective treatment modality.
Biopsy ; Chondrosarcoma, Mesenchymal* ; Cisplatin ; Diagnosis ; Drug Therapy* ; Emergencies ; Etoposide ; Head ; Heart Atria ; Heart* ; Humans ; Ifosfamide ; Lower Extremity ; Lung ; Middle Aged ; Neck ; Pericardial Window Techniques ; Pleural Effusion ; Thoracostomy ; Thoracotomy ; Thorax

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

12E7 Antigen ; Antigens, CD ; metabolism ; Cell Adhesion Molecules ; metabolism ; Child, Preschool ; Chondrosarcoma, Mesenchymal ; metabolism ; pathology ; surgery ; Diagnosis, Differential ; Humans ; Immunohistochemistry ; Male ; Neuroblastoma ; metabolism ; pathology ; Sarcoma, Ewing ; metabolism ; pathology ; Spinal Cord Neoplasms ; metabolism ; pathology ; surgery ; Spinal Neoplasms ; metabolism ; pathology ; Thoracic Vertebrae ; Vimentin ; metabolism