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

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

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

Extraskeletal chondrosarcoma is rare, making up only 1% of reported chondrosarcoma. We experienced 3 cases of extraskeletal chondrosarcoma, especially in vulva. They were suspected as lipoma of the vulva. The patients had noticed a small but growing mass on their vulva which had been palpated earlier. The masses were excised with a 2 cm resection margin. The final pathological reports confirmed extraskeletal mesenchymal chondrosarcoma (EMC) of the vulva revealing no microscopic lesions on the resection margins. After 24 months of following from the initial diagnosis, the patients remain without evidence of any recurrent. Management of EMC is not well studied due to the rare and variable nature of the disease. However, the surgery, such as we had, is the mainstay of local treatment with studies showing better survival in patients who undergo wide surgical resection. The establishment of adjuvant systemic pharmacotherapy could be expected in the future.
Chondrosarcoma ; Chondrosarcoma, Mesenchymal ; Humans ; Lipoma ; Vulva

No abstract available.
Chondrosarcoma, Mesenchymal* ; Mediastinum*

Mesenchymal chondrosarcoma is a rare disease with poor prognosis. Treatment including wide or radical excision is very important. Radiotherapy and chemotherapy are additional treatment options, but no conclusive results for their efficacy have been shown until date. Imaging modalities can give important clues for diagnosis and management planning. Angioembolization before surgery could be useful as prophylaxis to control intraoperative bleeding, increasing the likelihood of complete resection.
Chondrosarcoma, Mesenchymal ; Rare Diseases

Today, ultrasound imaging is being widely used to assess soft tissue lesions in the maxillofacial region. However, ultrasound investigations of intra-osseous lesions are rare, especially for tumors of the jaws. This report emphasized the capability of this useful imaging modality in identification of the characteristics of malignant conditions involving the bone. Mesenchymal chondrosarcoama, one of the unusual malignant conditions of the jaw, was presented in a young male with significant facial swelling. Different imaging modalities parallel with the histopathologic investigation confirmed the diagnosis. Interestingly, destruction of the bony cortex and new bone formation with a characteristic "sun ray appearance", highly suggestive of sarcomas, was manifested on the ultrasonograph. Thus, this report presented the ultrasonographic features of chondrosarcoma of mandible and considered the ultrasonography to be a useful imaging modality to evaluate intra-osseous jaw lesions.
Chondrosarcoma ; Chondrosarcoma, Mesenchymal ; Humans ; Jaw ; Male ; Mandible ; Osteogenesis ; Sarcoma ; Tomography, X-Ray Computed

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

Extraskeletal mesenchymal chondrosarcomas (EMCs) are relatively uncommon, and a location in the upper extremity, especially in the shoulder or axillary region, is rare. Furthermore, the radiographic findings of EMCs do not show any features that distinguish them from other neoplasms, and therefore, definitive diagnoses are made based on histological features. EMC is an aggressive tumor with a poor prognosis, and requires wide surgical excision. However, its treatment may involve peculiarities such as a difficulty in obtaining a proper surgical margin in the axillary region or shoulder. In this report, the authors present two rare cases of EMCs in the axillary region.
Axilla ; Chondrosarcoma, Mesenchymal ; Prognosis ; Shoulder ; Upper Extremity