Mesenchymal Chondrosarcoma

A rare aggressive variant of chondrosarcoma, characterized by a biphasic histologic pattern of small compact cells intermixed with islands of cartilaginous matrix. Mesenchymal chondrosarcomas have a predilection for flat bones; long tubular bones are rarely affected. They tend to occur in the younger age group and are highly metastatic. (DeVita Jr et al., Cancer: Principles & Practice of Oncology, 3d ed, p1456)

Also Known As:

Networked: 100 relevant articles (6 outcomes, 10 trials/studies)

Relationship Network

Disease Context: Research Results

Related Diseases

1.	Chondrosarcoma
2.	Neoplasm Metastasis (Metastasis)
3.	Neoplasms (Cancer)
4.	Osteosarcoma (Osteogenic Sarcoma)
5.	Sarcoma (Soft Tissue Sarcoma)

Experts

1.	Bovée, Judith V M G: 3 articles (01/2019 - 03/2008)
2.	Gelderblom, Hans: 3 articles (01/2019 - 03/2008)
3.	Wang, Lu: 2 articles (01/2022 - 02/2012)
4.	He, Tao: 2 articles (03/2021 - 01/2020)
5.	Lewis, Valerae O: 2 articles (01/2021 - 03/2014)
6.	Motoi, Toru: 2 articles (01/2021 - 02/2012)
7.	Aubert, Sébastien: 2 articles (10/2020 - 01/2009)
8.	Schwab, Joseph H: 2 articles (10/2020 - 12/2016)
9.	Horiuchi, Keisuke: 2 articles (01/2016 - 12/2012)
10.	Morioka, Hideo: 2 articles (01/2016 - 12/2012)

Drugs and Biologics

Drugs and Important Biological Agents (IBA) related to Mesenchymal Chondrosarcoma:

1.	Docetaxel (Taxotere)FDA Link 11/01/2008 - "Efficacy of weekly docetaxel and bevacizumab in mesenchymal chondrosarcoma: a new theranostic method combining xenografted biopsies with a mathematical model."
2.	Bevacizumab (Avastin)FDA Link 11/01/2008 - "Efficacy of weekly docetaxel and bevacizumab in mesenchymal chondrosarcoma: a new theranostic method combining xenografted biopsies with a mathematical model."
3.	Proteins (Proteins, Gene)FDA Link 01/01/2022 - "We previously described the HEY1-NCOA2 as a recurrent gene fusion in mesenchymal chondrosarcoma, an important breakthrough for characterizing this disease; however, little study had been done to characterize the fusion protein functionally, in large part due to a lack of suitable models for evaluating the impact of HEY1-NCOA2 expression in the appropriate cellular context. " 12/01/2001 - "However, phenotype similarities with other chondromyxoid tumours that also express the protein, like mesenchymal chondrosarcomas, suggest a relationship between MIC-2 reactivity and morphological differentiation." 01/01/2003 - "This study characterizes 3 cases of mesenchymal chondrosarcoma (MC) utilizing a proteomic approach that allows for the detection, visual quantification, cellular compartmentalization, and assessment of the functional state of certain proteins that may promote tumor growth and/or oppose apoptosis. "
4.	TrabectedinIBA 01/01/2020 - "This report aims to present a comprehensive review into available and newer treatment choices for mesenchymal chondrosarcoma, and to highlight trabectedin monotherapy as a possible therapeutic option for mesenchymal chondrosarcoma in the relapsed setting." 01/01/2020 - "Long lasting response with trabectedin monotherapy in relapsed metastatic mesenchymal chondrosarcoma." 01/01/2016 - "Results of sub-analysis of a phase 2 study on trabectedin treatment for extraskeletal myxoid chondrosarcoma and mesenchymal chondrosarcoma." 01/01/2016 - "Studies are also being conducted to expand the use of trabectedin not only as a first-line cancer drug, but also for a number of other clinical indications, for example, in the case of mesenchymal chondrosarcoma, for which trabectedin has been reported to be exceptionally active. "
5.	S100 Proteins (S 100 Protein)IBA 02/15/1988 - "S-100 protein was positive in both polygonal stromal cells and chondrocytes of chondroblastomas and in chondrocytes of mesenchymal chondrosarcoma. " 11/01/1984 - "Twelve cartilaginous tumors were studied by electron microscopy and the presence of S-100 protein was studied immunohistochemically in order to clarify the cell origin of chondroblastoma, chondromyxoid fibroma, and mesenchymal chondrosarcoma. " 09/01/1990 - "The small cells of mesenchymal chondrosarcoma failed to express S100 protein, whereas all components of the tumors (small cells, lacunar chondroblasts, and chondroid matrix) stained for Leu-7 antigen in six cases. " 11/15/1983 - "Using the peroxidase-antiperoxidase (PAP) immunohistochemical method, S-100 protein was found in well-differentiated chondrocytes of chondroma, chondroblastoma, mesenchymal chondrosarcoma, and osteosarcoma. " 11/01/1993 - "The value of immunohistochemical studies appears to lie particularly in the use of antibodies to LCA and S-100 protein to distinguish lymphoma and mesenchymal chondrosarcoma, and perhaps antibody to HBA71 to distinguish neural family lesions (such as Ewing's sarcoma), from other small cell tumors, such as small cell osteosarcoma."
6.	DesminIBA 10/01/2000 - "This study showed that, in addition to cartilaginous differentiation, mesenchymal chondrosarcomas may exhibit focal expression of desmin. " 10/18/2006 - "The clinical material, pathological and immunohistochemical characteristics (reaction to LCA, CD3, CD20, CD45RO, CD79a, CD99, NSE, S-100, Syn, CgA, CK7, CK19, EMA, Coll-II, Sarcomeric-Actin, Desmin, Ki-67,P53) of 2 cases of soft tissue mesenchymal chondrosarcoma in Jishuitan Hospital between 1995 and 2005 were reviewed and followed up. " 02/01/2010 - "Immunoprofile of mesenchymal chondrosarcoma: aberrant desmin and EMA expression, retention of INI1, and negative estrogen receptor in 22 female-predominant central nervous system and musculoskeletal cases." 01/01/2006 - "Desmin and myogenin reactivity in mesenchymal chondrosarcoma: a potential diagnostic pitfall." 02/01/2010 - "By immunohistochemistry, mesenchymal chondrosarcoma occasionally expresses aberrant desmin and EMA but is negative for SMA, myogenin MyoD1, GFAP, and keratins, refuting true smooth or skeletal muscle, epithelial, or meningothelial phenotype. "
7.	VimentinIBA 05/01/2004 - "Histologic examination demonstrated an admixture of undifferentiated mesenchymal cells and islands of mature hyaline cartilage, and immunohistochemistry studies revealing positivity for vimentin and S-100 were consistent with the diagnosis of mesenchymal chondrosarcoma. " 04/01/2005 - "A diagnosis of extraskeletal mesenchymal chondrosarcoma was made based on the histological pattern - characterized by the coexistence of cartilaginous islands and undifferentiated mesenchymal cells, results of Alcian blue staining at various pH, immunohistochemical reactivity against vimentin and S-100, and the absence of skeletal involvement or other primary tumour sites. "
8.	Matrix Metalloproteinase 14 (MT1-MMP)IBA 10/01/1999 - "The undifferentiated small round tumor cells of mesenchymal chondrosarcoma showed lower positive rates and extent scores for MT1-MMP (2/7, 0.7 +/- 0.5) and MMP-2 (3/7, 0.7 +/- 0.4) than for cartilaginous components of mesenchymal chondrosarcoma [MT1-MMP (4/7, 1.3 +/- 0.5) and MMP-2 (7/7, 1.9 +/- 0.3)] or conventional chondrosarcoma. " 10/01/1999 - "In this study, the expressions of MT1-MMP, MMP-2 and TIMP-2 were evaluated in 10 enchondromas, 34 conventional chondrosarcomas, 5 clear-cell chondrosarcomas, 7 mesenchymal chondrosarcomas and 8 dedifferentiated chondrosarcomas. "
9.	Collagen Type II (Type II Collagen)IBA 08/01/2005 - "Thus, the presence of type II collagen in the extracellular tumor matrix significantly facilitates the diagnosis of mesenchymal chondrosarcomas in the absence of histologically visible chondroid matrix formation." 08/01/2005 - "Type II collagen as specific marker for mesenchymal chondrosarcomas compared to other small cell sarcomas of the skeleton." 08/01/2005 - "In our study on a large series of mesenchymal chondrosarcomas (n=30) and other small cell sarcomas (Ewing's sarcomas (n=12), synovial sarcomas (n=6), hemangiopericytomas (n=5), small cell osteosarcomas (n=3), and desmoplastic small round cell tumors (n=1)), we could establish the presence of type II collagen in the extracellular tumor matrix of the small cell areas of mesenchymal chondrosarcomas as a specific and sensitive marker to identify mesenchymal chondrosarcomas and to exclude other small cell neoplasms (except chondroblastic areas in small cell osteosarcomas). "
10.	Tissue Inhibitor of Metalloproteinase-2IBA 10/01/1999 - "In this study, the expressions of MT1-MMP, MMP-2 and TIMP-2 were evaluated in 10 enchondromas, 34 conventional chondrosarcomas, 5 clear-cell chondrosarcomas, 7 mesenchymal chondrosarcomas and 8 dedifferentiated chondrosarcomas. "

Therapies and Procedures

1.	Drug Therapy (Chemotherapy) 01/01/2021 - "Adjuvant treatment can be beneficial for some subtypes such as chemotherapy for dedifferentiated and mesenchymal chondrosarcoma and radiation additionally for mesenchymal chondrosarcoma. " 01/01/2023 - "Total resection of mesenchymal chondrosarcomas is the gold standard for treatment and is typically followed by radiation and/or chemotherapy. " 09/01/2007 - "In this study, we describe a case of cardiac mesenchymal chondrosarcoma that responded to chemotherapy following surgical biopsy. " 02/01/2020 - "For mesenchymal chondrosarcomas, there may be a role for chemotherapy, though data on this is limited.Prognosis" 01/01/2019 - "Patients diagnosed with mesenchymal chondrosarcoma were all treated with multidrug chemotherapy, and the mean PFS was 6.7 months. "
2.	Precision Medicine 11/01/2008 - "The paucity of clinical treatment data on rare tumors, such as mesenchymal chondrosarcoma (MCS), emphasizes the need in theranostic tools for these diseases. " 11/01/2008 - "Efficacy of weekly docetaxel and bevacizumab in mesenchymal chondrosarcoma: a new theranostic method combining xenografted biopsies with a mathematical model."
3.	Therapeutics 01/01/2022 - "Multimodal therapy in the management of primary orbital mesenchymal chondrosarcoma." 10/01/2020 - "We report the case of a patient with metastatic mesenchymal chondrosarcoma of the left atrium who underwent resection, following a lack of response to anticoagulation therapy. " 01/01/2020 - "Although uncommon, intraspinal mesenchymal chondrosarcomas warrant both radical local resection and aggressive adjuvant therapy with chemoradiation to provide the greatest chance of progression-free survival." 01/01/2020 - "Intraspinal mesenchymal chondrosarcoma: An argument for aggressive local resection and adjuvant therapy based on review of the literature." 01/01/2020 - "The patient, who had been diagnosed of mesenchymal chondrosarcoma of maxillary sinus for 3 years, received surgical treatment of palliative endoscopic-assisted total left maxillary resection via mini Caldwell-Luc approach, and palliative enlarged resection due to the progress of residual lesions, followed by no adjuvant therapy. "
4.	Radiotherapy 12/01/2016 - "Twenty-three patients with mesenchymal chondrosarcomas were treated with surgery and radiation therapy (min. dose 44 Gy; max. " 12/01/2016 - "Clinical outcomes for patients after surgery and radiation therapy for mesenchymal chondrosarcomas." 03/01/2014 - "Radiation therapy is associated with fewer recurrences in mesenchymal chondrosarcoma." 06/01/1981 - "[A case of intracranial mesenchymal chondrosarcoma--changes observed by computed tomography before and after radiotherapy (author's transl)]." 12/01/2016 - "We report the outcome of 23 patients with mesenchymal chondrosarcomas treated with surgery and radiation therapy +/- chemotherapy. "
5.	Nephrectomy 01/01/2020 - "After a right nephrectomy was performed, the histopathological examination confirmed the diagnosis to be primary extraskeletal mesenchymal chondrosarcoma. " 11/01/2019 - "Robot-assisted radical nephrectomy for primary renal mesenchymal chondrosarcoma: case report and literature review." 05/01/2017 - "We report a 22-year-old male with a right renal mass; after radical nephrectomy, pathologic examination revealed primary extra skeletal mesenchymal chondrosarcoma." 04/01/2014 - "Right nephrectomy was performed and the histopathological examination confirmed the diagnosis to be renal mesenchymal chondrosarcoma. " 03/01/2015 - "Pathologic assessment of the open left radical nephrectomy specimen revealed a primary renal mesenchymal chondrosarcoma, a tumor that has only been documented in 5 prior case reports in the literature. "