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Cancer Jan 1994Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all... (Review)
Review
BACKGROUND
Extraskeletal mesenchymal chondrosarcoma is a rare tumor characterized by undifferentiated mesenchymal cells with islands of mature hyaline cartilage. Considering all sites, long-term survival is approximately 30%. Only seven cases of orbital mesenchymal chondrosarcoma have been reported.
METHODS
The records of three cases of orbital mesenchymal chondrosarcoma treated at the Columbia-Presbyterian Medical Center, and the seven previously reported cases of this tumor were reviewed to determine clinical characteristics and appropriate therapy.
RESULTS
Nine of the 10 patients were female; age of onset ranged from 10 to 35 years. Of eight patients with at least 2 years of observation, five survived 5 or more years after resection, or after resection plus adjuvant therapy. Two patients died of metastatic disease 2 and 5 years, respectively, after the initial treatment, and one died of pneumonia 6 months after surgery. Review of the histology of the three cases treated at Columbia-Presbyterian Medical Center did not identify distinct histologic types that might guide therapy. Presenting symptoms were typical of symptoms of an orbital mass: proptosis, pain, diplopia, change in visual acuity, ptosis, and tearing.
CONCLUSION
The small number of reported cases of mesenchymal chondrosarcoma of the orbit prevents definitive conclusions, but it appears that resection is adequate therapy in some cases. Extraskeletal mesenchymal chondrosarcoma of the orbit may have a better prognosis than tumors in other sites.
Topics: Adolescent; Adult; Child; Chondrosarcoma; Female; Humans; Male; Orbital Neoplasms
PubMed: 8293407
DOI: 10.1002/1097-0142(19940115)73:2<399::aid-cncr2820730227>3.0.co;2-g -
The Journal of Laryngology and Otology Sep 1994We report, to our knowledge, the 10th recorded case of mesenchymal chondrosarcoma (MC) occurring in the maxilla. Our case is the youngest person reported with a tumour... (Review)
Review
We report, to our knowledge, the 10th recorded case of mesenchymal chondrosarcoma (MC) occurring in the maxilla. Our case is the youngest person reported with a tumour in this location. The prognosis for cure is poor with a high incidence of local recurrence as well as metastases. Treatment is based on radical surgery. Radiotherapy and chemotherapy have a adjuvant role but additional experience with this tumour is required to define the most efficacious treatment.
Topics: Adolescent; Chondrosarcoma, Mesenchymal; Combined Modality Therapy; Female; Humans; Maxillary Neoplasms; Tomography, X-Ray Computed
PubMed: 7964146
DOI: 10.1017/s0022215100128129 -
Journal of Neuropathology and... Sep 2020Mesenchymal chondrosarcoma (MC) is a rare aggressive mesenchymal sarcoma. Specific markers for the differential diagnosis of MCs remain to be developed. OLIG2 expression...
Mesenchymal chondrosarcoma (MC) is a rare aggressive mesenchymal sarcoma. Specific markers for the differential diagnosis of MCs remain to be developed. OLIG2 expression has been reported only in neuroepithelial tumors. Recently, OLIG2 expression was found to be involved in the development of NCOA2 fusion-positive alveolar rhabdomyosarcomas. Therefore, we investigated whether OLIG2 expression could be used as a diagnostic marker for MC. We report the clinical pathological and immunohistochemical features of 14 MCs. All tumors showed typical pathological features including biphasic patterns with sheets of primitive round mesenchymal cells and interspersed islands of cartilage. These tumors expressed BCL2, SOX9, and CD99. OLIG2 was robustly expressed in 12/14 of MCs. NCOA2 rearrangement was found in 12 cases. OLIG2 expression was not found in the NCOA2 rearrangement-negative MCs. Notably, OLIG2 expression was not detected in 52 neoplasms (8 Ewing sarcomas, 23 hemangiopericytomas, and 21 chondrosarcomas) that are frequently misdiagnosed as MC. Our findings provide convincing evidence that OLIG2 can serve as a reliable marker in the differential diagnosis of MC and may be a unique neurodevelopmental gene expression signature for the NCOA2 rearranged MCs.
Topics: Adult; Biomarkers, Tumor; Bone Neoplasms; Child; Chondrosarcoma, Mesenchymal; Female; Gene Rearrangement; Humans; Immunohistochemistry; Male; Middle Aged; Nuclear Receptor Coactivator 2; Oligodendrocyte Transcription Factor 2; Young Adult
PubMed: 32770197
DOI: 10.1093/jnen/nlaa079 -
Pediatric Hematology and Oncology Oct 2010Mesenchymal chondrosarcomas are rare malignant tumors in pediatric age group. The authors present a case of mesenchymal chondrosarcoma located in the sacrum in a... (Review)
Review
Mesenchymal chondrosarcomas are rare malignant tumors in pediatric age group. The authors present a case of mesenchymal chondrosarcoma located in the sacrum in a 10-year-old-girl that was successfully treated with chemotherapy and radiotherapy after surgical excision. According to the authors' literature search, the patient is the first reported case of pediatric sacral primary mesenchymal chondrosarcoma. Mesenchymal chondrosarcoma cases in pediatric age group published in English literature was reviewed.
Topics: Antineoplastic Combined Chemotherapy Protocols; Child; Chondrosarcoma, Mesenchymal; Female; Humans; Male; Sacrum; Spinal Neoplasms; Treatment Outcome
PubMed: 20677924
DOI: 10.3109/08880018.2010.503333 -
Cureus Sep 2023Mesenchymal chondrosarcomas are extremely rare and aggressive tumors that primarily affect patients between the ages of 20 and 30. These neoplasms are typically found in...
Mesenchymal chondrosarcomas are extremely rare and aggressive tumors that primarily affect patients between the ages of 20 and 30. These neoplasms are typically found in the lower limbs and cranial region. Their occurrence within soft tissues is exceedingly rare, and the initial presentation often includes immediate metastatic dissemination. Given the extraordinarily low prevalence of extraskeletal mesenchymal chondrosarcoma, treatment approaches remain non-standardized. Surgical resection combined with neoadjuvant chemotherapy or radiotherapy is the most commonly favored strategy by medical teams. In this case report, we present the case of a 72-year-old patient with no specific medical history, who presented with a non-metastatic extraskeletal mesenchymal chondrosarcoma located in the popliteal fossa. The therapeutic intervention encompassed surgical resection followed by adjuvant radiotherapy. After 18 months of follow-up period, there was no evidence of local recurrence or distant metastases. The disparity between the patient's clinical characteristics and the existing medical literature may provide new insights into understanding this neoplastic entity.
PubMed: 37900409
DOI: 10.7759/cureus.45974 -
Canadian Journal of Ophthalmology.... Oct 2018
Topics: Adolescent; Biopsy; Chondrosarcoma, Mesenchymal; Diagnosis, Differential; Female; Humans; Magnetic Resonance Imaging; Ophthalmologic Surgical Procedures; Orbital Neoplasms; Tomography, Emission-Computed, Single-Photon; Tomography, X-Ray Computed; Ultrasonography
PubMed: 30340745
DOI: 10.1016/j.jcjo.2017.11.008 -
Cancer Reports (Hoboken, N.J.) Jan 2022Mesenchymal chondrosarcoma is a rare and aggressive bone tumor with few reports of primary tumor in the chest wall.
BACKGROUND
Mesenchymal chondrosarcoma is a rare and aggressive bone tumor with few reports of primary tumor in the chest wall.
CASE
We report a case of a 17-year-old male presenting with back pain and a posterior mediastinal mass. Imaging demonstrated what was thought to be a benign chondral tumor. The patient underwent resection which confirmed extraskeletal mesenchymal chondrosarcoma. The patient declined proposed adjuvant chemotherapy and underwent multiple resections for rapid local reoccurrence. He ultimately elected for hospice care.
CONCLUSION
The case highlights the importance of close disease monitoring and exploration of treatment options, given lack of established guidelines and consistent tumor features.
Topics: Adolescent; Bone Neoplasms; Chondrosarcoma, Mesenchymal; Humans; Male; Thoracic Wall; Tomography, X-Ray Computed
PubMed: 34132499
DOI: 10.1002/cnr2.1453 -
International Journal of Oral and... Oct 1998Mesenchymal chondrosarcoma (MCS), described in 1959 by Lichtenstein & Bernstein, is a very rare malignant tumour. Only 46 cases have been previously reported in the... (Review)
Review
Mesenchymal chondrosarcoma (MCS), described in 1959 by Lichtenstein & Bernstein, is a very rare malignant tumour. Only 46 cases have been previously reported in the jaws. Over a period of 30 years, four chondrosarcomas of mesenchymal type (of which one has already been published in 1987 with a three-month follow up) were treated in our department. Two patients have been followed for more than 17 years, of whom one has died of an intracranial extension and the other is still alive after surgical treatment of numerous recurrences and metastases. The two others are tumour-free with a follow up of 18 months and 11 years respectively. Histological, epidemiological and clinical features and treatment of MCS are discussed relevant to the data published in the literature.
Topics: Adolescent; Adult; Chondrosarcoma, Mesenchymal; Female; Humans; Jaw Neoplasms; Male; Prognosis; Survivors
PubMed: 9804199
DOI: 10.1016/s0901-5027(98)80065-9 -
European Journal of Cardio-thoracic... Oct 2020Metastasis of chondrosarcoma of skeletal origin to the heart is uncommonly reported in the literature, with the majority of cases involving right atrial metastases....
Metastasis of chondrosarcoma of skeletal origin to the heart is uncommonly reported in the literature, with the majority of cases involving right atrial metastases. Surgical resection remains the mainstay of treatment, and the literature has shown improved median survival with this form of therapy, possibly by reducing thromboembolic risk in this patient population. 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. This is the first report of surgical resection of left atrial metastatic disease prior to the onset of thromboembolic sequelae.
Topics: Bone Neoplasms; Chondrosarcoma; Chondrosarcoma, Mesenchymal; Heart Atria; Humans; Metastasectomy
PubMed: 32413904
DOI: 10.1093/ejcts/ezaa116 -
Asian Journal of Surgery Apr 2023
Topics: Male; Humans; Chondrosarcoma, Mesenchymal; Prostate; Pelvis
PubMed: 36347744
DOI: 10.1016/j.asjsur.2022.10.056