Primary intracranial extraskeletal myxoid chondrosarcoma (original) (raw)
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Primary Intracranial Myxoid Chondrosarcoma: A case report
2018
Objective: Intracranial extraskeletal myxoid chondrosarcoma is a very rare tumor; up to date, only ten cases have been reported. Case report: We present a case report of a 65-year-old female admitted to Zagazig university hospitals by right side hemiplegia of gradual onset and progressive course over three weeks. Magnetic resonance imaging (MRI) revealed left intra-axial parieto-occipital space occupying lesion, multiloculated, hypodense with central necrosis, heterogeneous enhancement, minimal brain edema, and causing a mass effect. The patient underwent subtotal resection using sonar guided craniotomy. The histopathological and immunohistochemical evaluation confirmed a diagnosis of extraskeletal myxoid chondrosarcoma. One month after initial discharge, the patient underwent radiotherapy. About 6 months after surgery, the patient was deteriorated and died. Conclusion: Intracranial extraskeletal myxoid chondrosarcoma is a rare malignant cartilaginous tumor. Pathological diagnosis is the gold standard and radical excision is the standard treatment.
Extraskeletal myxoid chondrosarcoma: a case report with adjuvant intraoperative treatment
Journal of surgical case reports, 2020
Extraskeletal myxoid chondrosarcoma is a rare form of malignant mesenchymal neoplasm mainly localized into the limbs, particularly in the thigh and popliteal fossa. It has been classified as a low-grade sarcoma so far, but it shows a tendency to relapse and metastasize. In the early stage of disease, surgery represents the only chance of cure. In case of diffuse metastatic disease, systemic chemotherapy with anthracyclines is the standard of care. In this paper, we present a case of a patient affected by this rare disease and the analysis of radiological, surgical and histopathological aspects.
Extraskeletal Chondrosarcoma: Long-term Follow-up of a Patient with Metastatic Disease
Cureus, 2018
Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma with an indolent course and poor response to systemic treatment. We present a case of a 53-year-old male who presented with right gluteal extraskeletal myxoid chondrosarcoma. He was treated with wide local excision after receiving 50 Gray of neoadjuvant radiation therapy. Three years later he was found to have a left lower lobe lung nodule that was slowly increasing in size. He underwent a left lower lobectomy and the nodule was confirmed to be consistent with the patient's history of EMC. One year later, lung imaging showed multiple small nodules bilaterally consistent with metastatic disease. The patient opted for watchful waiting approach. Routine follow-up imaging for four years shows a very slow progression of his disease burden. He continues to be asymptomatic. This case demonstrates the natural course of EMC and argues in favor of the watchful waiting approach in treating this disease.
Extraskeletal Myxoid Chondrosarcoma: Long-Term Survival in the Setting of Metastatic Disease
Case Reports in Orthopedics
Three cases of extraskeletal myxoid chondrosarcoma (EMC) in patients who presented with pulmonary metastases and were managed with long-term close observation without systemic intervention are presented. Follow-up imaging showed slow progression of their disease over several years, and the patients remained asymptomatic from their pulmonary metastases. This clinical experience provides insight into the natural history of the disease and suggests that some patients may experience long-term survival and remain asymptomatic even without systemic intervention, thereby improving their quality of life by avoiding potentially debilitating treatments.
Clinical Practice, 2018
The extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant accounting for less than 3% of soft tissue tumors. EMC is considered an intermediate aggressive neoplasm with a slow-growing and indolent clinical course. Nevertheless, local recurrence is frequent and distant metastases arise in about 50%-90% of cases. For these patients therapeutic options are scarce due to the poorly sensitivity of EMC to cytotoxic chemotherapy and targeted therapy. We report the case of a caucasian adult man with bulky locoregional and mediastinal disease resistant to antracyclinbased chemotherapy and targeted therapy. Then, a third line chemotherapy with trabectedin was used obtaining temporary disease stabilization. Due to arising of symptoms and modest increase of lesions, radiotherapy was added with four consecutive treatments on right gluteal mass, large right and left paravertebral lesions, and mediastinal bulky disease, respectively. Rapidly, near complete regression of masses was noted. After 41 months from metastasis and 28 after beginning of chemotherapy with trabectedin and complementary radiotherapy, the patient is in good clinical conditions and the disease under control. In our experience, radiotherapy is a valid option in metastatic ECM and when associated with trabectedin a potent synergistic effect could be obtained.
Annals of Surgical Oncology, 2020
Background. Extraskeletal myxoid chondrosarcoma (EMC) is a rare sarcoma of uncertain origin, marked by specific chromosomal translocations involving the NR4A3 gene, and usually characterized by an indolent course. Surgery (with or without radiotherapy) is the treatment of choice in localized disease. The treatment for advanced disease remains uncertain. In order to better evaluate prognostic factors and outcome, a retrospective pooled analysis of patients with EMC treated at three Italian Sarcoma Group (ISG) referral centers was carried out. Methods. All patients with localized EMC surgically treated from 1989 to 2016 were identified. Diagnosis was centrally reviewed according to WHO 2013. Only patients with NR4A3 rearrangement were included. Results. Sixty-seven patients were identified: 13 (20%) female, 54 (80%) male. Median age was 56 years (range 18-84). Numbers and type of translocation were: 50 (80%) NR4A3-EWS, 10 (16%) NR4A3-TAF15, 1 (2%) NR4A3-TCF12, and 1 (2%) NR4A3-TFG. Median follow-up was 55 months (range 2-312). Five-and ten-year overall
Clinical Sarcoma Research
Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue tumor that typically affects the lower limbs of men between the ages of 50 and 60. EMC of the shoulder is rare with a high risk of local recurrence and distant metastasis. A planned surgical excision in sarcoma referral centers (SRCs) is mandatory to obtain the best outcome. The role of chemotherapy (CHT) and Radiotherapy (RT) on soft tissue chondrosarcoma is still controversial. Case presentation A 47-year-old man presented to our referral center with a history of EMC in the right shoulder excised with microscopic positive surgical margins in a non-referral center. Staging imaging exams did not reveal distant metastasis or residual disease, but during follow-up a local recurrence was detected. After a multidisciplinary discussion, preoperative radiotherapy was administered with a total dose of 50 Gy, and then the patient underwent wide surgical excision. Histological examination was negative for viable tumor...
Benefit of Radiotherapy in Extraskeletal Myxoid Chondrosarcoma
American Journal of Clinical Oncology, 2018
Objectives: Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignancy for which the role of radiotherapy is not well-defined. We examine the effect of external beam radiotherapy (EBRT) on cancerspecific survival (CSS) for patients with localized EMC, in a propensity score weighted, population-based analysis. Materials and Methods: The Surveillance, Epidemiology, and End Results database (1973 to 2012) was queried for cases of localized EMC arising from soft connective tissues of the trunk and extremities treated with surgery and/or EBRT. Inverse probability treatment weighting was utilized, with survival analysis by weighted Cox regression and Kaplan-Meier analysis with log-rank testing. The primary endpoint was CSS. Results: One hundred seventy-two patients were identified, diagnosed from 2004 to 2012. Ninety-four percent and 32% of 156 assessable patients underwent surgery and EBRT, respectively. By inverse probability treatment weighting, balancing covariates of age group, sex, race, grade, T stage, N stage, receipt of surgery, and anatomic site, we observed CSS of 97% versus 85% and 94% versus 85% in patients receiving EBRT versus no EBRT, at 3 and 5 years, respectively, at median follow-up of 33 months, P = 0.01. A trend toward an overall survival benefit associated with EBRT was noted, P = 0.06. Further adjusting for type of resection performed, CSS benefit persisted, 97% versus 85% at 3 years and 94% versus 85% at 5 years, P = 0.02, with trend toward an overall survival benefit, P = 0.08. Conclusions: The receipt of EBRT is associated with a CSS benefit in localized EMC. Aggressive local therapy, including EBRT, should be considered in these patients.