Abstract
BackgroundExtraskeletal 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 presentationA 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 cells. No relapse occurred in a 24-months post-operative follow up.ConclusionsThe case here described suggests the importance of patient’s management in SRCs. A planned combined treatments with both surgery and RT seems to be the best choice to improve local control. RT seems to be promising within this specific histotype. Further studies are needed to confirm if the observed efficacy of combined treatments reflects in a consistent survival benefit for EMC patients.
Highlights
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
Further studies are needed to confirm if the observed efficacy of combined treatments reflects in a consistent survival benefit for EMC patients
There is no scientific agreement about the effectiveness of chemotherapy (CHT) and RT on soft tissue chondrosarcoma [8,9,10,11,12] and a very small number of cases and studies that may guide local management strategies for patients with EMC; have been reported; for this reason, management of these patients in sarcoma referral centers (SRCs) is strongly recommended since they are associated with a significantly better outcome; each therapeutic decision has to be taken within a dedicated multidisciplinary tumor board (MTB) [7, 13]
Summary
In EMC, as in all STS, an optimal treatment strategy is crucial to guarantee the best outcomes for the patients. Patients evaluation in referral centers is mandatory to ensure accurate diagnosis and management. A multidisciplinary approach targeted on the single case characteristics of the specific case is the cornerstone to obtain the best oncologic and functional outcome for the patient. In agreement with literature data, our histopathological findings suggest RT to be promising to achieve the highest chance for local control and prolonged survival, especially in EMC patients. Further studies are needed to confirm if the improved local control guaranteed by combined RT and wide surgical excision will reflect in a consistent survival benefit for EMC patients and if it is possible to identify which are the patients who could have the most benefits from radiotherapy
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