Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy respo
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Clinical Sarcoma Research Open Access
CASE REPORT
Locally recurrent extraskeletal myxoid chondrosarcoma of the shoulder: a case of complete neoadjuvant radiotherapy response Luca Improta1, Sergio Valeri1*, Rossana Alloni1, Chiara Pagnoni1, Francesco Mallozzi Santa Maria1, Beniamino Brunetti1, Carlo Greco1, Irene Aprile2, Mirella Maselli1, Bruno Vincenzi1 and Alessandro Gronchi3
Abstract 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 cells. No relapse occurred in a 24-months post-operative follow up. Conclusions: The 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. Keywords: Soft tissue sarcoma (STS), Sarcoma referral centers (SRCs), Extraskeletal myxoid chondrosarcoma (EMC), Preoperative radiotherapy, Wide surgical margins Background Extraskeletal myxoid chondrosarcoma (EMC) is a rare malignant soft tissue tumor with a high risk of local recurrence and metastasis but with an indolent course [1]. Literature data reveal EMC to have an indolent but resilient course with late risk of metastatic progression and local recurrence in the vast majority of patients [2, 3]. Typically, it affects patients between the ages of about *Correspondence: [email protected] 1 Campus Bio-Medico University of Rome, Via Alvaro del Portillo, 200, 00128 Roma, RM, Italy Full list of author information is available at the end of the article
50 and 60 years, more frequently men than women. EMC has a reported overall survival rate at 5 years of 82%, at 10 years of 65%, at 15 years of 58%. The lower limbs are the most common site of EMC while the shoulder is an extremely rare localization [4, 5].The most common site of metastasis is the lung ( more than half of all patients); moreover metastases in deep soft tissue, lymph node, bone and brain have also been reporte
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