Abstract
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.
Highlights
Soft tissue sarcomas are rare tumors that originate from mesenchymal cells
Chondrosarcoma is a soft tissue sarcoma that is histologically characterized by the production of chondroid matrix
Extraskeletal myxoid chondrosarcoma (EMC) is a rare tumor characterized by cords or strands of small cells immersed in a myxoid matrix [1]
Summary
Soft tissue sarcomas are rare tumors that originate from mesenchymal cells. Even though there are multiple distinct histological subtypes, they are usually lumped together in clinical trials to produce adequate sample size. The case presented is an example of the usual presentation of this rare tumor in a patient who opted not to receive systemic treatment, allowing us to see the natural course of this disease. FIGURE 2: 10/2012: One year after resection of primary tumor, computed tomography (CT) lung shows new left lower lung nodule. Pathology did confirm that the nature of this nodule was consistent with the patient’s history of EMC He had surveillance imaging regularly afterward and was found to have multiple small pulmonary nodules in August of 2013 (Figure 3). Routine surveillance imaging since August of 2013 showed a progressive but slow increase of size of the multiple pulmonary nodules as well as the appearance of new nodules (Figures 4-7).
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