Extraskeletal Chondrosarcoma: Long-term Follow-up of a Patient with Metastatic Disease

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.

A rare case of primary ovarian mesenchymal chondrosarcoma in pregnancy

“…The most common sites of origin of chondrosarcoma are the lower extremities, which are known as the primary sites, followed by the meninges, lung, neck, mandible, heart, and orbit. It is more common in women and mainly presents in the second to third decades of life [4,5]. Although most cases of uterine chondrosarcoma have been reported in postmenopausal women, our case was in reproductive age.…”

“…There is no strong evidence favoring a specific optimal option for systemic therapy. Given the aggressive nature of this tumor and poor response to treatment, some experts prefer watchful waiting in metastatic cases following wide local excision [5]. Due to the rarity of this entity, there is no established treatment protocol, and we speculated that this chemotherapy might be of benefit in this case, as the tumor was grade 3 and had a high mitotic rate.…”

“…Overall, no single best stain for this tumor has been identified, and a group of stains should be used to reach a certain diagnosis. Molecular genetic characteristics of these tumors can now be detected, including translocation (9;22) (q22; q11), resulting in the EWSR1/NR4A3 sequence in the majority of patients [5]. Wide and adequate resection of the involved area to treat localized disease is the most promising treatment option for EMC, followed by adjuvant systemic chemotherapy to treat metastatic cases.…”

“…Wide and adequate resection of the involved area to treat localized disease is the most promising treatment option for EMC, followed by adjuvant systemic chemotherapy to treat metastatic cases. Adjuvant chemotherapy has been reported to increase the survival rate in some studies, but there is not enough evidence to reach a consensus on this matter [4,5,7]. In general, EMC is known as a highly potent tumor for distant metastasis.…”

Extraskeletal Myxoid Chondrosarcoma: Long-Term Survival in the Setting of Metastatic Disease

“…Supporting the findings in this report is one published case report of a patient with EMC who developed multiple pulmonary metastases after initial resection of their primary tumor and a solitary pulmonary nodule. This patient was followed with close observation for four years, remaining asymptomatic with slow progression of their pulmonary nodules [ 9 ]. Despite the lack of effectiveness of systemic therapies in treating EMC, patients who present with metastatic disease are often managed with a range of chemotherapeutic agents and radiation therapy, which can carry severe side effects and possess an inherent morbidity [ 4 , 7 ].…”