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. 2022 Aug;52(4):1183-1189.
doi: 10.55730/1300-0144.5422. Epub 2022 Aug 10.

Surgical outcomes of extraskeletal myxoid chondrosarcoma

Coşkun Ulucaköy  1 , İsmail Burak Atalay  1 , Aliekber Yapar  2 , Ahmet Yiğit Kaptan  3 , İzzet Bingöl  1 , Mehmet Doğan  4 , Mehmet Fatih Ekşioğlu  1
Affiliations

Surgical outcomes of extraskeletal myxoid chondrosarcoma

Coşkun Ulucaköy et al. Turk J Med Sci. 2022 Aug.

Abstract

Background: Extraskeletal myxoid chondrosarcoma (EMC) is a rare soft tissue sarcoma. The aim of this study is to present the results of the patients we treated with the diagnosis of EMC as an oncology reference center.

Methods: Information on 13 patients diagnosed with EMC between 2006 and 2018 was retrospectively reviewed. Patients' demographic information, tumor sizes, surgical treatments, chemotherapy and radiotherapy statuses, follow-up times, recurrences, and metastases were recorded.

Results: Mean patient age was 53.6 ± 15 years (range: 28-73). In 8 patients, the tumor was located in the lower limbs, most commonly in the thigh (46.2%). Mean follow-up period was 52.8 ± 19.9 (24-96) months. All patients underwent wide resections and only one had a positive surgical margin. In follow-up, 5 (38.5%) patients experienced recurrence; 6 patients had lung metastasis (46.2%) and 7 patients (53.8%) died. Mean tumor size was 10.4 ± 3.2 (5-17) cm. Median survival time was 61 (50.5-71.4) months and 5-year survival rate was 51.8%. There was no significant difference between survival times according to age, gender, side, limb location, postoperative radiotherapy, recurrence, or presence of lung metastasis. The cut-off value for death obtained by ROC analysis of tumor size was 11 cm.

Discussion: EMC is a rare soft tissue sarcoma with high local recurrence and metastasis capacity. Tumor size and metastatic disease are poor prognostic criteria. If it is a localized disease, the first option should be wide resection.

Keywords: Extraskeletal; chondrosarcoma; metastasis; recurrence; tumor.

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Conflict of interest statement

Conflict of interest

The authors declare that they have no conflicts of interest.

Figures

Figure 1
Figure 1
A) Combined view of undifferentiated areas of hyaline cartilage and small cells in the tumor (magnification 100×, H&E); B) Small undifferentiated cell component (magnification 400×, H&E); C) Immunohistochemical S100 positivity in cartilaginous areas (magnification 200×).
Figure 2
Figure 2
Receiver operating characteristic (ROC) curves for tumor size (PPV: positive predictive value, NPV: negative predictive value).
Figure 3
Figure 3
OS curve of patients according to tumor size groups.
Figure 4
Figure 4
OS curve of patients according to CT groups.
See this image and copyright information in PMC

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Supplementary concepts