Extraskeletal Myxoid Chondrosarcoma: A Comparative Study of Imaging and Pathology

Abstract

The purpose of this study was to achieve better understanding of extraskeletal myxoid chondrosarcoma (EMC). 13 cases of EMC confirmed by surgery biopsy were retrospectively studied. All patients underwent preoperative CT or/and MRI examinations. Among six patients who underwent preoperative CT examinations, six cases of lesions exhibited hypodensity on unenhanced image, three cases of tumor showed funicular spots or patchy calcification, and four cases of tumor did not show any obvious enhancement after enhanced CT scan. Among ten patients who underwent preoperative MRI examination, 8 cases of tumor revealed uniform or slight hyposignal intensity on T1WI, 10 cases of tumor demonstrated lobulated hypersignal intensity with multiple low signal intensity of interval septa on T2WI, and 5 cases of lesions indicated characteristic appearance: septa enhancement with tumor stroma between interval septa being unenhanced. EMC usually occurred at older men and at certain location such as limbs, trunk, and subcutaneous tissues. EMC usually exhibited low density mass (mostly 20-40HU) with calcification and in a portion of the cases showed light or no enhancement on CT. On MRI, EMC showed lobulated hypersignal intensity on T2WI with characteristic arc, septa, or interval septa enhancement.

Postoperative Radiation Therapy in Patients with Extracranial Chondrosarcoma: A Joint Study of the French Sarcoma Group and Rare Cancer Network

“…Of 52 (28.6%) myxoid CHS, 41 EMC were however included as CHS by centers belonging to the GSF-GETO, with histology being centrally-reviewed by expert pathologists. This histological subtype is characterized by the translocation t(9;22)(q22;q12), resulting from the fusion of EWSR1 and NRA3 [24] or a translocation between NRA3 and TAF15 or TCF12 [24][25][26].…”

<p>Low-Grade Chondrosarcoma In The Sellar Area: Case Report And Literature Review</p>

“…Owing to the pathological features of the myxoid matrix, small round nuclei, and cytoplasm vacuoles, LGC can easily be misdiagnosed as chordoma17 and extraskeletal myxoid chondrosarcoma (EMC),18 but the biological behaviors, treatment, and prognosis of these diseases differ, emphasizing the importance of accurate identification. Chordomas and chondrosarcomas are two major malignant bone neoplasms occurring at the skull base;17 chordomas display distinct lobulation, the tumor cells and matrix are separated by fibrous bands, tumor cells form a web or strands, and the fibrous septum is a classic feature.…”

Ectomesenchymal chondromyxoid tumour: an uncommon characteristic tumour of the anterior tongue

“…Other areas where this tumour has been found include the base of the tongue and hard palate; however, occurrence of the tumour on the hard palate is still debated 1. A malignant aspect of ECMT is extraskeletal myxoid chondrosarcoma, which is extremely rare but has been reported in the head and neck 9…”