Clinical and radiologic features of extraskeletal myxoid chondrosarcoma including initial presentation, local recurrence, and metastases

Abstract

BackgroundThe aim of the study was to evaluate the clinical and imaging features of extraskeletal myxoid chondrosarcoma (EMC) including initial presentation, recurrence, and metastases.Patients and methods.In this institutional review board-approved retrospective study, imaging features of 13 patients with pathologically proven EMC seen from August 1995 to December 2011 were analyzed. The group included 3 women and 10 men and the mean age was 54 years (range 29–73 years). Imaging studies were evaluated by two radiologists in consensus. Location, size, and imaging features of primary tumors were recorded as well as the presence of recurrent disease and location of metastases.ResultsAmong 13 patients, 3 died during the timeframe of this study. Nine patients had primary tumor in the lower extremity, and average tumor size was 9.3 cm (range 3.3–18 cm). On MRI, primary tumors were hyperintense on T2, isointense to muscle on T1, and demonstrated peripheral/septal enhancement. Three patients had local recurrence and 12 had metastatic disease, with lung involvement being the most common. Tumor density on contrast enhanced CT ranged from 8.2 to 82.9 Hounsfield unit (HU). FDG-PET/CT imaging was performed in 3 patients. One patient had no FDG avid disease and 2 patients had metastatic disease with standard uptake values (SUV) of 2.8 and 7.4. The patient with intense FDG uptake demonstrated more solid appearing tumor burden and had the shortest survival.ConclusionsEMC is a rare tumor that often occurs in the lower extremities and frequently metastasizes to the lungs. Increased tumor density and increased FDG uptake may be related to more aggressive disease.

Nonepithelial Neoplasms of the Pancreas, Part 2: Malignant Tumors and Tumors of Uncertain Malignant PotentialFrom the Radiologic Pathology Archives

“…Because of their similar morphology, immunohistochemistry, and cytogenetic alterations (t [11;22]), these tumors are documented together with the Ewing sarcoma family (37). prognostic information for tumor grading (increased uptake correlates with higher grade) and predicting risk for local recurrence or metastatic disease (35,36). Like other types of sarcoma, they manifest as large solid masses with frequent cystic degeneration and necrosis ( Fig 10) (13,38,39).…”

Extraskeletal Myxoid Chondrosarcoma: A Comparative Study of Imaging and Pathology

“…Typically, distinctive enhancements were not observed in enhanced CT scans. Neena [ 10 ] mentioned that EMC showed mild enhancement in enhanced CT scans. Zhang [ 22 ] reported that EMC revealed an irregular-shaped soft tissue mass with clear boundary and uniform density.…”

“…Studies in the past were aimed at improving disease control in the surgical resection, radiotherapy, with some reported benefit from tyrosine kinase inhibitor sunitinib malate in the metastatic setting [ 7 – 10 ]. The literature contains few cases in which the imaging characteristics of EMC are discussed, and therefore a complete imaging and pathology interpretation of this disease is lacking.…”

Breast Metastasis of Extraskeletal Myxoid Chondrosarcoma: A Case Report

“…EMC typically presents as a slow-growing mass in the deep soft tissues of the lower extremities. It usually involves proximal extremities and limb girdle (60%), distal extremities (20%), and trunk (20%), but can have several unusual sites of origin like the scrotum, finger [ 6 ], nasal cavity, central nervous system, and vulva [ 7 ]. Recent studies have found that EMC has high propensity for metastases along with a high rate of local recurrences.…”

“…Studies have also shown that tumor size (≥10 cm) is also an important prognostic factor in terms of survival rate as well as metastases [ 2 ]. However, a study by Kapoor et al showed no correlation between the tumor size and metastases-free interval [ 6 ]. Rhabdoid phenotype and anaplasia may be associated with poor prognosis [ 2 ].…”