Anthracyclinebased chemotherapy in extraskeletal myxoid. Extraskeletal myxoid chondrosarcoma drilon 2008 cancer. Images were acquired with a ccd camera and processed with analysis imaging software soft imagining system, munster, germany. Head and neck is an extremely rare site for this kind of tumor with very few cases reported till. Rare, first described in 1972 50 65% males, median age 50 52 years, range 6 89 years 80% in proximal extremities or limb girdles, 20% in trunk. A closer look at extraskeletal myxoid chondrosarcoma within the. Extraskeletal myxoid chondrosarcoma emc is a rare softtissue sarcoma, which primarily occurs deep in the extremities, especially in skeletal muscle, or tendon. A phenotypically and genotypically distinct mesenchymal malignancy with tumourspecific fusion genes, which affects older adults and represents 2. In the present paper, we report one such case of intermediate grade myxoid chondrosarcoma of left side of maxilla in a 12yearold girl. Cases of intracranial extraskeletal myxoid chondrosarcoma. We describe herein, a 51yearold asian female initially manifested with signs of severe anemia who presented with. Doctors use the results of the biopsy and imaging studies to develop a. Treatment was provided within a compassionate use program, with the approval of the institutional ethics committee.
Emcs is marked by 40% incidence of metastases in spite of its indolent behaviour. The most common site of distant metastases is the lung, but soft tissues and lymph nodes can also be involved. Chordoma and chondrosarcoma of the skull base are rare tumors with overlapping presentations and anatomic imaging features but different prognoses. The standard treatment for primary, localised extraskeletal myxoid chondrosarcoma is surgery, but patients with advanced disease need medical treatment with drugs. Learn indepth information on molecular testing for extraskeletal myxoid chondrosarcoma, on why the laboratory test is performed, specimen collected, the significance of the results, and its use in diagnosing medical conditions. Myxoid chondrosarcoma definition of myxoid chondrosarcoma. Extraskeletal myxoid chondrosarcoma chordoid sarcoma. Extraskeletal myxoid chondrosarcoma is characterized by the reciprocal chromosomal translocation t9. There are no new materials included in any database or software from this article. Oct 12, 2005 to describe the mri features of extraskeletal myxoid chondrosarcoma in comparison with clinicopathologic findings. Extraskeletal myxoid chondrosarcoma emc is a rare lowgrade malignant mesenchymal neoplasm of the soft tissues, that differs from other sarcomas by unique histology and characteristic chromosomal translocation. Extraskeletal myxoid chondrosarcoma emc is a rare soft tissue tumor. Myxoid liposarcoma myxoid liposarcoma mlps is the second most common group of adipocyticlipogenic sarcomas accounting for about 30% to 35% of all liposarcomas. Mr imaging of clear cell chondrosarcoma typically shows homogeneous intermediate signal intensity with t1weighted sequences and heterogeneous high signal intensity with.
These lesions have marked high water content, related to the extensive myxoid stroma and betterdifferentiated areas of hyaline cartilage,, fig 6. Chondrosarcoma is a member of a category of tumors of bone and soft tissue known as sarcomas. It is generally resistant to conventional chemotherapy, and, to the best of our. A magnetic resonance image may be the better imaging option for detection of. Extraskeletal myxoid chondrosarcoma emcs is a rare soft tissue sarcoma of. It is the second most common primary malignancy of bone, and one of the most.
The study comprised 12 male subjects and seven female subjects with a mean age of 53 years range 1676 years. Extraskeletal myxoid chondrosarcoma is an uncommon softtissue malignancy that. Images were acquired with a ccd camera and processed with analysis imaging software. My partner was diagnosed with a high grade sarcoma in his right thigh which was removed en mass. Recently, a randomized phase 2 study in patients with translocationrelated sarcomas unresponsive or intolerable to standard chemotherapy was conducted, which showed clinical benefit of trabectedin compared with best supportive care bsc. Myxoid chondrosarcoma of bone in a 50yearold woman with a gluteal. Extraskeletal myxoid chondrosarcoma emc accounts for 2.
Among six patients who underwent preoperative ct examinations, six cases of lesions exhibited hypodensity on unenhanced image, three cases of tumor. Mesenchymal chondrosarcoma nord national organization for. Myxoid chondrosarcoma has a median age at diagnosis of 50 years and is more commonly diagnosed in males than females at a ratio of approximately 2. Emc of the pleura has been described, however, no case of primary emc arising from lung has been previously reported. Dedifferentiated and mesenchymal chondrosarcoma are high grade tumors that. Dec 04, 2018 chondrosarcoma is a collective term for a group of tumors that consist predominantly of cartilage and that range from lowgrade tumors with low metastatic potential to highgrade, aggressive tumors characterized by early metastasis. Elsevier mr imaging of dedifferentiated chondrosarcoma stephen eustace, md, nancy baker, md, howard lan, md, anil wadhwani, md, and david dorfman, md mr imaging of a dedifferentiated chondrosarcoma of the humerus is presented and correlated with gross and microscopic histology from the surgical speci men. There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous differentiation and neuroendocrine differentiation is even. Although myxoid liposarcoma is a subtype of liposarcoma, it may be difficult to establish the correct diagnosis with magnetic resonance mr imaging due to the lack of fat signal intensity. A comparative study of imaging and pathology lingzhang,1 ruoningwang,2 rongxu,1 genggengqin,1 andleiyang 3 departmentofriology,nanfanghospital,southernmedicaluniversity,guangzhou, china. Chondrosarcoma symptoms, prognosis, treatment, survival rate. Molecular testing for extraskeletal myxoid chondrosarcoma.
It is generally resistant to conventional chemotherapy, and, to the best of. Clinical and radiologic features of extraskeletal myxoid. Extraskeletal myxoid chondrosarcoma emc is a slowly growing, painless tumor of. It is found in both bone and soft tissues see extraskeletal chondrosarcoma. Molecular and clinicopathologic heterogeneity of intracranial.
Traditionally regarded a lowgrade indolent malignancy with only late local recurrences, 40% to 50% of cases are now acknowledged to metastasize after 10 or more years. Sep 11, 2018 extraskeletal myxoid chondrosarcoma emc is an infrequent, lowgrade tumor of the soft tissues, forming around bones. Extraskeletal myxoid chondrosarcoma of the midfoot presenting as charcot arthropathy. Extraskeletal myxoid chondrosarcoma is a sarcoma arising in soft tissue with relatively slow growth and frequent local recurrence as well as innumerable metastatic sites in the lungs, a diagnosis that is usually less chemotherapy sensitive than mesenchymal chondrosarcoma. Mixoid chondrosarcoma or neuronal cancer survivors network. Wayne slone, in handbook of neurooncology neuroimaging second. Subtypes vary substantially chondrosarcoma nos and myxoid chondrosarcoma have a five year survival of 70%, but mesenchymal chondrosarcoma only 50%, and dedifferentiated.
Chondrosarcomas are malignant cartilaginous tumors that account for 25% of all primary malignant bone tumors. Differential diagnosis myxoid liposarcoma surgical. Ct is best for examining tumor matrix mineralization, while mri is best for assessing marrow and soft tissue involvement. A combination of imaging features including certain characteristic signs, clini cal features, and patient demographics can help the radiologist in coming to a specific diag. Oct 11, 2012 extraskeletal myxoid chondrosarcoma emcs is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein ewsr1chn the latter even called nr4a3 or tec. The purpose of this study was to achieve better understanding of extraskeletal myxoid chondrosarcoma emc. Its sensitivity to chemotherapy is reported to be low. Extraskeletal myxoid chondrosarcoma is a rare subtype of sarcoma that typically affects the deep soft tissue of adults mainly in the 4th to 7th decade of life, with an approximately 2.
Chondrosarcoma is a type of sarcoma that affects the bones and joints. Mri features of extraskeletal myxoid chondrosarcoma. Mesenchymal chondrosarcoma nord national organization. All images were analyzed by two boardcertified radiologists, who specialize in musculoskeletal imaging. Mar 07, 2011 creative thinking how to get out of the box and generate ideas. Extraskeletal myxoid chondrosarcoma emcs is a rare soft tissue sarcoma of uncertain differentiation, characterized in most cases by a translocation that results in the fusion protein ewsr1chn the latter even called nr4a3 or tec. About 30% of skeletal system cancers are chondrosarcomas.
Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma that has unusual ultrastructural and molecular features. There is an uncertain differentiation there is no evidence yet showing that emc exhibits the feature of cartilaginous. Myxoid chondrosarcomas are rare, intermediate grade subtype of. Going along with its more aggressive clinical course, the lesion is predominantly lytic, with the high. On imaging, extraskeletal myxoid chondrosarcoma presents as a large well defined lobulated heterogeneous lesion, often with the presence of a pseudocapsule.
Nevertheless, extraskeletal myxoid chondrosarcoma has a rare gene fusion ewsr1nr4a3 that is useful for. Maturation if present, occurs towards center of lobules. Lee, md although myxoid liposarcoma is a subtype of liposarcoma, it may be. Unlike other primary bone cancers that mainly affect children and adolescents, chondrosarcoma can present at any age. What are the 3 imaging tests commonly ordered for the workup of a possible chondrosarcoma. In the present paper, we report one such case of intermediate grade myxoid chondrosarcoma of left side of maxilla in a 12yearold. It has become the primary imaging modality for evaluation of softtissue masses accessible to us.
Such tumors include myxoid liposarcoma, extraskeletal myxoid chondrosarcoma, intramuscular myxoma, ganglion. Extraskeletal myxoid chondrosarcoma emc is an infrequent, lowgrade tumor of the soft tissues, forming around bones. Specialized imaging techniques may be used to help evaluate the size. Diagnosis mainly relies on imaging, morphology, and. Mr imaging of dedifferentiated chondrosarcoma sciencedirect. Myxoid chondrosarcoma of maxilla in a pediatric patient. A combination of imaging features including certain characteristic signs, clinical features, and patient demographics can help the radiologist in coming to a specific diagnosis or in narrowing down the differential diagnoses. Extraskeletal myxoid chondrosarcoma emc is a rare lowgrade malignant mesenchymal neoplasm of the soft tissues, that. Myxoid chondrosarcoma an overview sciencedirect topics. Emc has a male preference, and this occurs in soft tissue area in patients who. Myxoid chondrosarcoma is a type of chondrosarcoma it has been associated with a t9. Extraskeletal myxoid chondrosarcoma emc is an uncommon lowgrade sarcoma of the soft tissue, usually presenting as a slowgrowing mass in men in their fifth to sixth decades. The aim of the study was to evaluate the clinical and imaging features of extraskeletal myxoid chondrosarcoma emc including initial presentation, recurrence, and metastases.
Extraskeletal myxoid chondrosarcoma is a rare tumor, encompassing 2. Intraosseous myxoid chondrosarcoma is a rare soft tissue tumor. Mean ages reported in various series range from 46 to 57 years. Primary chondrosarcoma is the third most common primary malignant tumor of bone, constituting 20%27% of all primary malignant osseous neoplasms. Diagnostic utility of molecular investigation in extraskeletal myxoid chondrosarcoma stefania benini, stefania cocchi, gabriella gamberi, giovanna magagnoli,y daniela vogel, cristina ghinelli,y alberto righi, piero picci,y marco alberghini, and marco gambarotti from the departments of pathology and experimental oncology,y rizzoli institute, bologna, italy. Diagnosis of extraskeletal myxoid chondrosarcoma in the thigh. Fluorescence staining was visualized with an olympus bx61 microscope olympus, volketswil, switzerland equipped with dapi, spectrumgreen and spectrumorange filters. It is a malignant tumor histologically characterized by round to oval mesenchymal cells, small signet ring lipoblasts, and rich network of capillaries in a myxoid stroma. Diagnostic utility of molecular investigation in extraskeletal myxoid chondrosarcoma stefania benini, stefania cocchi, gabriella gamberi, giovanna magagnoli,y daniela vogel, cristina ghinelli,y alberto righi, piero picci,y marco alberghini, and marco gambarotti. It involves the deep soft tissues of the proximal limbs, especially the thigh and popliteal fossa. Chondrosarcoma is a malignant tumor composed of cartilageproducing cells. Us is useful in differentiating solid masses from cystic lesions. Chondrosarcoma mesenchymal and extraskeletal myxoid symptoms, treatment, prognosis, survival rate.
There are numerous types of primary chondrosarcomas, including conventional intramedullary, clear cell, juxtacortical, myxoid, mesenchymal, extraskeletal, and dedifferentiated. Chondrosarcoma is a cancer composed of cells derived from transformed cells that produce cartilage. We describe herein, a 51yearold asian female initially manifested with signs of severe anemia who. However, unlike other soft tissue sarcomas, it does not have specific clinical symptoms or radiological features, which can make its diagnosis difficult.
To describe the mri features of extraskeletal myxoid chondrosarcoma in comparison with clinicopathologic findings. Myxoid chondrosarcoma is a rare histologic variant of chondrosarcoma that occurs in both bone and soft tissue and is considered an intermediategrade tumor. Middleaged to elderly individuals are most prone to this tumor formation. Extraskeletal myxoid chondrosarcoma is an extremely rare malignancy. Esmcs differs from the primary skeletal chondrosarcoma given the myxoid changes, and is classified as a tumour of uncertain. Males are affected about twice as often as females. Extraskeletal myxoid chondrosarcoma emcs and mesenchymal. Insm1 expression and its diagnostic significance in. With rare exception, most of the radiology literature has consisted of case reports or has been. Myxoid chondrosarcoma is rare histological variant of chondrosarcoma that usually occurs in limbs. Jul 14, 2016 trabectedin is reported to be particularly effective against translocationrelated sarcoma.
Chondrosarcoma is a collective term for a group of tumors that consist predominantly of cartilage and that range from lowgrade tumors with low metastatic potential to highgrade, aggressive tumors characterized by early metastasis. Plain radiographs, mri, and ct are commonly ordered for the workup of a possible chondrosarcoma. The authors reported a retrospective study on myxoid liposarcomas mls, evaluating factors that may influence overall survival os, local recurrencefree survival lrfs, metastasisfree survival mfs, and analyzing the metastatic pattern. Aug 28, 2012 extraskeletal myxoid chondrosarcoma emc is a rare softtissue sarcoma, which primarily occurs deep in the extremities, especially in skeletal muscle, or tendon. The literature documenting the imaging features of extraskeletal myxoid chondrosarcoma is limited. Extraskeletal chondrosarcoma radiology reference article. Pathology they tend to be of higher grade than runofthemill conventional intramedullary chondrosarcomas, with the majority being of t. Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma. Extraskeletal myxoid chondrosarcoma is rather different from mesenchymal chondrosarcoma.
We hypothesized that these tumors might be distinguished by using diffusionweighted mr imaging. Trabectedin is reported to be particularly effective against translocationrelated sarcoma. Extraskeletal myxoid chondrosarcoma emc is a genetically distinct sarcoma with a propensity for local recurrence and metastasis despite an indolent course. Histologically as well as on imaging, these tumors are difficult to distinguish from chordomas.
Magnetic resonance imaging of benign soft tissue neoplasms in adults. Extraskeletal myxoid chondrosarcoma chordoid sarcoma definition and pathogenesis a rarely occurring soft tissue cartilaginous malignancy with the tendency for lr. When myxoid liposarcomas have a purely cystic appearance at mr imaging, both. Extraskeletal myxoid chondrosarcoma, a rare soft tissue sarcoma primarily. Subsequently, magnetic resonance imaging mri revealed a. Extraskeletal myxoid chondrosarcoma emc is a slowly growing, painless tumor of adults over 35 years of age. Results of subanalysis of a phase 2 study on trabectedin. Pazopanib for treatment of advanced extraskeletal myxoid.
It is a rare cancer that accounts for about 20% of bone tumors and is diagnosed in approximately 600 patients each year in the united states. Mri features of extraskeletal myxoid chondrosarcoma request pdf. Usually a good prognosis 75% five year survival in one large data set. Mri is the modality of choice for characterization of myxoid softtissue tumors. The standard treatment for primary, localised extraskeletal myxoid chondrosarcoma is surgery, but patients with advanced disease need medical. All patients underwent preoperative ct orand mri examinations. Case report primary intraosseous myxoid chondrosarcoma arising. Mri findings, evaluated by two radiologists with agreement by consensus, were compared for histopathologic features. Extraskeletal myxoid chondrosarcoma emc is a rare subgroup within soft tissue sarcomas. Histologically as well as on imaging, these tumors are difficult to distinguish from chordomas and accounting for its alternative name of chordoid chondrosarcoma.
Appearance at mr imaging with histologic correlation1 misook sung, md heong s. Researcharticle extraskeletal myxoid chondrosarcoma. Mesenchymal chondrosarcoma is an extremely rare, often aggressive form of cancer. Extraskeletal chondrosarcomas make up only 2% of softtissue sarcomas and only 1% of all chondrosarcomas.