ABSTRACT
Purpose To explore the clinicopathologic characteristics, pathologic diagnosis and differential diagnosis of extraskeletal myxiod chondrosarcoma. Methods The clinical and pathological features were studied with HE and immunohistochemical staining in 5 cases of extraskeletal myxoid chondrosarcoma, and the related literatures were also reviewed. Results Extraskeletal myxoid chondro-sarcoma was male predominance (4 males: 1 female), and the age ranged from 32 to 62 years with an average of 52 years. Four tumors occurred in thigh and another in chest. Histologically, the neophlasm were composed of round and oval cells. Immunohisto-chemically, the tumor cells were positive for vimentin in five cases, synaptophysin expressed in one case, S-100 showed focal positivity in two cases, but negative for CK, p63, EMA, CD34, SMA in all cases. Molecular genetics detection shows chromosome exchange be-tween number 9 and 22. Conclusion Extraskeletal myxoid chondrosarcoma is a rare tumor which has no special characteristics in clinical and iconography, and its diagnosis mainly depends on the histopathologic features, immunohistochemistry and molecular genet-ics. Extraskeletal myxoid chondrosarcoma grows slowly, but have a high tendency for local recurrence and metastases.
ABSTRACT
Background and purpose: Extraskeletal myxoid chondrosarcoma (EMC) is a malignancy of uncertain differentiation tumor characterized by a multinodular structure and abundant myxoid matrix. Its preferred sites were the deep soft tissues of the extremities. The aim of this study was to investigate the clinicopathologic characteristics, diagnosis and differential diagnosis of EMC. Methods: Seven cases of EMC were analyzed for clinicopathological and immunohistochemical features with review of the related literature. Results: It occurred predominantly in females (male/female=2︰5). Five cases were located in low extremities and two in upper limb girdles, more commonly near the joint. Grossly, the masses showed as grey, lobular and somewhat transparent with a relatively well-deifned margin. Microscopically, the small ovary or plump spindle-shaped cells arranged in strand and cord patterns and lobular architecture which separated by delicate fibrous networks with an abundant myxoid but hypovascular background. And the tumors were immunoreactive for vimentin, and partly for S-100 and EMA, meanwhile, negative for CK. Conclusion:EMC is a rare soft tissue sarcoma with distinctive histopathological features. It should be distinguished from some mimics especially those tumors with a myxoid stroma or chondroid differentiation, such as chordoma and chondrosarcoma.
ABSTRACT
Extraskeletal myxoid chondrosarcoma is a rare soft-tissue sarcoma, mostly occurring in the proximal extremities and limb girdle. Majority of the patients are in fifth and sixth decades of life with male preponderance. We report here a case of primary extraskeletal myxoid chondrosarcoma of the uterine adnexa involving the broad ligament and fallopian tube synchronously without any evidence of uterine/ovarian involvement in a young multiparous female of 27 years. After the histopathological diagnosis, re-excision of the tumor bed with wide local margins was recommended. Since the tumor has an aggressive course, with propensity for late recurrence and metastases to lungs, the patient must be considered for long-term follow-up.
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In this report, we share our experience of a case of maxillo-facial extraskeletal myxoid chondrosarcoma, a very rare location for this neoplasm. In addition, a literature review is provided. The patient, a 61-year-old male, had a maxillary mass encroaching on the nasal cavity. After debulking, the tumor recurred, attaining its pre-surgical proportion in two months. The patient improved clinically with radiation and remained stable for about one year. However, he ultimately developed metastases in his lung which were treated with palliative chemotherapy with a good outcome lasting three months. We could find only eight reported cases of this tumor in the head region of which two are in the maxilla; hence, ruling out other primary sites is mandatory for a patient presenting with a primary head and neck mass. Surgical removal may be complicated because of the location. A combination of surgery and radiation is the management of choice, with palliative chemotherapy in metastasis.
Subject(s)
Humans , Male , Middle Aged , Chondrosarcoma , Head , Lung , Maxillary Sinus , Nasal Cavity , Neck , Neoplasm MetastasisABSTRACT
We describe a 69-year-old woman who presented with a dedifferentiated extraskeletal myxoid chondrosarcoma arising in the left masticator space. Computed tomography and magnetic resonance imaging revealed a 5 cm sized mass in the left masticator space. Histologically, the tumor consisted of two distinct areas. The less cellular area was a low-grade extraskeletal myxoid chondrosarcoma, composed of strands or cords of uniform spindle cells and abundant myxoid stroma. The more cellular, dedifferentiated area corresponded to a high grade myxofibrosarcoma, consisting of anaplastic tumor cells in myxoid stroma and geographic necrosis. The tumor cells of the former area were positive for S-100 protein, microtubule-associated protein-2 (MAP-2) and class III beta-tubulin, but negative for cytokeratin, smooth muscle actin, and desmin. The tumor cells in the latter, pleomorphic area showed MAP-2 and beta-tubulin immunoreactivity with a high Ki-67 labeling index. Based on its histologic and immunohistochemical features, the tumor was considered a dedifferentiated extraskeletal myxoid chondrosarcoma.
Subject(s)
Aged , Female , Humans , Actins , Cell Dedifferentiation , Chondrosarcoma , Desmin , Keratins , Magnetic Resonance Imaging , Muscle, Smooth , Necrosis , S100 Proteins , TubulinABSTRACT
We describe a 69-year-old woman who presented with a dedifferentiated extraskeletal myxoid chondrosarcoma arising in the left masticator space. Computed tomography and magnetic resonance imaging revealed a 5 cm sized mass in the left masticator space. Histologically, the tumor consisted of two distinct areas. The less cellular area was a low-grade extraskeletal myxoid chondrosarcoma, composed of strands or cords of uniform spindle cells and abundant myxoid stroma. The more cellular, dedifferentiated area corresponded to a high grade myxofibrosarcoma, consisting of anaplastic tumor cells in myxoid stroma and geographic necrosis. The tumor cells of the former area were positive for S-100 protein, microtubule-associated protein-2 (MAP-2) and class III beta-tubulin, but negative for cytokeratin, smooth muscle actin, and desmin. The tumor cells in the latter, pleomorphic area showed MAP-2 and beta-tubulin immunoreactivity with a high Ki-67 labeling index. Based on its histologic and immunohistochemical features, the tumor was considered a dedifferentiated extraskeletal myxoid chondrosarcoma.
Subject(s)
Aged , Female , Humans , Actins , Cell Dedifferentiation , Chondrosarcoma , Desmin , Keratins , Magnetic Resonance Imaging , Muscle, Smooth , Necrosis , S100 Proteins , TubulinABSTRACT
Objective:To investigate the clinicopathological characteristics of extraskeletal myxoid chondrosarcoma(EMC).Methods:Nine cases of extraskeletal myxoid chondrosarcoma were studied.Immunohistochemical analysis was performed in all cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.Follow-up information was available for seven patients.Results:There were 7 males and 2 females whose ages ranged from 31 to 69 years(median 52.78 years).Local pain or tenderness and the presence of a palpable mass were the main complaints of the patients.The tumors were located mainly in the lower extremities(66.7%).Most tumors were deep-seated.They usually had a distinct multinodular configuration delineated by fibrous connective tissue.The tumor cells were arranged in delicate intersecting strands,rings,and garlands for the most part.The myxoid matrix was abundant in most cases.Immunohistochemical analysis was performed in all cases and ultrastructural studies were done in 2 extraskeletal myxoid chondrosarcomas.EMC expressed vimentin(100%,9/9),neuron-specific enolase(77.8%,7/9),S-100 protein(66.7%,6/9),synaptop hysin and chromogranin A(22.2%,2/9),None of the tumors expressed EMA and desmin.Ultrastructurally,EMC was characterized by distinct cords of cells immersed in a glycosaminoglycan rich matrix.The cells were rich in mitochondria,had well-developed Golgi apparatus and there were numerous smooth vesicles.In many cells,there were also prominent glycogen deposits and lipid droplets.Some tumor cells had intracisternal microtubules.In one of 2 extraskeletal myxoid chondrosarcomas there were 140～180 nm diameter membrane-bound dense-core secretory granules in cell bodies.Conclusion:Extraskeletal myxoid chondrosarcoma is a rare soft tissue sarcoma characterized by distinctive morphological and cytogenetical features.It was believed to represent a variant of soft-tissue chondrosarcoma owing to its histological resemblance to chondroblastic tissue in the early stages of cartilage development or chondroid tumors such as skeletal chondrosarcoma.However,the chondroid nature has been a subject of controversy,and its line of differentiation remains to be determined.A substantial proportion of EMC show immunophenotypic and/or ultrastructural evidence of neuroendocrine differentiation.EMC has high potential of local recurrence and metastasis,and a high disease-associated death rate.