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.
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Extraskeletal myxoid chondrosarcoma of the parotid gland NyimiBushabu Fidele, Wu Tianfu, Bing Liu, Yanfang Sun, Zhao Yifang Annals of Maxillofacial Surgery.2019; 9(2): 439. CrossRef
Myxoid chondrosarcoma of maxilla: A rare case report Hiralal Ash, Ajoy Kumar Shahi, Kabita Chatterjee, Dipankar Samaddar Journal of Oral and Maxillofacial Surgery, Medicine, and Pathology.2016; 28(3): 273. CrossRef
Maxillo-facial Extraskeletal Myxoid Chondrosarcoma: A Case Report and Discussion Ratnadeep Ganguly, Abhishek Mukherjee The Korean Journal of Pathology.2011; 45(6): 639. CrossRef
Dedifferentiated chondrosarcoma is an uncommon bone tumor, defined as a tumor in which two components -a low-grade chondrosarcoma and a high-grade non-cartilaginous sarcoma-coexist with abrupt interface. We report a rare case of giant-cell rich dedifferentiated chondrosarcoma occurred in the right distal femur shaft of a 60 year-old female. The plain X-ray film showed an irregular radiolucent mass. The T2-weighted MRI revealed a heterogeneous high signal intensity. It was an irregular mass composed of bluish-white, translucent chondroid elements and yellowish solid components with extraosseous invasion.
Microscopically, a low-grade chondrosarcoma and a giant-cell rich spindle cell sarcoma with areas resembling giant cell tumor were recognized with abrupt transition.
Immunohistochemical staining revealed a S100 protein positivity in chondroid cells and a few spindle cells. CD68 was strongly positive in giant cells. Vimentin was positive in both components and smooth muscle actin was positive in some spindle cells. There was no cytokeratin, desmin and myogenin immunopositivity. It is important to be aware of this rare variant of dedifferentiated chondrosarcoma to avoid the misdiagnosis of more common bone tumors including giant cell tumors.
Dedifferentiated chordoma is a rare pathologic entity presenting an additional sarcomatous component in otherwise classical chordoma. It has been also emphasized that this neoplasm is classified as a distinct entity because of its different clinical settings and aggressive behavior.
Dedifferentiation is a peculiar phenomenon but its histogenesis has remained controversial. A 50-yera-old man developed a huge tumor mass in the retrorectal, presacral area, featured with two histological components. The one was a typical chordoma accounted for approximately 60% of the mass and the other was made up of highly cellular, plemorphic, undifferentiated tumor cells, reminiscent partly to the cells of plemorphic malignant fibrous histiocytoma.
Ultrastructural features and immunoreactivity against cytokeratin, S-100 protein and alpha-1-antichymotrypsin in both portions support that histologically different components of this neoplasm derive from the same origin. To our knowledge, this is the first case of dedifferentiated chordoma in Korea.