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
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.
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Intracranial Metastasis of Extracranial Chondrosarcoma: Systematic Review With Illustrative Case Charles E. Mackel, Harry Rosenberg, Hemant Varma, Erik J. Uhlmann, Rafael A. Vega, Ron L. Alterman Brain Tumor Research and Treatment.2023; 11(2): 103. CrossRef