Extra Skeletal Myxoid Chondrosarcoma of Hand – A Case Report with Review of Literature
DOI:
https://doi.org/10.56501/intjhistopatholinterpret.v11i2.521Keywords:
Chondrosarcoma; NR4A3 gene; Myxoid; pazopanibAbstract
Extraskeletal myxoid chondrosarcoma (EMC) arising from soft tissues is a rare neoplasm morphologically characterized by ill-defined nodular masses composed of cords and strands of small eosinophilic cells separated by fibrous septae along with abundant myxoid stroma. We report a middle-aged man presenting with swelling on his hand for the last 11 years. He reports occasional pain in the swelling without any increase in its size or change of color. Radiologically, it mimicked a giant cell tumor of the tendon sheath. We received a white gelatinous tumor mass showing myxoid and hemorrhagic areas on cut section. Histo-morphological examinations suggested a diagnosis of EMC. Immunohistochemistry showed a strong expression of S100 and NSE. This slow-growing mass mimicking a benign giant cell tumor of tendon sheath warranted an urgent examination for metastasis and further treatment.
References
D.R. Lucas, G. Stenman, Extraskeletal myxoid chondrosarcoma, in: Fletcher CD, Bridge JA, Hogendoorn P, Mertens F. World Health Organization Classification of Tumours. Pathology and Genetics of Tumours of Soft Tissue and Bone, IARC Press, Lyon, 2013, pp. 223–224.
Enzinger FM, Shiraki M. Extraskeletal myxoid chondrosarcoma: an analysis of 34 cases. Human pathology. 1972 Sep 1;3(3):421-35.
Brenca M, Stacchiotti S, Fassetta K, Sbaraglia M, Janjusevic M, Racanelli D, et al. NR4A3 fusion proteins trigger an axon guidance switch that marks the difference between EWSR1 and TAF15 translocated extraskeletal myxoid chondrosarcomas. The Journal of Pathology. 2019 Sep;249(1):90-101.
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Copyright (c) 2022 Shamaila Sameen, Siddiqui Bushra, Alam Feroz, Nahid Zeeshan
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