Jérôme-Marson Valérie, Uro-Coste Emmanuelle, Lacoste-Collin Laetitia, Gomez-Brouchet Anne, Serrano Elie, Delisle Marie-Bernadette
Service d'Anatomie et Cytologie Pathologiques, Hôpital Rangueil, Avenue Jean Poulhès, 31000 Toulouse cedex.
Ann Pathol. 2003 Jun;23(3):253-7.
Extraskeletal myxoid chondrosarcoma is a rare but distinct entity with special clinicopathological, immunohistochemical, cytogenetical and outcome features. This tumor developed from soft tissues. A few cases have been reported in the head and neck in the literature. We report two new cases of extraskeletal myxoid chrondrosarcoma presenting in such an unusual site: one involved the left nasal cavity of a 67-year-old man and the second the sphenoidal sinus of a 71-year-old woman. The microscopic examination revealed nests of round small cells dispersed in a myxoid stroma. The myxoid material was stained with Alcian Blue with and without hyaluronidase application whereas no PAS staining was observed. The immunohistochemical staining showed reactivity with S-100 protein and vimentin in two cases and with EMA in one case. These results allowed us to exclude other differential diagnoses: soft tissue tumors with a myxoid stroma (myxoma, myxoid liposarcoma and myxofibrosarcoma). No staining with anti-KL1 allowed us to exclude chordoma. Curative surgery was not possible. Both patients were given radiotherapy and the tumor regressed in one.
骨外黏液样软骨肉瘤是一种罕见但独特的实体瘤，具有特殊的临床病理、免疫组化、细胞遗传学及预后特征。该肿瘤起源于软组织。文献中曾有少数头颈部病例报道。我们报告两例发生于不寻常部位的骨外黏液样软骨肉瘤新病例：一例发生于一名67岁男性的左侧鼻腔，另一例发生于一名71岁女性的蝶窦。显微镜检查显示圆形小细胞巢散在于黏液样基质中。黏液样物质经阿尔辛蓝染色，无论是否应用透明质酸酶，均未见PAS染色。免疫组化染色显示，两例对S-100蛋白和波形蛋白呈阳性反应，一例对EMA呈阳性反应。这些结果使我们能够排除其他鉴别诊断：具有黏液样基质的软组织肿瘤（黏液瘤、黏液样脂肪肉瘤和黏液纤维肉瘤）。抗KL1染色阴性使我们能够排除脊索瘤。无法进行根治性手术。两名患者均接受了放疗，其中一名患者的肿瘤消退。
