原发于腹壁的骨外黏液样软骨肉瘤1例
| 作者: |
1李玲玉,
1杨海生,
1陈玲玲,
1方迪,
1官兵
1 上海市第六人民医院金山分院病理科，上海 201599 |
| 通讯: |
官兵
Email: g_bguan@163.com |
| DOI: | 10.3978/j.issn.2095-6959.2022.05.038 |
| 基金: | 上海市金山区卫生健康专项科研课题(JSKJ-KTQN-2020-07)。 |
摘要
本例骨外黏液样软骨肉瘤(extraskeletal myxoid chondrosarcoma，EMC)发生于右侧腹壁，影像学显示腹股沟内结节影；光镜下肿瘤呈多结节状，由宽窄不一的纤维结缔组织间隔，肿瘤细胞呈卵圆形或短梭形，条索状、梁状或网格状排列，细胞之间富于黏液样或黏液软骨样基质，侵犯周围脂肪及骨骼肌；免疫组织化学染色结果符合EMC的典型标记特点。EMC为起源未定的软组织恶性肿瘤，具有独特的组织学形态、免疫组织化学标记和分子遗传学特点。目前病例数量较少，并且大部分病例失随访，缺乏相关治疗和预后资料，仍需进一步的病例积累和观察。
关键词：
骨外黏液样软骨肉瘤；临床病理；鉴别诊断
Primary extraskeletal myxoid chondrosarcoma of the abdominal wall: A case report
CorrespondingAuthor: GUAN Bing Email: g_bguan@163.com
DOI: 10.3978/j.issn.2095-6959.2022.05.038
Foundation: This work was supported by the Health Special Scientific Research Project of Jinshan District of Shanghai (JSKJ-KTQN-2020-07), China.
Abstract
This case of extraskeletal myxoid chondrosarcoma (EMC) occurred in the right abdominal wall. The imaging showed nodule shadow in inguinal region. Microscopically, the tumor was multinodular, separated by fibrous connective tissue of different width. The tumor cells were oval or short fusiform, arranged in cords, beams or grids. The cells were rich in mucinous or mucilaginous cartilage like matrix, and invaded surrounding fat and skeletal muscle. The results of immunohistochemical staining were consistent with the typical characteristics of EMC. EMC is a soft tissue malignant tumor of undetermined origin, which has unique histological morphology, immunohistochemical markers and molecular genetic characteristics. At present, the number of cases is small, and most of the cases are not followed up. There is a lack of relevant treatment and prognosis data. Further case accumulation and observation are still needed.
Keywords：
extraskeletal myxoid chondrosarcoma; clinicopathology; differential diagnosis