Immunoprofile of mesenchymal chondrosarcoma: aberrant desmin and EMA expression retention of INI1, and negative estrogen receptor in 22 female-predominant central nervous system and musculoskeletal cases
Immunoprofile of mesenchymal chondrosarcoma: aberrant desmin and EMA expression retention of INI1, and negative estrogen receptor in 22 female-predominant central nervous system and musculoskeletal cases
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DOI：
10.1016/j.anndiagpath.2009.09.003
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发表时间：
2010-02-01
影响因子：
2
通讯作者：
Rushing, Elisabeth J.
中科院分区：
文献类型：
--
作者：
Fanburg-Smith, Julie C.;Auerbach, Aaron;Rushing, Elisabeth J.
间质软骨肉瘤是一种罕见的恶性肿瘤，在鉴别诊断其他小，圆形蓝细胞肿瘤，包括非典型畸胎瘤在中枢神经系统（CNS）和横纹肌肉瘤在肌肉骨骼（MSK）的位置。我们回顾了CNS和MSK病例的形态学，并应用了一组免疫染色。档案案件是从我们的档案里找出来的获得免疫组化和随访。22例病例包括5例CNS（均为女性;平均年龄30.2岁）和17例MSK（11例女性和6例男性;平均年龄31.1岁）。CNS和MSK的例子都有相似的圆形细胞，鹿角形血管模式，增加的有丝分裂活性，和位于中央的透明软骨岛。中枢神经系统的例子表现出更多的梭形和MSK病例更多的坏死。INI 1保留在所有研究的肿瘤中。上皮细胞膜抗原（EMA）和结蛋白分别在35%和50%的病例中表达。所有病例的圆形细胞MyoD 1、肌细胞生成素、平滑肌肌动蛋白（SMA）、胶质细胞酸性蛋白（GFAP）、角蛋白和雌激素受体以及一组其他抗体均为阴性。80%的随访患者在平均5年内发生肺转移和/或死亡。中枢神经系统和MSK间叶软骨肉瘤主要发生在成年女性，预后不良。CNS和MSK组之间仅存在细微的形态学差异。通过免疫组化，间叶性软骨肉瘤偶尔表达异常结蛋白和EMA，但SMA，肌细胞生成素MyoD 1，GEAP和角蛋白阴性，反驳了真正的平滑肌或骨骼肌，上皮或脑膜炎表型。保留的INI 1将这些肿瘤与非典型畸胎样肿瘤分开。尽管在我们的病例中女性明显占优势，但间叶性软骨肉瘤中雌激素受体呈阴性。爱思唯尔公司出版
Mesenchymal chondrosarcoma is a rare malignant tumor in the differential diagnosis of other small, round blue cell tumors, including atypical teratoid tumor in the central nervous system (CNS) and rhabdomyosarcoma in the musculoskeletal (MSK) locations. We reviewed the morphology of CNS and MSK cases and applied a panel of immunostains. Archival cases were pulled from our files. Imunohistochemistry and follow-up were obtained. Twenty-two cases included 5 CNS (all female; mean age, 30.2) and 17 MSK (11 female and 6 male; mean age, 31.1). Both CNS and MSK examples had similar round cells, staghorn vascular pattern, increased mitotic activity, and centrally located hyaline cartilage islands. The CNS examples demonstrated more spindling and the MSK cases more necrosis. INI1 was retained in all tumors studied. Epithelial membrane antigen (EMA) and desmin were expressed focally in 35% and 50% of cases, respectively. The round cells of all cases were negative for MyoD1, myogenin, smooth muscle actin (SMA), glial fibrillary acid protein (GFAP), keratins, and estrogen receptor, as well as a panel of other antiobodies. Eighty percent of patients with follow-up had pulmonary metastases and/or died within a mean of 5 years. The CNS and MSK mesenchymal chondrosarcoma predominantly affects adult females with poor prognosis. There are only subtle morphologic differences between the CNS and MSK groups. By immunohistochemistry, mesenchymal chondrosarcoma occasionally expresses aberrant desmin and EMA but is negative for SMA, myogenin MyoD1, GEAP, and keratins, refuting true smooth or skeletal muscle, epithelial, or meningothelial phenotype. Retained INI1 separates these tumors from atypical teratoid tumor. Despite marked female predominance in our series, estrogen receptor is negative in mesenchymal chondrosarcoma. Published by Elsevier Inc.