Weissferdt Annikka, Kalhor Neda, Moran Cesar A
From the Department of Pathology, The University of Texas MD Anderson Cancer Center, Houston.
Am J Clin Pathol. 2015 May;143(5):659-64. doi: 10.1309/AJCPFHFX07JFCRGU.
Three patients with Ewing sarcomas showing extensive neural differentiation are presented.
The patients were two women and one man between the ages of 15 and 35 years. Anatomically, one tumor was in the lung, one in the testis, and one in the cervix uteri. The symptoms were determined by the location of the neoplasm and included respiratory symptoms, testicular pain, and pelvic manifestations, respectively. Complete surgical resection of the tumors was performed.
Histologically, all neoplasms showed similar characteristics-namely, a neoplastic cellular proliferation arranged in sheets and composed of small blue cells with round to oval nuclei and inconspicuous nucleoli typical for Ewing sarcoma. In addition, in two cases, there were areas characterized by the presence of neuropil, ganglion cells, and small cells most compatible with ganglioneuroblastoma, while in one tumor, the neural component was characterized by the presence of small cells with prominent perivascular pseudorosettes more closely resembling ependymoma. Immunohistochemical studies in all cases and molecular analysis in two tumors were in keeping with a diagnosis of Ewing sarcoma.
The recognition of such histologic variants is important in the diagnostic assessment of these tumors to avoid misinterpretation, especially in small biopsy specimens.
介绍3例显示广泛神经分化的尤因肉瘤患者。
患者为2名女性和1名男性，年龄在15至35岁之间。从解剖学角度看，1例肿瘤位于肺部，1例位于睾丸，1例位于子宫颈。症状由肿瘤位置决定，分别包括呼吸道症状、睾丸疼痛和盆腔表现。对肿瘤进行了完整的手术切除。
组织学上，所有肿瘤均表现出相似特征，即肿瘤细胞呈片状增殖，由小蓝细胞组成，细胞核圆形至椭圆形，核仁不明显，这是尤因肉瘤的典型特征。此外，2例中存在以神经纤维、神经节细胞和与神经母细胞瘤最相符的小细胞为特征的区域，而在1例肿瘤中，神经成分以存在具有明显血管周围假菊形团的小细胞为特征，更类似于室管膜瘤。所有病例的免疫组织化学研究以及2例肿瘤的分子分析均符合尤因肉瘤的诊断。
认识到这种组织学变异在这些肿瘤的诊断评估中很重要，可避免误诊，尤其是在小活检标本中。
