Division of Medical Oncology, Washington University in St. Louis, St. Louis, MO 63110, USA.
Siteman Cancer Center, St. Louis, MO 63110, USA.
Med Sci (Basel). 2021 Oct 15;9(4):62. doi: 10.3390/medsci9040062.
Ewing sarcoma (ES), "Ewing-like sarcoma" (ELS) and desmoplastic small round cell tumors (DSRCT) can masquerade as other tumor types, particularly neuroendocrine neoplasms and receive inappropriate treatment. We retrieved 115 cases of ES, ELS and DSRCT seen over 17 years in a tertiary center. An initial misdiagnosis or incomplete diagnosis occurred in 6/93 (6.4%) of ES/ELS and 5/22 (22.7%) of DSRCT cases. The most frequent misdiagnosis was small cell neuroendocrine carcinoma. While any misdiagnosis or incomplete classification is almost certainly multifactorial, the most common identified reason for erroneous/incomplete initial reporting was expression of neuroendocrine markers. Other contributing factors included keratin expression, older patient age and apparently unusual tumor location. Most patients treated with a non-sarcoma chemotherapy regimen expired, while those who received a sarcoma-related regimen were alive as of last evaluation. Increased awareness of this diagnostic pitfall is needed in evaluating cases of round cell malignancies.
尤因肉瘤（ES）、“类尤因肉瘤”（ELS）和促结缔组织增生性小圆细胞肿瘤（DSRCT）可能伪装成其他肿瘤类型，尤其是神经内分泌肿瘤，并接受不适当的治疗。我们在一个三级中心检索了 17 年来的 115 例 ES、ELS 和 DSRCT 病例。在 93 例 ES/ELS 中有 6 例（6.4%）和 22 例 DSRCT 中有 5 例（22.7%）最初误诊或不完全诊断。最常见的误诊是小细胞神经内分泌癌。虽然任何误诊或不完全分类几乎肯定是多因素的，但最初报告错误/不完整的最常见原因是神经内分泌标志物的表达。其他促成因素包括角蛋白表达、患者年龄较大和肿瘤位置明显异常。大多数接受非肉瘤化疗方案治疗的患者死亡，而接受肉瘤相关方案治疗的患者在最后一次评估时仍存活。在评估小圆细胞恶性肿瘤病例时，需要提高对这一诊断陷阱的认识。
