PDF(Pubmed)
Abstract：
Primary desmoplastic small round cell tumor (DSRCT) in the head and neck region is extremely rare. There is limited information about its clinicopathological characteristics, prognosis, and treatment modalities. The purpose of this study is to provide a comprehensive review of DSRCT occurring primarily in the head and neck, to demonstrate its peculiar morphology and immunohistochemical expression, and to address the differential diagnoses. A total of 25 cases were collected after a thorough review of the relevant literature. DSRCT was most frequently reported in the major salivary glands, followed by the eyes. Furthermore, some cases were misinterpreted as poorly differentiated carcinoma, Ewing sarcoma, and olfactory neuroblastoma. Diagnosing DSRCTs in the head and neck can be very challenging due to their rarity in this location, overlapping morphology, and immunohistochemistry. In these cases, following a systemic approach helps to solve diagnostic problems.
摘要：
头颈部原发性促纤维增生性小圆细胞瘤（DSRCT）极为罕见。关于其临床病理特征的信息有限，预后,和治疗方式。这项研究的目的是提供主要发生在头颈部的DSRCT的全面审查，为了证明其独特的形态学和免疫组织化学表达，并解决鉴别诊断。经过对相关文献的全面回顾，共收集了25例病例。DSRCT最常见于主要唾液腺，跟着眼睛。此外，一些病例被误解为低分化癌，尤因肉瘤,和嗅觉神经母细胞瘤.诊断头部和颈部的DSRCT可能非常具有挑战性，因为它们在这个位置很少，重叠形态，和免疫组织化学。在这些情况下,遵循系统的方法有助于解决诊断问题。
