鼻腔鼻窦促纤维增生性小圆细胞肿瘤：病例报告及文献复习。
Sinonasal desmoplastic small round cell tumor: a case report and review of the literature.
机构信息
Department of Otolaryngology, The Second Hospital of Shandong University, 247#, Beiyuan Street, Jinan, 250033, China.
Department of Otolaryngology, Weifang People's Hospital, No. 151, Guangwen Street, Kuiwen District, Weifang, 261000, China.
出版信息
BMC Cancer. 2019 Aug 31;19(1):868. doi: 10.1186/s12885-019-6076-4.
BACKGROUND
Desmoplastic small round cell tumor (DSRCT) is a rare malignancy with poor prognosis that generally involves the peritoneum. Its diagnosis can be achieved only by immunohistochemistry and cytogenetic studies.
CASE PRESENTATION
In the current report, a 55-year-old female was admitted in our hospital for evaluation of right eye epiphora and right nasal intermittent bleeding. Imaging examination revealed a large soft tissue mass in the right nasal cavity and ethmoid sinus. After an explorative surgery, the pathological findings confirmed the presentation of sinonasal DSRCT. Immunohistochemistry and cytogenetic studies confirmed the diagnosis of DSRCT in this patient. Surgical resection, chemotherapy, and radiotherapy was performed, and she died 2 months after operation.
CONCLUSION
This reported case draws attention to the importance of novel treatments and including DSRCT in the differential diagnosis of sinonasal tumors.
背景
促结缔组织增生性小圆细胞肿瘤（DSRCT）是一种预后不良的罕见恶性肿瘤，通常累及腹膜。其诊断只能通过免疫组织化学和细胞遗传学研究来实现。
病例介绍
在本报告中，一名 55 岁女性因右眼溢泪和右侧鼻腔间歇性出血而入院。影像学检查显示右侧鼻腔和筛窦有一大块软组织肿块。在进行了探查性手术后，病理结果证实为鼻内 DSRCT。免疫组织化学和细胞遗传学研究证实了该患者的 DSRCT 诊断。进行了手术切除、化疗和放疗，患者在手术后 2 个月死亡。
结论
本报告病例提请注意新型治疗方法的重要性，并将 DSRCT 纳入鼻窦肿瘤的鉴别诊断。
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