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SMARCB1(INI-1)缺失性鼻腔鼻窦癌的病理临床特征分析

Pathologic and clinical characteristics of SMARCB1(INI-1)-deficient sinonasal carcinoma

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目的 总结SMARCB1(INI-1)缺失性鼻腔鼻窦癌(SDSC)的临床特征、组织形态、免疫组化表达、诊断、鉴别诊断、治疗及随访状况,提高对该病的认识水平.方法 对深圳大学总医院诊治的1例SDSC患者的临床及病理相关资料进行整理,回顾性分析相关文献.结果 患者TNM分期为T3N0M0;CT和MRI显示右侧鼻腔及筛窦内占位,伴骨质破坏;组织学见肿瘤细胞形态较为一致,伴轻度多形性,呈上皮样或基底细胞样形态,核分裂像较易见,未见明显鳞状或腺样分化特征,肿瘤周边可见少许残留的正常黏膜上皮;免疫组化染色分析见肿瘤细胞INI-1细胞核表达完全缺失.结论 SDSC是鼻窦未分化癌的一种新亚型,较为罕见;具有高度侵袭性,易复发、早期转移、预后差等特点;形态学分化差的鼻腔鼻窦癌行INI-1免疫组化检测,有助于该亚类的准确诊断;术前TPF(多西他赛+顺铂+5-氟尿嘧啶)化疗方案促使病灶缩小,有助于根治性手术切除.
Objective To Summary the clinical characteristics,histological morphology,immunophenotype,diagnostic features,differential diagnosis,treatment and follow-up of SMARCB1(INI-1)deficient sinonasal carcinoma(SDSC),and deepen the understanding of this disease.Methods The clinicopathologic data of one SDSC patient diagnosed and treated in Shenzhen University General Hospital were retrospectively and literatures were reviewed.Results The patient's TNM stage was T3N0M0.The computed tomography(CT)and magnetic resonance image(MRI)showed a mass occupying the right nasal cavity and ethmoid sinus with bone destruction.Microscopically,the tumor cells were composed of epithelioid or basal cell-like morphology,most were consistent with mild pleomorphism.The mitotic image was easy to see,no obvious squamous or adenoid differentiation was observed,and a few residual normal mucosal epithelium could be seen around the tumor.Immunohistochemical staining showed that INI-1 nuclear expression of tumor cells was completely absent.Conclusions SDSC is a new subtype of undifferentiated carcinoma of sinuses,which is relatively rare.It is highly invasive with poor prognosis,easy to relapse and early metastasis.INI-1 immunohistochemical detection was performed for the poorly differentiated carcinoma of sinonasal,which was helpful for the accurate diagnosis of this subcategory.Preoperative TPF(Docetaxel+cisplatin+5-fluorouracil)chemotherapy helps to shrink the lesion and facilitate radical surgical excision.

SMARCB1(INI-1)-deficient sinonasal carcinomaPathologyCytologyClinical characteristics

王军、李文婷、孟宇宏、付先利、李丽明、张庆丰、吕云罡

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深圳大学总医院病理科,广东深圳 518071

深圳大学总医院耳鼻咽喉头颈外科,广东深圳 518071

深圳大学总医院影像科,广东深圳 518071

SMARCB1(INI-1)缺失性鼻腔鼻窦癌 病理 细胞学 临床特征

深圳市医疗卫生三名工程项目

SZSM201911007

2024

中国耳鼻咽喉颅底外科杂志
中南大学

中国耳鼻咽喉颅底外科杂志

CSTPCD
影响因子:0.643
ISSN:1007-1520
年,卷(期):2024.30(3)
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