鼻腔鼻窦畸胎癌肉瘤7例临床病理分析

Sinonasal teratocarcinosarcoma:a clinicopathological analysis of seven cases

目的探讨鼻腔鼻窦畸胎癌肉瘤(SNTCS)的临床病理特征、鉴别诊断及预后。方法分析7例SNTCS的临床病理及免疫表型特点,其中3例用荧光原位杂交(FISH)法检测12p基因,并进行随访及复习相关文献。结果男性5例,女性2例,平均年龄54岁。7例均位于鼻腔、鼻窦,其中2例累及颅内额叶。镜下可见良性及恶性上皮、间叶、神经成份混杂分布,上皮包括良性纤毛柱状上皮、腺体及鳞状上皮(部分为胞质透明鳞状上皮)、腺癌,间叶成份包括梭形纤维母细胞、平滑肌、软骨、骨、纤维肉瘤、软骨肉瘤、横纹肌肉瘤,神经成份主要为原始神经上皮伴菊形团形成,可见神经纤维基质及神经元分化。不同肿瘤成分表达相应上皮、间叶、神经的免疫组化标记,其中原始神经成份局部Calretinin及TTF1阳性,3例行FISH检测12p示均无扩增。7例术后均行放疗和(或)化疗,2例死亡,5例存活。结论 SNTCS肿瘤组织学形态复杂多样,主要由畸胎瘤样和癌肉瘤样成分构成,确诊需多取材、依赖病理组织学及免疫组化。

Objective To investigate the clinicopathological feature,differential diagnosis and prognosis of sinonasal teratocarcinosarcoma(SNTCS).Methods The clinicopathological and immunohistochemical features of 7 SNTCS cases were analyzed,12 p gene’s status of 3 cases were detected by FISH with follow-up and related literatures were reviewed.Results The patients were 5 males and 2 females,with average age of 54 years.All tumors were located in the nasal cavity and paranasal sinus,and 2 cases involved intracranial frontal lobe.Microscopically,all 7 tumors were composed of admixed benign and malignant epithelia,mesenchyme and neuroepithelium.Epithelial elements included benign ciliated columnar epithelium,glandular and squamous epithelium(some squamous cells with clear cytoplasm)and adenocarcinoma.The mesenchymal elements consisted of fusiform fibroblast,smooth muscle,cartilage,bone,fibrosarcoma,chondrosarcoma,and rhabdomyosarcoma.Neuroepithelial elements demonstrated primitive neuroepithelial cells with focal rosette and neuropil,and neuron formation.Different components expressed corresponding epithelial,mesenchymal,neuroepithelial immunophenotypes.Calretinin and TTF1 were locally expressed in primitive neuroepithelial elements.Fluorescent in situ hybridization detected absence of 12 p amplification in 3 cases.All 7 patients underwent radiotherapy and/or chemotherapy after surgical resection,among them 2 died and 5 survived.Conclusion SNTCS is characterized by the complicated morphology,mainly composed of malignant teratoma-like and carcinosarcoma-like elements.The final definite diagnosis requires thorough sampling and relies on histopathologic and immunohistochemical results.