Yang Shudong, Sun Rongchao, Liang Jiabei, Zhou Zhiyi, Zhou Jing, Rui Jun
Wuxi People's Hospital, Nanjing Medical University, Wuxi, Jiangsu, People's Republic of China.
Int J Surg Pathol. 2013 Feb;21(1):37-43. doi: 10.1177/1066896912457202. Epub 2012 Aug 24.
The goal of this study was to assess the pathological and differential diagnoses of sinonasal teratocarcinosarcoma (SNTCS) in order to ultimately improve the diagnosis and treatment of this rare disease. Data from 2 cases of sinonasal teratocarcinosarcoma from the Wuxi People's Hospital (China) were analyzed. The clinical presentation for these patients consisted of nasal obstruction, epistaxis, and headache. On further physical examination, the presence of a polypoid mass was identified and, despite surgery and radiotherapy, both cases experienced recurrence. Histologically, the tumors showed a heterogeneous mixture of components from the 3 germ layers, primitive neuroepithelial elements, diagnostic immature squamous cell nests (clear cell nests), and various epithelial and mesenchymal components. Staining of the different germ layers corresponded with the appropriate immune markers. In case 1, the postradiotherapy resection specimen was completely dominated by a mature teratoma, with a complete absence of the corresponding adenocarcinoma and fibrosarcoma components. To date, this is the first study describing this composition within an SNTCS recurrent tumor. In summary, SNTCS is a rare tumor characterized by the presence of benign and malignant epithelial, mesenchymal, and dysembryomal components. Owing to its heterogeneous histologic appearance, adequate sampling and recognition of all SNTCS components are needed for future diagnosis. Currently, surgical removal, postoperative radiotherapy, and a histology-specific multidrug chemotherapy appear to be the best therapeutic approach. Future individualized therapy may also hold promise.
本研究的目的是评估鼻窦畸胎癌肉瘤（SNTCS）的病理及鉴别诊断，以最终改善这种罕见疾病的诊断和治疗。对来自无锡市人民医院（中国）的2例鼻窦畸胎癌肉瘤病例数据进行了分析。这些患者的临床表现包括鼻塞、鼻出血和头痛。进一步体格检查发现有息肉样肿物，尽管进行了手术和放疗，两例均出现复发。组织学上，肿瘤显示出由三个胚层、原始神经上皮成分、具有诊断意义的未成熟鳞状细胞巢（透明细胞巢）以及各种上皮和间充质成分组成的异质性混合物。不同胚层的染色与相应的免疫标志物相符。在病例1中，放疗后切除标本完全由成熟畸胎瘤主导，完全没有相应的腺癌和纤维肉瘤成分。迄今为止，这是首次描述SNTCS复发性肿瘤中这种成分的研究。总之，SNTCS是一种罕见肿瘤，其特征是存在良性和恶性上皮、间充质及胚胎发育异常成分。由于其组织学表现异质性，未来诊断需要对所有SNTCS成分进行充分取材和识别。目前，手术切除、术后放疗以及组织学特异性多药化疗似乎是最佳治疗方法。未来个体化治疗也可能大有希望。
