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鼻窦畸胎样癌肉瘤

Teratoid carcinosarcoma of the paranasal sinuses.

作者信息

Shanmugaratnam K, Kunaratnam N, Chia K B, Chiang G S, Sinniah R

出版信息

Pathology. 1983 Oct;15(4):413-9. doi: 10.3109/00313028309085168.

DOI:10.3109/00313028309085168
PMID:6674870
Abstract

Malignant tumours with teratoid or blastomatous features are exceptionally rare in the upper respiratory tract with only 8 documented cases, including the 3 in this report. The tumours occurred in adults aged 27 to 62 yr, and the sites of origin were the ethmoid sinus (4 cases), ethmoid and other paranasal sinuses (2 cases), unspecified sinuses (1 case) and nasopharynx (1 case). The disease was rapidly fatal in 3 cases in which treatment was restricted to surgery and was associated with longer survivals in those given supportive radiotherapy. The tumours are locally aggressive and may invade soft tissues, bone, orbit and cranial cavity. Histologically, the tumours are characterized by a mixture of epithelial and mesenchymal components including cellular elements with immature or embryonal characteristics. These tumours, variously termed malignant teratoma, blastoma, teratocarcinoma or teratoid carcinosarcoma, probably comprise a homogeneous group of neoplasms since their histological and biological features are essentially similar. It is postulated that the tumours develop from primitive embryonic tissues or pluripotential cells that have remained sequestered in the sinonasal tract.

摘要

具有畸胎样或胚细胞瘤特征的恶性肿瘤在上呼吸道极为罕见,仅有8例文献记载,包括本报告中的3例。这些肿瘤发生于27至62岁的成年人,起源部位为筛窦(4例)、筛窦及其他鼻窦(2例)、未明确的鼻窦(1例)和鼻咽(1例)。3例患者仅接受手术治疗,病情迅速恶化,而接受辅助放疗的患者生存期较长。这些肿瘤具有局部侵袭性,可侵犯软组织、骨骼、眼眶和颅腔。组织学上,肿瘤的特征是上皮和间充质成分混合,包括具有不成熟或胚胎特征的细胞成分。这些肿瘤,被分别称为恶性畸胎瘤、胚细胞瘤、畸胎癌或畸胎样癌肉瘤,可能构成一组同质的肿瘤,因为它们的组织学和生物学特征基本相似。据推测,这些肿瘤起源于原始胚胎组织或多能细胞,这些细胞一直隐匿于鼻窦道中。

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