Maiti Mousam, Dutta Mainak, Datta Jayati, Mukherjee Arundhati, Ghosh Debangshu, Mallick Ajay
Department of Otorhinolaryngology and Head-Neck Surgery, All India Institute of Medical Sciences, Kalyani, NH-34 Connector, Basantapur, Saguna, Nadia, Kalyani, West Bengal, 741245, India.
Department of Pathology, Drs. Tribedi & Roy Diagnostic Laboratory, Kolkata, West Bengal, India.
Eur Arch Otorhinolaryngol. 2025 May;282(5):2745-2751. doi: 10.1007/s00405-024-09154-2. Epub 2024 Dec 26.
Clinicopathologic illustration of sinonasal teratocarcinosarcoma (SNTCS) in a middle-aged man, highlighting the difficulties and challenges encountered during surgical intervention, histopathologic diagnosis, and its overall management.
Case report and literature review.
A 40-year-old man having recurrent epistaxis for three months presented with a dark-colored protruding polypoid nasal mass. Magnetic resonance imaging revealed a large, heterogeneous gadolinium-enhanced infiltrative lesion in the left nasal cavity with a T2-hypointense trans-septal zone. The mass abutted the cribriform plate, lamina papyracea, and septum, involved the inferior and middle turbinates, and blocked the choanae through the nasopharynx. There was profuse hemorrhage when an endoscope-assisted biopsy was attempted. Histopathology from the debulked specimen suggested SNTCS. The diagnosis was confirmed on immunohistochemistry. A metastatic search turned negative. However, at three weeks, the patient returned with a recurrence. A repeat debulking was done, and he was thereafter put on adjuvant chemotherapy. A second recurrence/residual lesion was noted midway through the chemotherapy regimen. However, it resolved after completion of the chemotherapy and subsequent image-guided radiotherapy, and the patient continued to be disease-free till the last follow-up at six weeks postradiotherapy.
SNTCS is an extremely aggressive malignancy that is seldom encountered in routine otolaryngology and pathology practice, with only a few reports of SNTCS documented. It is histologically composed of epithelial, mesenchymal, and primitive neuroectodermal elements with areas of undifferentiation. They are notorious for troublesome intra-operative bleeding, making complete surgical excision difficult, and the absence of a tumor-free margin often results in recurrence. Owing to their variegated and heterogeneous tissue composition, histopathologic diagnosis is enormously challenging without a representative tissue sample and immunohistochemistry. In spite of prompt and energetic multimodality treatment, survival rate is dismal.
对一名中年男性鼻窦畸胎癌肉瘤（SNTCS）进行临床病理说明，强调手术干预、组织病理学诊断及其整体管理过程中遇到的困难和挑战。
病例报告及文献复习。
一名40岁男性，反复鼻出血3个月，就诊时发现鼻腔有一深色突出的息肉样肿物。磁共振成像显示左侧鼻腔有一个大的、不均匀的钆增强浸润性病变，伴有一个T2低信号的跨鼻中隔区域。肿物毗邻筛板、纸样板和鼻中隔，累及下鼻甲和中鼻甲，并通过鼻咽阻塞后鼻孔。在内镜辅助活检时出现大量出血。减瘤标本的组织病理学提示为SNTCS。免疫组化确诊。转移检查结果为阴性。然而，三周后患者复发。再次进行减瘤手术，之后给予辅助化疗。在化疗方案进行到一半时发现第二次复发/残留病变。然而，在化疗及随后的影像引导放疗完成后病变消退，患者在放疗后六周的最后一次随访时仍无疾病复发。
SNTCS是一种极具侵袭性的恶性肿瘤，在常规耳鼻喉科和病理学实践中很少见，仅有少数SNTCS的报道。它在组织学上由上皮、间充质和原始神经外胚层成分组成，伴有未分化区域。它们因术中出血麻烦而臭名昭著，使得完整的手术切除困难，且无肿瘤切缘常导致复发。由于其组织构成多样且异质性，在没有代表性组织样本和免疫组化的情况下，组织病理学诊断极具挑战性。尽管采取了迅速而积极的多模式治疗，生存率仍然很低。
