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具有横纹肌样特征的完整鼻窦癌。

-Intact Sinonasal Carcinoma with Rhabdoid Features.

作者信息

Mulry Erin, Blake Danielle M, Hegde Poornima, Falcone Todd E

机构信息

Department of Surgery, Division of Otolaryngology-Head and Neck Surgery, University of Connecticut, Farmington, CT, USA.

Department of Otolaryngology, RWJ Barnabas Health-Cooperman Barnabas Medical Center, Livingston, NJ, USA.

出版信息

Case Rep Otolaryngol. 2021 Nov 18;2021:6075130. doi: 10.1155/2021/6075130. eCollection 2021.

DOI:10.1155/2021/6075130
PMID:34840844
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC8616702/
Abstract

Sinonasal malignancies are known for their associated poor prognosis and diversity of histologic features. While poor prognosis is largely due to advanced disease at presentation, histologic features also play a significant role. Therefore, accurate pathologic diagnosis is of utmost importance. Here, we describe a 63-year-old male with chronic left-sided nasal obstruction and left-sided epistaxis who was found to have a large mass occupying most of the nasal cavity extending through the nasopharynx to just below the nasopharyngeal surface of the soft palate. During surgical excision, the mass was noted to originate from the floor of the maxillary sinus with erosion of the medial wall of the maxillary sinus. Pathology revealed a diagnosis of INI1-intact poorly differentiated composite carcinoma with rhabdoid phenotype and sarcomatoid and squamous cell carcinoma foci arising within an inverted papilloma. Included in this report is a detailed description of both the patient's medical course and this pathologically novel sinonasal neoplasm. We aim to elucidate this rare tumor's complex features in order to improve future diagnosis and stimulate prospective research on sinonasal malignancies with complex histology.

摘要

鼻窦恶性肿瘤以其预后差和组织学特征多样而闻名。虽然预后差主要是由于就诊时疾病已处于晚期,但组织学特征也起着重要作用。因此,准确的病理诊断至关重要。在此,我们描述了一名63岁男性,有慢性左侧鼻塞和左侧鼻出血症状,发现鼻腔内有一巨大肿块,占据大部分鼻腔,延伸至鼻咽部,直至软腭鼻咽面下方。手术切除时,注意到肿块起源于上颌窦底部,并伴有上颌窦内侧壁侵蚀。病理诊断为INI1完整的低分化复合癌,具有横纹肌样表型,在倒置乳头状瘤内出现肉瘤样和鳞状细胞癌灶。本报告详细描述了患者的病程以及这种病理上新颖的鼻窦肿瘤。我们旨在阐明这种罕见肿瘤的复杂特征,以改善未来的诊断,并推动对具有复杂组织学的鼻窦恶性肿瘤的前瞻性研究。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c886/8616702/3293a2dc22c8/CRIOT2021-6075130.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c886/8616702/a19b8e219975/CRIOT2021-6075130.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c886/8616702/3293a2dc22c8/CRIOT2021-6075130.002.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c886/8616702/a19b8e219975/CRIOT2021-6075130.001.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/c886/8616702/3293a2dc22c8/CRIOT2021-6075130.002.jpg

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本文引用的文献

1
SMARCA4-deficient Sinonasal Carcinoma: A Series of 10 Cases Expanding the Genetic Spectrum of SWI/SNF-driven Sinonasal Malignancies.SMARCA4 缺陷型鼻腔鼻窦癌:10 例系列病例拓宽了 SWI/SNF 驱动型鼻腔鼻窦恶性肿瘤的遗传学谱。
Am J Surg Pathol. 2020 May;44(5):703-710. doi: 10.1097/PAS.0000000000001428.
2
Clinical outcomes of sinonasal squamous cell carcinomas based on tumor etiology.基于肿瘤病因的鼻窦鳞状细胞癌的临床转归。
Int Forum Allergy Rhinol. 2017 May;7(5):508-513. doi: 10.1002/alr.21899. Epub 2017 Feb 16.
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SMARCA4-deficient Sinonasal Carcinoma.
SMARCA4缺陷型鼻窦癌
Head Neck Pathol. 2017 Dec;11(4):541-545. doi: 10.1007/s12105-017-0783-4. Epub 2017 Feb 7.
4
Prognostic Indicators for Salvage Surgery of Recurrent Sinonasal Malignancy.复发性鼻窦恶性肿瘤挽救性手术的预后指标
Otolaryngol Head Neck Surg. 2016 Jan;154(1):104-12. doi: 10.1177/0194599815606699. Epub 2015 Sep 30.
5
SMARCB1(INI1)-deficient sinonasal basaloid carcinoma: a novel member of the expanding family of SMARCB1-deficient neoplasms.SMARCB1(INI1)缺陷型鼻腔鼻窦基底细胞癌:SMARCB1 缺陷型肿瘤家族的新成员。
Am J Surg Pathol. 2014 Sep;38(9):1274-81. doi: 10.1097/PAS.0000000000000236.
6
INI1 expression is retained in composite rhabdoid tumors, including rhabdoid meningiomas.INI1表达在复合性横纹肌样肿瘤中保留,包括横纹肌样脑膜瘤。
Mod Pathol. 2005 Jul;18(7):951-8. doi: 10.1038/modpathol.3800375.