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一名8岁儿童的咽旁间隙混合性周围神经鞘瘤,具有神经纤维瘤和施万细胞瘤的特征——一种罕见的实体。

Hybrid peripheral nerve sheath tumor of parapharyngeal space having features of neurofibroma and schwannoma in an 8-year-old child - A rare entity.

作者信息

Chaturvedi Himani Tiwari, Chaturvedi Chandrashekher, Nandy Dipayan Jayanta

机构信息

Department of Oral Pathology, Dharmsinh Desai University, Nadiad, Gujarat, India.

Department of Surgery, Gujarat Surgical Hospital, Vadodara, Gujarat, India.

出版信息

J Oral Maxillofac Pathol. 2022 Feb;26(Suppl 1):S96-S102. doi: 10.4103/jomfp.jomfp_123_21. Epub 2022 Feb 28.

Abstract

Tumors of the parapharyngeal space (PPS) are extremely rare inpediatric age group. Out of all head-and-neck neoplasms, PPS tumors comprise only 0.5%. Majority of neoplasms in poststyloid PPS are neurogenic tumors. Conventional benign peripheral nerve sheath tumor (PNST) includes neurofibromas, schwannomas and perineuriomas. Hybrid PNSTs are rare entities having combined features of more than one histologic type, and the World Health Organization Classification of Tumors of the Central Nervous System, in the latest 2016, 4 edition, has recently recognized and published it. The most common hybrid tumor is schwannoma/perineuroma followed by neurofibroma/schwannoma and neurofibroma/perineuroma. Here, we are reporting a hybrid PNST which was completely excised and having combined features of neurofibroma and schwannoma, confirmed by both histopathologically and immunohistochemically, in an 8-year-old child, in PPS.

摘要

咽旁间隙(PPS)肿瘤在儿童年龄组中极为罕见。在所有头颈肿瘤中,PPS肿瘤仅占0.5%。茎突后PPS的大多数肿瘤是神经源性肿瘤。传统的良性周围神经鞘瘤(PNST)包括神经纤维瘤、神经鞘瘤和神经束膜瘤。混合性PNST是具有多种组织学类型组合特征的罕见实体,世界卫生组织中枢神经系统肿瘤分类在2016年最新第4版中最近已认可并公布。最常见的混合性肿瘤是神经鞘瘤/神经束膜瘤,其次是神经纤维瘤/神经鞘瘤和神经纤维瘤/神经束膜瘤。在此,我们报告一例在PPS的8岁儿童中完全切除的混合性PNST,经组织病理学和免疫组织化学证实具有神经纤维瘤和神经鞘瘤的组合特征。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/8b4f/9017848/a7280ddb44b7/JOMFP-26-96-g001.jpg

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