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下颌骨原发性骨内混合性施万瘤-神经束膜瘤

Primary intra-osseous Hybrid Schwannoma-Perineurioma in the mandible.

作者信息

Bergamini Mariana-Lobo, Noberto Luanna-Priscilla-Montenegro, Torres Gabriel-Barroso-Marocco-de Abreu, Soares Haroldo-Arid, Martins Fabiana, Costa Andre-Luiz-Ferreira, de Souza Suzana-Orsini-Cantanhede-Machado, Braz-Silva Paulo-Henrique

机构信息

Department of Stomatology, School of Dentistry, University of São Paulo, São Paulo, Brazil.

Division of Odontology, Municipal Hospital Carmino Caricchio, São Paulo, Brazil.

出版信息

J Clin Exp Dent. 2020 Sep 1;12(9):e888-e891. doi: 10.4317/jced.57035. eCollection 2020 Sep.

Abstract

Benign nerve sheath tumours include perineuriomas, schwannomas and neurofibromas. Hybrid schwannoma-perineurioma represents a cutaneous, subcutaneous or occasionally intra-osseous tumour with schwannian cytomorphology and perineurioma-like architecture consisting of a mixture of both types of cells. These tumours can develop at any age and there is no gender-predilection. Tongue is the most frequently affected site, followed by palate, mouth floor, jugal mucosa, lips and, more rarely, mandible. We present a case of hybrid tumour with schwannoma-perineurioma morphology located on the right mandibular body (intra-osseous) of a 54-year-old female patient. The tumour was symptomatic and evolving for six months. Microscopically, it was encapsulated and highly cellularised, presenting fascicular aspect and exhibiting scant mitotic activity. The tumour consisted of distinct cellular populations involving fusiform cells, cells with wavy and hyperchromatic nucleus or even epithelioid cells. Positive immunostaining for S-100 and epithelial membrane antigen (EMA) was observed. The lesion was completely removed under general anaesthesia, with the patient showing no clinical or radiographic sign of relapse after two-year follow-up. Despite the limited knowledge on the pathogenesis of Hybrid Schwannoma-Perineurioma, these tumours seem to present a non-aggressive biological behaviour. Conservative surgery provides adequate solution without recurrence, even after a long-term follow-up. Nerve sheath tumours, Schwannoma, Perineurioma, Immunohistochemistry.

摘要

良性神经鞘瘤包括束状神经瘤、神经鞘瘤和神经纤维瘤。混合性神经鞘瘤-束状神经瘤是一种皮肤、皮下或偶尔为骨内的肿瘤,具有雪旺氏细胞形态学和束状神经瘤样结构,由两种类型的细胞混合组成。这些肿瘤可在任何年龄发生,无性别倾向。舌是最常受累的部位,其次是腭、口底、颊黏膜、唇,下颌骨较少受累。我们报告一例54岁女性患者右下颌骨体(骨内)的具有神经鞘瘤-束状神经瘤形态的混合性肿瘤病例。该肿瘤有症状且已发展6个月。显微镜下,它被包膜包裹且细胞高度密集,呈束状外观,有少量有丝分裂活性。肿瘤由不同的细胞群组成,包括梭形细胞、核呈波浪状且深染的细胞甚至上皮样细胞。观察到S-100和上皮膜抗原(EMA)免疫染色阳性。在全身麻醉下将病变完全切除,患者在两年随访后无临床或影像学复发迹象。尽管对混合性神经鞘瘤-束状神经瘤的发病机制了解有限,但这些肿瘤似乎表现出非侵袭性生物学行为。保守手术提供了充分的解决方案,即使经过长期随访也不会复发。神经鞘瘤、神经鞘瘤、束状神经瘤、免疫组织化学。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/9065/7511049/503a8847d22a/jced-12-e888-g001.jpg

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