Hybrid neurofibroma/schwannoma of the orbit

Q4 Medicine Revista Espanola de Patologia Pub Date : 2023-04-01 DOI:10.1016/j.patol.2022.03.002

Lourdes Salazar-Huayna , Lourdes Naranjo , Cleofé Romagosa , Miguel Ángel Arcediano , Sahyly Siurana , Santiago Ramón y Cajal , Carme Dinarès

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Abstract

Hybrid neurofibroma/schwannoma is a rare variant of hybrid peripheral nerve sheath tumours (HPNST). A Medline search up to December 2021 identified only six cases of this tumour in the orbit. We report the case of a 78-year-old man who presented with left exophthalmos. Computed tomography showed a left intraconal orbital mass. The clinico-radiological diagnosis was consistent with an intraconal cavernous angioma. Orbitotomy was performed, obtaining an 18 × 16 × 11 mm mass. Two different morphologies were seen microscopically, diagnostic of hybrid neurofibroma/schwannoma. HPNSTs of the orbit are uncommon and most reported cases showed a hybrid neurofibroma/schwannoma morphology. Hybrid neurofibroma/schwannomas have been associated with neurofibromatosis and schwannomatosis. Local recurrences have been reported. The correct identification of these tumours is important due to their potential use as a syndromic marker.

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眼眶混合型神经纤维瘤/神经鞘瘤

混合型神经纤维瘤/神经鞘瘤是一种罕见的混合型外周神经鞘肿瘤。截至2021年12月，Medline的搜索只发现了6例眼眶中的这种肿瘤。我们报告一位78岁男性的病例，他表现为左眼球突出。计算机断层扫描显示左侧眼眶内肿块。临床放射学诊断与眼眶内海绵状血管瘤一致。行眼眶切开术，获得18×16×11mm的肿块。显微镜下可见两种不同的形态，诊断为混合性神经纤维瘤/神经鞘瘤。眼眶HPNST并不常见，大多数报告的病例显示混合性神经纤维瘤/神经鞘瘤形态。混合型神经纤维瘤/神经鞘瘤与神经纤维瘤病和神经鞘瘤病有关。据报道局部复发。这些肿瘤的正确识别是重要的，因为它们可能用作综合征标志物。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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