Adamantinoma-like Ewing Sarcoma of the Nasal Cavity: A Rare Clinical Presentation with Deceptive Histopathologic Features.

Head and neck pathology Pub Date : 2022-09-01 Epub Date: 2021-12-02 DOI:10.1007/s12105-021-01396-4

Sunil Pasricha, Saloni Pahwa, Ankush Jajodia, Sumit Goyal, Mudit Agarwal, Ankur Kumar, Gurudutt Gupta, Anila Sharma, Garima Durga, Meenakshi Kamboj, Anurag Mehta

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引用次数: 2

Abstract

Ewing sarcoma (ES) is a malignant round cell tumour (MRCT) that usually involves bone and soft tissue of young and paediatric populations. ES of the head and neck region is uncommon. Adamantinoma-like Ewing sarcoma (ALES) is a rare variant of ES that shows complex epithelial differentiation on histopathology and immunohistochemistry (IHC). It demonstrates a t(11;22) translocation and EWSR1- FLI1 fusion. Most documented cases of ALES of the head and neck region were initially misdiagnosed as epithelial tumours. We present a rare case of ALES of the nasal cavity in a young female. The patient subsequently underwent chemotherapy and showed an excellent response. Awareness of this entity is important for pathologists and oncologists due to its distinct therapeutic and prognostic implications. We propose performing upfront NKX2.2 and CD99 IHC studies, as well as other lineage specific IHC markers, in any poorly differentiated MRCT of head and neck region.

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鼻腔金刚素瘤样尤文氏肉瘤:一种罕见的临床表现和具有欺骗性的组织病理特征。

尤文氏肉瘤(ES)是一种恶性圆细胞瘤(MRCT)，通常累及骨骼和软组织的年轻人和儿童人群。头颈部ES并不常见。金刚素瘤样尤文氏肉瘤(ALES)是一种罕见的ES变体，在组织病理学和免疫组化(IHC)上表现出复杂的上皮分化。它显示了t(11;22)易位和EWSR1- FLI1融合。大多数记录的头颈部ALES病例最初被误诊为上皮性肿瘤。我们提出一个罕见的情况下，ALES的鼻腔在一个年轻的女性。患者随后接受了化疗，并表现出良好的反应。意识到这个实体是重要的病理学家和肿瘤学家由于其独特的治疗和预后意义。我们建议在头颈部任何低分化MRCT中进行NKX2.2和CD99免疫组化研究，以及其他谱系特异性免疫组化标记。

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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Head and neck pathology

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