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复发性面部混合型神经鞘黏液瘤

Recurring mixed-type neurothekeoma of the face.

作者信息

Müller Cornelia S L, Tilgen Wolfgang, Kutzner H, Pföhler Claudia

出版信息

Dermatoendocrinol. 2009 Jul;1(4):220-2. doi: 10.4161/derm.1.4.9442.

Abstract

BACKGROUND

Neurothekeomas are rare, benign neoplasms, typically occurring in younger patients with a remarkable predilection for the female population. The nomenclature and derivation of these tumors is controversial. The rarity of this unusual skin tumor in daily routine histopathologic findings prompted the following report.

OBSERVATION

We report a 17-year-old girl with an asymptomatic nodule at the inner angle of the left eye with slow progression in size within 12 months to 1 cm in diameter. Multiple treatments with laser surgery were performed and yielded no persistent remission of the tumor. Histopathologic examination revealed a non-encapsulated dermal tumor, composed of multiple, closely situated medium-sized nodules, separated by a myxoid collagenrich stroma, without epidermal alteration. In summary, due to the histoarchitecture and immunoprofile of the tumor, a mixed-type cellular neurothekeoma was diagnosed.

CONCLUSION

We think that it is important to be aware of these uncommon soft tissue lesions and the pitfalls of mixed-type neurothekeomas that often cause diagnostic problems. The aim of this report is to help to avoid misdiagnoses of malignant mesenchymal tumors with serious consequences, including extensive surgical therapy or radiation.

摘要

背景

神经鞘黏液瘤是一种罕见的良性肿瘤,通常发生于年轻患者,女性群体尤为多见。这些肿瘤的命名和起源存在争议。这种不常见的皮肤肿瘤在日常组织病理学检查中较为罕见,因此有了以下报告。

观察

我们报告一例17岁女孩,左眼内眦有一无症状结节,12个月内大小缓慢进展至直径1厘米。多次进行激光手术治疗,肿瘤均未持续缓解。组织病理学检查显示为非包膜性真皮肿瘤,由多个紧密相邻的中等大小结节组成,结节间为富含黏液样胶原的间质,表皮无改变。综上所述,根据肿瘤的组织结构和免疫表型,诊断为混合型细胞性神经鞘黏液瘤。

结论

我们认为,认识到这些不常见的软组织病变以及混合型神经鞘黏液瘤常导致诊断困难的陷阱很重要。本报告的目的是帮助避免对恶性间充质肿瘤的误诊,以免造成包括广泛手术治疗或放疗等严重后果。

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