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头皮多形性皮肤肉瘤伴颅内间隙受累:罕见病例的处理。病例说明

Pleomorphic dermal sarcoma of the scalp with intracranial space involvement: management of a rare entity. Illustrative case.

作者信息

Altieri Roberto, Cuomo Roberto, Vitale Giovanni, Limongelli Sebastiano, Massariello Domenico, La Rocca Giuseppe, Corvino Sergio, Barbarisi Manlio

机构信息

Multidisciplinary Department of Medical-Surgical and Dental Specialties, University of Campania "Luigi Vanvitelli," Naples, Italy.

Department of Medicine, Plastic Surgery and Neuroscience, University of Siena, Siena, Italy.

出版信息

J Neurosurg Case Lessons. 2024 Dec 30;8(27). doi: 10.3171/CASE24457.

DOI:10.3171/CASE24457
PMID:39740210
原文链接:https://pmc.ncbi.nlm.nih.gov/articles/PMC11694190/
Abstract

BACKGROUND

Exophytic tumors of the calvaria (ETCs) remain a challenging pathology because of their complex management. The authors discuss the case of a woman with a large exophytic mass of the right frontotemporal region and underline their decision-making process on the management of this unique case and possible similar ones.

OBSERVATIONS

Neuroradiological findings showed a calvarial tumor with both epicranial and intracranial extension involving the frontotemporal bone with a mixed component (lytic and sclerotic) and dural infiltration with a pseudonodular pattern. A wide en bloc excision from the skin to the dura mater was performed. The compound 5-aminolevulinic acid (5-ALA) was not very useful in identifying the tumor boundaries. One-step cranioplasty and a skin pedicle flap were used to reconstruct the anatomical defect. Acellular dermal matrix was used to repair the uncovered calvaria. Pathological examination confirmed the diagnosis of pleomorphic dermal sarcoma (PDS).

LESSONS

In the evaluation of an ETC, PDS should be considered. Wide en bloc excision, if achievable, should be considered the gold standard. The 5-ALA was not helpful, and a pedicle skin flap could be considered as a less invasive alternative to microsurgical reconstruction. The use of an acellular matrix implant on the contralateral exposed galea increases the rate of reconstruction success. https://thejns.org/doi/10.3171/CASE24457.

摘要

背景

由于颅骨外生性肿瘤(ETC)的治疗复杂,其仍然是一个具有挑战性的病理学问题。作者讨论了一名患有右侧额颞部巨大外生性肿物的女性病例,并强调了他们对该特殊病例及可能的类似病例的治疗决策过程。

观察结果

神经放射学检查结果显示,颅骨肿瘤累及额颞骨,有颅外和颅内扩展,具有混合成分(溶骨性和硬化性),并呈假结节样硬脑膜浸润。从皮肤至硬脑膜进行了广泛的整块切除。复方5-氨基酮戊酸(5-ALA)在确定肿瘤边界方面不太有用。采用一步颅骨成形术和带蒂皮瓣修复解剖缺损。使用脱细胞真皮基质修复裸露的颅骨。病理检查确诊为多形性皮肤肉瘤(PDS)。

经验教训

在评估ETC时,应考虑PDS。如果可行,广泛整块切除应被视为金标准。5-ALA没有帮助,带蒂皮瓣可被视为一种侵入性较小的显微外科重建替代方法。在对侧暴露的帽状腱膜上使用脱细胞基质植入物可提高重建成功率。https://thejns.org/doi/10.3171/CASE24457 。

https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/bd2f30398f92/CASE24457_figure_5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/6e4e08600b20/CASE24457_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/e59017d7c61b/CASE24457_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/0403d9bf5b19/CASE24457_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/64b3941e0304/CASE24457_figure_4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/bd2f30398f92/CASE24457_figure_5.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/6e4e08600b20/CASE24457_figure_1.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/e59017d7c61b/CASE24457_figure_2.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/0403d9bf5b19/CASE24457_figure_3.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/64b3941e0304/CASE24457_figure_4.jpg
https://cdn.ncbi.nlm.nih.gov/pmc/blobs/24e3/11694190/bd2f30398f92/CASE24457_figure_5.jpg

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Pleomorphic dermal sarcoma of the scalp: Review of management and distinguishing features from atypical fibroxanthoma.头皮多形性皮肤肉瘤:治疗及与非典型纤维黄色瘤鉴别特征的综述
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