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喉部孤立性纤维瘤:罕见表现下的诊断挑战。

Laryngeal solitary fibrous tumour: diagnostic challenge in a rare presentation.

机构信息

Mid and South Essex NHS Foundation Trust, UK.

The Princess Alexandra Hospital NHS Trust, UK.

出版信息

Ann R Coll Surg Engl. 2022 Jul;104(7):e219-e225. doi: 10.1308/rcsann.2021.0332. Epub 2022 Apr 21.

Abstract

Solitary fibrous tumours (SFT) are rare spindle cell mesenchymal neoplasms, most commonly appearing as well-circumscribed localised lesions arising from the abdominal or pelvic peritoneum. Their presence in the head and neck region is very rare; even more so in the post-cricoid region, with only one case described in the literature to date. The clinical behaviour of SFT may vary from benign to malignant, but only 10-15% of SFT demonstrate local recurrence or distant metastasis. The usual presenting symptoms of dysphagia and weight loss are very vague to give a definite diagnosis, and examination, even under general anaesthesia, combined with biopsies can sometimes be misleading, as in our case. Here, we describe a very challenging case of supraglottic SFT originating from the post-cricoid area. Our 73-year-old patient presented with dysphagia and upon examination a large supraglottic mass was revealed. Biopsies from the mass pointed to malignancy and the patient underwent total laryngectomy. The laryngeal specimen contradicted the biopsy and revealed a rare solitary fibrous tumour with benign features and thus adjuvant treatment was not necessary. Ever since the patient has been attending regular follow-up sessions and remains disease-free 18 months after surgery.

摘要

孤立性纤维瘤(SFT)是一种罕见的梭形细胞间叶性肿瘤,最常表现为起源于腹部或盆腔腹膜的边界清楚的局限性病变。它们在头颈部的存在非常罕见;在环后区更为罕见,迄今为止,文献中仅描述了一例。SFT 的临床行为可能从良性到恶性不等,但只有 10-15%的 SFT 表现出局部复发或远处转移。通常的表现症状为吞咽困难和体重减轻,这些症状非常模糊,无法做出明确的诊断,即使在全身麻醉下进行检查并结合活检,有时也会产生误导,就像我们的病例一样。在这里,我们描述了一个非常具有挑战性的起源于环后区的会厌上 SFT 病例。我们的 73 岁患者因吞咽困难就诊,检查发现会厌区有一个大的肿块。肿块的活检提示恶性肿瘤,患者接受了全喉切除术。喉部标本与活检结果不符,显示为罕见的具有良性特征的孤立性纤维瘤,因此无需辅助治疗。自那时以来,患者定期接受随访,手术后 18 个月仍无疾病。

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本文引用的文献

1
Laryngeal Solitary Fibrous Tumor: A Case Report and Systematic Review.喉孤立性纤维瘤:一例报告及系统综述
J Voice. 2021 Jan;35(1):136-142. doi: 10.1016/j.jvoice.2019.08.010. Epub 2019 Sep 26.
2
Solitary fibrous tumor.孤立性纤维性肿瘤
Transl Gastroenterol Hepatol. 2018 Nov 21;3:94. doi: 10.21037/tgh.2018.11.02. eCollection 2018.
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Immunohistochemistry in diagnosis of soft tissue tumours.免疫组织化学在软组织肿瘤诊断中的应用。
Histopathology. 2011 Jun;58(7):1001-12. doi: 10.1111/j.1365-2559.2010.03707.x. Epub 2010 Dec 10.
8
Solitary fibrous tumor of the larynx.喉孤立性纤维瘤
Head Neck Pathol. 2008 Jun;2(2):67-74. doi: 10.1007/s12105-008-0044-7. Epub 2008 Mar 15.

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