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宫颈乳腺外肌纤维母细胞瘤——一种罕见的实体瘤。

Cervical Extra-Mammary Myofibroblastoma- An Unusual Entity.

作者信息

Wee Eric Tan Jin, Sairin Mohd Eksan, Ahmad Abd Razak, Hassan Fazilah, Lazim Norhafiza Mat

机构信息

Department of Otorhinolaryngology-Head and Neck Surgery, School of Medical Sciences, Universiti Sains Malaysia, Kubang Kerian, Kelantan 16150 Malaysia.

Department of Otorhinolaryngology, Hospital Melaka, Melaka, Malaysia.

出版信息

Indian J Otolaryngol Head Neck Surg. 2025 May;77(5):2164-2167. doi: 10.1007/s12070-025-05462-z. Epub 2025 Apr 22.

Abstract

Extra-mammary myofibroblastoma is a rare, benign spindle cell lesion mainly found in the axillary and inguinal regions. It strictly resembles its mammary counterpart with few reported cases occurring in the head and neck regions. We present a case of a middle aged woman with right sided anterior neck swelling. A diagnosis could not be made on tissue biopsy and the swelling persistently bothering the patient, thus surgical removal had been done under general anaesthesia. Histological examination showed spindle cells in clusters, with positive CD34 and desmin expression and also positive Rb1 expression. In myofibroblastoma Rb1 expression should be lost in majority of the cases. In view of histopathological dilemma, the sample was sent for fluorescence in situ hybridization (FISH) analysis. The final diagnosis of extra-mammary myofibroblastoma was made. This case is rare due to the unusual location of this tumour. Furthermore, we want to highlight the difficulties in differentiating between its malignant counterpart on pathological examination. Extra-mammary myofibroblastoma carry an excellent prognosis with low reoccurrence rate after surgical removal.

摘要

乳腺外肌纤维母细胞瘤是一种罕见的良性梭形细胞病变,主要见于腋窝和腹股沟区。它与乳腺肌纤维母细胞瘤极为相似,仅有少数病例发生于头颈部。我们报告一例中年女性,右侧颈前肿胀。组织活检未能明确诊断,且肿胀持续困扰患者,因此在全身麻醉下进行了手术切除。组织学检查显示梭形细胞呈簇状,CD34和结蛋白表达阳性,Rb1表达也为阳性。在肌纤维母细胞瘤中,大多数病例Rb1表达应缺失。鉴于组织病理学上的困境,将样本送去进行荧光原位杂交(FISH)分析。最终诊断为乳腺外肌纤维母细胞瘤。该病例因肿瘤位置不寻常而罕见。此外,我们想强调在病理检查中区分其恶性对应物的困难。乳腺外肌纤维母细胞瘤预后良好,手术切除后复发率低。

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