Low-grade myofibroblastic sarcoma of the palate

Low-grade myofibroblastic sarcoma(LGMS) is a rare,malignant tumor with myofibroblastic differentiation.Despite it being classified as a distinct entity by the World Health Organization,a few cases were reported in the oral and maxillofacial region.Here,a LGMS developed on the palate of a 73-year-old man who presented with a 1-cm tumor on the posterior border of the palate.Based on the histological and immunohistochemical features,a diagnosis of LGMS was established.The tumor was resected,and no recurrence was observed over 2 years.Although the tongue is the most preferred site for LGMS,it may occur in any region of the oral cavity.     

鼻腔鼻窦低度恶性肌纤维母细胞肉瘤一例

正1病例资料患者,女,51岁。因"反复鼻阻5月余,加重2月余"于2017年2月3日入院。既往史:2007年行鼻中隔矫正术,2012年行左鼻息肉手术,余无特殊。入院专科情况:外鼻无畸形,双侧鼻腔见大量样新生物堵塞(图1),鼻中隔后份骨质破坏,右侧鼻腔新生物来源于左侧鼻腔,表面血供丰富,鼻腔未见明显分     

上颌窦低级别肌纤维母细胞肉瘤的临床诊治

目的探讨上颌窦低级别肌纤维母细胞肉瘤的临床特点、诊断和治疗。方法回顾性分析4例上颌窦低级别肌纤维母细胞肉瘤患者的临床资料和病理特征。结果 4例患者均采用上颌骨切除术,其中1例为上颌骨扩大切除,1例患者行术前放疗且剂量大于60 Gy。术后随访6个月至3年,未见复发,未出现其他并发症。结论上颌窦低级别肌纤维母细胞肉瘤为低度恶性肉瘤,术中冰冻切片诊断困难,确诊需术后病理及免疫组化结果。根据病灶范围行上颌骨切除或扩大切除达到根治肿瘤的目的。     

头颈部低度恶性肌纤维母细胞肉瘤二例分析

目的 探讨头颈部低度恶性肌纤维母细胞肉瘤(LGMS)的临床表现、影像学表现、病理表现、治疗方法及预后。 方法 回顾性分析2例头颈部LGMS患者的临床资料并复习相关文献,总结其临床表现、影像学表现、病理表现及治疗方法、预后情况。 结果 头颈部LGMS的临床表现取决于肿瘤的部位,声门区的LGMS以声音嘶哑为首发症状,随着肿瘤增大可出现呼吸困难,颈部的LGMS表现为无痛性肿块。肿瘤表面光滑,似有被膜,界不清,基底广泛,表面可有坏死。CT表现为均匀或不均匀的中等密度肿瘤,肿瘤内可见钙化,偶可破坏骨质,增强后呈现轻度均匀强化或环形强化。MRI扫描T₁W2呈低信号或等限号,T₂W1呈均匀或不均匀高信号,肿瘤内有条索信号影,增强后肿瘤实质呈周边明显强化表现。光镜下见肿瘤细胞由梭形细胞和小多角形细胞组成,呈束状、疏松状、编织状排列,弥漫性浸润性生长,可侵犯黏膜鳞状上皮、肌肉,肿瘤细胞界限不清,纺锤形,核为细长型,核增大、深染、不规则。免疫组化见Vimentin、SMA、desmin、actin等阳性表达,S-100等阴性表达。治疗方法以广泛手术切除为主,术后随访12~23个月,均无复发及转移。 结论 头颈部LGMS临床及影像学表现无特异性,病理诊断必须结合免疫组织化学检查,广泛手术切除是最有效的治疗方法,肿瘤对放化疗不敏感,肿瘤的复发与是否切除彻底有关。     

颞骨炎性肌纤维母细胞瘤1例报告并文献复习

目的：探讨颞骨炎性肌纤维母细胞瘤的临床表现,术中所见,以及组织病理学特征。方法：结合文献复习,报告1例颞骨炎性肌纤维母细胞瘤患者的临床资料并行光镜检查和免疫组织化学染色分析。结果：肿瘤呈侵袭性生长,有明显的骨质吸收、破坏。病理组织学检查主要由梭形细胞和大量炎症细胞构成。免疫组织化学显示波形蛋白、平滑肌特异性肌动蛋白以及CD68均为阳性,S-100、CK均为阴性。结论：颞骨炎性肌纤维母细胞瘤是一种少见的呈侵袭性生长的肿瘤,容易局部复发。治疗以手术为主,辅以激素治疗。     

Differential diagnosis of proptosis: Report of 2 cases

We rarely encounter patients with proptosis, however, it is known that inflammation and extension of paranasal sinus tumors may cause this condition. We should consider the diseases that may cause proptosis to facilitate the diagnosis. Recently, we treated proptosis in 2 cases, one with inflammatory myofibroblastic tumor (IMT) and the other with malignant lymphoma. As these 2 diseases show various clinical features and these features frequently overlap, it is difficult to differentiate between IMT and malignant lymphoma without pathological examinations. In this study, we review the relevant literature reports and discuss the difficulties in establishing a clinicopathological diagnosis and present a slight distinction in magnetic resonance imaging.     

上颌窦炎性肌纤维母细胞瘤1例并文献复习

目的:探讨炎性肌纤维母细胞瘤的临床特征、诊断与治疗方法。方法:报告1例上颌窦炎性肌纤维母细胞瘤,并复习相关的国内外的文献。结果:炎性肌纤维母细胞中,CT扫描示不规则的软组织密度影,有侵蚀性的骨质破坏,边界不清。病理示瘤组织由梭形纤维细胞及大量炎性细胞组成。免疫组织化学检测中Vimentin、SMA呈阳性,Desmin等呈阴性。结论:上颌窦的炎性肌纤维母细胞瘤是非常罕见的,诊断主要依靠病理及免疫组织化学检测。炎性肌纤维母细胞瘤中存在染色体的易位,这种易位导致间变性淋巴瘤激酶的过度表达,而呈现一种肿瘤的发展过程,其生物学特性仍然没有统一的说法。     

A case of an inflammatory myofibroblastic tumor of the mastoid presenting with chronic suppurative otitis media

Inflammatory myofibroblastic tumors, also known as inflammatory pseudotumors, include a diverse group of lesions characterized by inflammatory cell infiltration and variable fibrotic responses. Their occurrence in the temporal bone is unusual. We present a case of an inflammatory myofibroblastic tumor of the mastoid bone in a 55-year-old female who complained of repeated otorrhea and a hearing impairment. On an otoscopic exam, her tympanic membrane was perforated. The computed tomographic imaging revealed a soft-tissue density-filled middle ear and mastoid with sclerotic bony change. The initial clinical diagnosis was chronic suppurative otitis media and mastoiditis, and the patient underwent a canal-wall down tympanomastoidectomy through a retroauricular approach. A histopathologic examination showed the presence of myofibroblastic spindle cells with mixed inflammatory infiltrates, and the pathologic diagnosis was inflammatory myofibroblastic tumor.     

4例喉炎性肌纤维母细胞瘤临床表现与组织病理学特点分析

目的分析4例喉炎性肌纤维母细胞瘤（IMT）患者的临床表现及组织病理学特点、并对患者进行术后随访,为IMT临床诊治提供经验。方法回顾性总结2007年10月—2019年6月复旦大学附属眼耳鼻咽喉科医院诊治的4例喉IMT患者的临床资料,对其临床表现、组织病理学特点及其治疗方法和预后进行统计学分析。结果4例喉IMT患者病变位于声门上及声门区,根据患者的病变范围及病情严重程度,选择显微喉镜手术或开放性手术,术后随访25~140个月,除1例患者死于其他疾病外,其余患者均恢复良好且未见复发和转移。术后切除标本均行组织病理学检查,免疫组织化学结果显示：Vimentin（+）,SMA（部分+）,Desmin（-）,CK（-）,Ki67（+）≤45%,ALK（-）,CD34（血管+）,CD68(±)。结论喉IMT是一种具有复发潜能的低度恶性头颈部肿瘤,其诊断依赖于组织病理学免疫组织化学检查。目前的治疗手段仍首选手术切除为主,必要时行术后放（化）疗,术后需密切随访。     

Low‐cost dual‐action aural foreign‐body extractor

Adequate visualization, appropriate equipment, a cooperative patient, and a skilled physician are keys to successful foreign body removal. The first attempt at removal is critical because success rates markedly decrease after the first failed attempt. Laryngoscope, 119:351–354, 2009     

An unusual presentation of a rare soft tissue tumor in the tongue: Inflammatory myofibroblastic tumor

Inflammatory myofibroblastic tumor (IMT) is a rare soft tissue neoplasm of intermediate biologic potential. Here, we describe an unusual presentation of IMT in the oral tongue of a pediatric patient. In contrast to other presentations in the literature suggestive of a malignant process, the presentation seen here appears benign. However, unlike the more common benign pediatric tongue lesions, IMT does have aggressive and even mortal potential. Though rare, it is important to keep in mind atypical presentations like the one described here and consider IMT on the differential diagnosis of a tongue lesion in a pediatric patient.     

Inflammatory myofibroblastic tumor of the parotid gland: case report and review of the literature

An inflammatory myofibroblastic tumor, previously known as an inflammatory pseudotumor, is an uncommon neoplasm. This tumor, which has characteristic morphological and immunohistochemical features, is mostly seen in the lung. Herein we present a rare case of an inflammatory myofibroblastic pseudotumor of the parotid gland as well as a review of the literature. The patient was a 66-year-old man with recurrent painful swelling of the parotid gland. A total parotidectomy with preservation of the facial nerve branches was performed. The patient showed no signs of recurrence>3 years after surgery. The presence of clonal cytogenic abnormalities supported the neoplastic origin of this process. The treatment consisted of complete resection. Clinicians should however be aware that an inflammatory myofibroblastic tumor may mimic a reactive process.     

鼻及咽部树突细胞肿瘤4例临床分析并文献复习

目的：探讨树突细胞肿瘤的临床表现、病理特点、治疗和预后。方法：4例鼻及咽部树突细胞肿瘤患者中，滤泡状树突细胞肉瘤（FDCS）2例，朗格汉斯组织细胞增多症（LCH）1例，朗格汉斯细胞肉瘤（LCS）1例。2例FDCS中有1例行综合治疗（手术加放化疗），1例仅行放化疗；1例LCH及1例LCS患儿均行手术治疗。结果：2例FDCS患者，l例术后行4个疗程CHOP方案及同步放疗（50Gy）后达完全缓解，1例仅行放化疗者随访7个月仍带瘤生存；1例LCH患儿随访2年余，肿瘤无复发及转移；1例LCS患儿，术后未行放化疗，随访10个月后死亡。结论：树突细胞肿瘤罕见，临床易误诊，确诊需病理组织形态、免疫组织化学及电镜相结合，FDCS、LCH及LCS具有独特的病理表现、免疫表型、诊治及预后。