12例头颈部侵袭性纤维瘤病临床分析

目的:探讨头颈部侵袭性纤维瘤病临床特点及治疗方法。方法:回顾性分析12例头颈部侵袭性纤维瘤病患者的临床资料。11例行局部广泛切除,另1例行姑息性切除术联合术后放疗。结果:术后6例复发,其中1例复发2次,5例复发1次。所有患者随访至今无一例死亡。结论:头颈部侵袭性纤维瘤病发病率较低,症状无特异性,主要依靠术后病理确诊;治疗上以手术切除为主,对于复发或难以彻底切除的患者,可配合放疗和化疗;此病有高度复发倾向,但预后较好。     

头颈部侵袭性纤维瘤病2例及文献复习

目的:探讨头颈部侵袭性纤维瘤病的临床表现、病理特征、治疗方式及预后,为临床医生正确诊治及降低该病复发率提供帮助。方法:回顾性分析2例头颈部侵袭性纤维瘤病的临床资料并复习相关文献。结果:侵袭性纤维瘤病以浸润性生长、局部易复发及无远处转移为特点。病理学表现为成纤维细胞单克隆增生,由分化良好的成纤维细胞和肌成纤维细胞组成,无恶性细胞学特征和有丝分裂相。本组患者中1例第1次手术后复发,再次手术后随访6个月无复发;另1例手术后随访6个月未复发。结论:头颈部侵袭性纤维瘤病症状无特异性,诊断主要依靠术后病理,彻底手术切除是治疗并减少复发的主要手段。对于复发或难以完整切除的患者,可配合放化疗。     

手术联合放疗治疗头颈部侵袭性纤维瘤病12例报道及文献复习

目的探讨头颈部侵袭性纤维瘤病最佳的治疗方法。方法对12例头颈部侵袭性纤维瘤病进行手术治疗,其中7例术后辅助放射治疗。结果所有患者术后随访5～15年,7例行肿瘤切除加术后放疗者无复发,5例行单纯肿瘤广泛切除者术后7～18个月复发;总复发率为41.7%。术中切缘行病理检查,切缘阴性者复发率为14.3%。结论术中确定阴性切缘可以降低头颈部侵袭性纤维瘤病复发率,但头颈部病变多难以获得阴性切缘,局部切除后易复发,术后放疗能降低复发率。     

头颈部侵袭性纤维瘤病(3例报告及文献复习)

目的：探讨一种头颈部少见肿瘤--侵袭性纤维瘤病的临床表现、病理特点、治疗选择及转归。方法：报告3例头颈部侵袭性纤维瘤病的临床资料并复习相关文献。结果：①侵袭性纤维瘤病是一种纤维增生性良性或低度恶性肿瘤生物学呈局部浸润性和破坏性生长；②病理学表现为细胞中心和胶原周围成纤维细胞单克隆增生，由分化良好的成纤维细胞和肌成纤维细胞组成，缺乏恶性的细胞学特征和有丝分裂相，电镜示丰富的胶原网包绕多形态细胞增生，免疫组化波形蛋白和肌动蛋白阳性；③手术是主要治疗措施，多数病例需多次手术，术后复发率可高达70％，常需配合放射治疗和化学治疗；④1例接受3次手术仍复发，另2例手术后随访3-6个月未复发。结论：侵袭性纤维瘤病是一种少见的纤维增生性肿瘤，头颈部少见；以手术治疗为主，有高度复发倾向，但预后较好；对于复发或难以完整切除的病例，常需配合放射治疗和化学治疗。     

鼻腔鼻窦骨化纤维瘤9例

目的：探讨提高鼻腔鼻窦骨化纤维瘤诊治水平的途径。方法：回顾性分析9例鼻腔鼻窦骨化纤维瘤患者的临床资料。根据病变范围不同，其中3例选择上颌窦进路，将鼻腔上颌窦肿块完整切除；6例在全身麻醉下行颈侧切开术。结果：2例因手术切除不彻底，分别于术后8个月和9个月复发而再次手术，其余7例随访2～10年无复发，无并发症发生。结论：鼻腔鼻窦骨化纤维瘤是一种以青少年发病为主，具有局部侵袭性的良性肿瘤，临床症状、特别是CT扫描是临床诊断的基础，彻底切除肿瘤是惟一有效的治疗方法。     

蝶窦良性侵袭性病变的临床诊治分析

目的　探讨蝶窦良性侵袭性病变的临床特点及内镜治疗。方法　回顾性分析19例蝶窦良性侵袭性病变的临床资料。19例均行内镜下经蝶入路病变切除。结果　内 翻性乳头状瘤6例,骨化纤维瘤7例,软骨瘤2例,嗜酸性肉芽肿4例。临床症状以头痛及视觉障碍多见。影像学共同特征是蝶窦骨壁破坏,周围结构受不同程度侵袭。术中发生脑脊液鼻漏1例,I期修复成功。术后全部患者无眶内及颅内并发症。随访3～5年,手术全切除15例无复发;次全或大部分切除4例,1例病灶无增大;3例复发,其中1例再次手术治愈,2例恶变并颅内转移死亡。结论　蝶窦良性侵袭性病变呈恶性肿瘤样行为,具有侵袭性、易复发性。内镜下彻底切除病变是防止复发的重要治疗方法。但有别于恶性肿瘤,应注意避免扩大切除范围。     

颞骨骨化纤维瘤

目的：探讨颞骨骨化纤维瘤的诊断和治疗，提高对该病的认识。方法：报道1例颞骨骨化纤维瘤男性患儿，取耳后沟外切口，暴露瘤体，彻底切除肿瘤组织，封闭外耳道残端，取自体腹壁脂肪填塞术腔；术后定期随诊。结果：术后病理证实为颞骨骨化纤维瘤，术后随访1年无复发，外耳道内端封闭完好。结论：颞骨骨化纤维瘤临床罕见，虽为良性肿瘤，但可压迫和侵袭邻近重要组织和器官，引起功能障碍和颅内感染,一旦确诊，应尽早完整切除。     

儿童颅底肿瘤经鼻内镜外科手术治疗分析

摘要：目的探讨分析鼻内镜手术在治疗儿童颅底肿瘤中的应用。 方法回顾性分析中南大学湘雅医院鼻颅底外科 2010年6月—2019年11月收治确诊为颅底肿瘤并行鼻内镜治疗的患儿共44例,其中鼻咽纤维血管瘤17例,朗格汉斯组织细胞增生症7例,骨化纤维瘤5例,原始神经外胚层肿瘤2例,横纹肌肉瘤2例,脑膜瘤2例,间叶性软骨错构瘤1例,颅咽管瘤1例,表皮样囊肿1例,血管纤维脂肪瘤1例,生殖细胞瘤1例,骨母细胞瘤1例,脊索瘤1例,侵袭性垂体腺瘤1例,纤维瘤病1例。 结果44例患儿中43例在鼻内镜下完全切除,仅1例鼻咽纤维血管瘤分期切除。术后44例均进行定期随访,随访时间3个月至9年。5例失访,其中骨母细胞瘤1例,脑膜瘤1例,横纹肌肉瘤1例,鼻咽纤维血管瘤2例;12例术后接受放化疗,其中朗格汉斯组织细胞增生症7例,原始神经外胚层肿瘤 2例,横纹肌肉瘤1例,脊索瘤1例,生殖细胞瘤1例,随访至今患儿状况良好,均无复发及转移;1例鼻咽纤维血管瘤复发;再次手术后治愈;其余26例患儿术后未见复发。结论鼻内镜颅底手术治疗儿童颅底肿瘤是可行、有效及安全的。     

儿童头颈部淋巴管畸形的外科治疗

目的 探讨儿童头颈部淋巴管畸形的外科治疗方法和疗效.方法 回顾性总结北京大学第一医院自1998年1月至2008年12月住院治疗的11例儿童短径大于4 cm的头颈部淋巴管畸形.11例儿童头颈部淋巴管畸形均行增强CT或MRI扫描检查,治疗均采用外科手术治疗,其中行肿物及腮腺浅叶切除4例,肿物及腮腺全切除2例,颈清扫术式切除肿物2例,颈清扫术式联合开胸术切除肿物1例,颌下肿物切除术2例.术中采用假包膜外剥离法及顺行解剖面神经主干法,肿物与面神经粘连紧密的2例行肿物剔除术.其中6例行外周面神经解剖,2例行面神经下颌缘支解剖.结果 11例儿童头颈部肿物均彻底切除,无器官功能异常,无明显外形变化,治愈率为100%.3例在行面神经下颌缘支解剖和肿物切除时牵拉下颌缘支出现轻度面瘫(下颌缘支力弱),1例颈部广泛肿物行颈清扫术后出现Homer综合征,均在神经营养治疗后术后1～3个月内恢复.1例1岁患儿行颈清扫术式联合开胸术切除肿物的术中输血150 ml.所有病例随访6～121个月,随访中位数32个月,未见肿物复发.结论 采用面神经解剖及(或)颈部解剖、肿物假包膜外剥离法等手术可以根治儿童头颈部大型淋巴管畸形,是保留功能、防止畸形的有效方法.     

手术治疗儿童腺样体肥大致反复发作的分泌性中耳炎

目的：探讨治疗儿童经手术切除肥大腺样体后致顽固性分泌性中耳炎的疗效。方法：对32例行腺样体切除后患儿进行听力、声阻抗、纯音测听方面的检查。结果：治愈28例,好转3例,无效1例,有效率96.9%。结论：切除腺样体可作为治疗儿童反复发作的分泌性中耳炎（otitis media with effusion OME）的手段之一。     

Six cases of fibromatosis of the head and neck in children

Six cases of aggressive fibromatosis occurring in the head and neck in children are described. Fibromatosis is a rare, benign but locally infiltrative condition. Primary excision is not always possible in the head and neck areas. It is not always appropriate to treat children using the same modalities as adults due to associated growth problems. The case histories illustrate the management difficulties that can be encountered when treating the paediatric population.     

Gastric scirrhous carcinoma as a rare cause of aggressive fibromatosis in the neck

Aggressive fibromatosis is characterized by locally aggressive proliferation of fibroblasts and occasionally occurs in the head and neck. Although the etiology of this disease is still controversial, recent studies have shown that high levels of growth factors are found in these lesions. This article reports a case of 51-year-old woman with aggressive fibromatosis in the neck and inguinal region bilaterally. Gastric fiberscopic examination revealed a complication of advanced gastric scirrhous carcinoma. There were remarkably high levels of basic fibroblast growth factor (bFGF) and platelet-derived growth factor (PDGF) in the serum. Postmortem autopsy examination was performed and scattered gastric carcinoma cells were observed in the fibromatosis lesions. Immunohistological staining showed positive expression of bFGF in the fibromatoses and stomach. We concluded that this was a rare case in which bFGF and PDGF released from gastric carcinoma cells caused aggressive fibromatosis by promoting unregulated proliferation of fibroblasts and collagen production locally.     

Aggressive fibromatosis of the parapharyngeal space: two cases and treatment recommendations

Aggressive fibromatosis is an uncommon tumor that is locally aggressive but not malignant. Therefore, its classification falls between the benign and malignant neoplasms of soft-tissue origin. In the past, these lesions were misdiagnosed as low-grade fibrosarcomas. Otolaryngologists should be familiar with these lesions because as many as 15% of them occur in the head and neck. The treatment of choice is wide surgical excision, which is often difficult. Postexcision recurrence rates are high. Nonsurgical treatment includes radiation and chemotherapy, both of which are usually reserved for recurrences. We describe two cases of aggressive fibromatosis of the parapharyngeal space, and we review the available treatment options.     

Aggressive fibromatosis of the neck treated with a combination of chemotherapy and indomethacin

Aggressive fibromatosis (desmoid tumor) of the neck is rare. When feasible, surgery is the best treatment option. However, complete excision with negative margins is not possible in most cases because of the involvement of vascular and nervous structures. Also, surgery results in poor functional and aesthetic outcomes. Sometimes debulking surgery with positive margins is performed, but the anatomy of the neck is a challenge for oncologic surgeons, and recurrences are not uncommon. Radiotherapy is seldom employed for the same reasons. On the other hand, systemic treatment with chemotherapy, hormone therapy, and noncytotoxic agents such as nonsteroidal anti-inflammatory drugs (NSAIDs) has been used with good results. We report a case of inoperable aggressive fibromatosis of the neck that was successfully treated for about 21 months with a combination of chemotherapy and the NSAID indomethacin. As far as we know, this is the first reported use of a combination of chemotherapy and an anti-inflammatory drug in the treatment of aggressive fibromatosis of the neck. We also review the literature on cases of aggressive fibromatosis of the neck that have been reported over the past 12 years.     

Aggressive fibromatosis of the head and neck (desmoid tumours)

Desmoid tumours are histologically benign fibrous neoplasms arising from the musculoaponeurotic structures throughout the body. They are characterized as infiltrative, usually well-differentiated firm overgrowth of fibrous tissue and are locally aggressive. An aggressive clinical behaviour with tendency for recurrence makes the treatment of these relatively rare fibrous tumours difficult. Local recurrence rates are reported as high as 70 per cent of cases, the recurrence of desmoid fibromatosis in the head and neck is difficult to ascertain because of the different classification schemes used by different authors. A review of cases of desmoid tumours of the head and neck presented in the last 10 years is reported in this paper. Complete surgical excision of desmoid tumours is considered to be the only effective method of cure by most authorities.     

Desmoid tumours of the head and neck: A case report

IntroductionDesmoid tumours of the head and neck, also known as fibromatosis, are rare, locally invasive benign tumours with high recurrence rate, causing considerable morbidity. Complete surgical excision of desmoid tumours is considered to be the only effective treatment.Case reportWe present a case of fibromatosis of the right posterolateral region of the neck in a 56-year-old woman who presented with right neck mass. The patient underwent complete excision of the tumour with no adjuvant therapy. No recurrence or neurological deficit was observed 2 years after surgery.ConclusionAlthough desmoid tumour is a benign neoplasm with no metastatic potential, treatment is challenging due to its aggressive, infiltrative behaviour with a tendency to recur.     

Synovial cell sarcoma: diagnosis,treatment, and outcomes

OBJECTIVES/HYPOTHESIS: Synovial cell sarcoma is a mesenchymal tumor predominantly of the lower extremities. Three percent of cases arise in the head and neck region. It is thought that head and neck synovial sarcoma has a better prognosis than tumors of the extremities. Our experience has demonstrated aggressive behavior of this neoplasm in the head and neck. This compelled us to compare our experience with other studies. STUDY DESIGN: Retrospective chart review. METHODS: We obtained the records of patients diagnosed with head and neck synovial sarcoma from the Tumor Registry of the University of Louisville School of Medicine (Louisville, KY) and affiliated hospitals for data compiled between January 1990 and December 2000. Data on patient demographics, clinical findings and symptoms, histological findings, treatment, extent of disease, recurrence, and survival were recorded. The literature was reviewed identifying reports of synovial cell sarcoma. RESULTS: Five consecutive patients with synovial cell sarcoma were assessed at our facility. The median patient age was 28.2 years. All of the patients underwent an aggressive primary surgical excision followed by irradiation. All patients received chemotherapy after recurrence. Four of the five patients had local recurrence, and all five of the patients developed distant metastases. Three of the patients have died, and two are alive with evidence of disease. Novel sites are reported including the ethmoid sinus and the parotid gland. This group demonstrated a 40% 5-year overall survival, which was lower than the 60% 5-year survival reported in the literature for all sites. CONCLUSIONS: Synovial cell sarcoma of the head and neck is a disease of young people and carries a poor prognosis. The aggressive nature of the disease may require modification of accepted treatment modalities and sequence.