口腔颌面-头颈部胚胎型横纹肌肉瘤临床诊治探讨

目的:探讨头颈部胚胎型横纹肌肉瘤的临床特点、诊断、治疗及预后.方法:回顾性分析上海交通大学医学院附属第九人民医院2000-09--2010-10收治的12例头颈部胚胎型横纹肌肉瘤患者的临床资料.结果:12例患者中9例获得随访.5例术前化疗患者中3例短期疗效达到PR,1例达到MR,化疗有效率达到60%;8例患者行手术治疗...     

头颈部横纹肌肉瘤11例临床病理学分析

目的：探讨头颈部横纹肌肉瘤（rhabdomyosarcoma,RMS）的临床病理学特征、免疫表型及鉴别诊断。方法：回顾性分析11例头颈部 RMS 的临床资料、病理形态和免疫组织化学标记结果。结果：11例患者中男性8例,女性3例。年龄2～51岁,中位年龄25岁。主要表现为头颈部痛性或无痛性肿块,组织学分型：胚胎型8例,腺泡型3例。免疫组织化学结果显示瘤细胞均表达结蛋白、波形蛋白;肌调节蛋白（MyoD1）、生肌蛋白（myogenin）的阳性率分别为45％、55％;2例表达突触素,所有病例均不表达广谱角蛋白、白细胞共同抗原及 S-100。术后随访6个月～5年,其中2例死亡,4例复发,2例转移。结论：头颈部横纹肌肉瘤罕见,临床症状不典型,病理诊断需结合组织学形态及免疫表型。     

口腔颌面部横纹肌肉瘤及综合治疗

目的　探讨口腔颌面部横纹肌肉瘤的诊疗方法。方法　选择1981~2001年在四川大学华西口腔医院颌面外科治疗的横纹肌肉瘤患者共74例,按WHO(1972)的横纹肌肉瘤TNM分期和国际横纹肌肉瘤协作组(1988)对该瘤的临床分期进行分期,探讨横纹肌肉瘤病理分型,临床分期及治疗方法与预后的关系。结果　74例患者中,有 34例行扩大切除术,18例行局部切除术,术后所有患者均接受了不同剂量的放疗,另有22例患者因病变范围广或是儿童而只接受了放疗或者放、化疗联合治疗。随访病例的5年生存率随病期和治疗方案的不同而有显著差异, 肿瘤复发和手术切除边缘及临床分期明显相关。结论　采用扩大切除术加术后放疗对提高本病的治愈率,减少复发有明显效果;晚期或儿童患者采用局部切除联合放、化疗对于提高5年生存率有良好效果。     

头颈部软骨肉瘤16例临床分析

目的探讨头颈部软骨肉瘤的临床表现、病理特点、鉴别诊断、治疗及预后。方法回顾分析我院自1997～2009收治的16例头颈部软骨肉瘤的临床资料,并对其进行随访。结果头颈部软骨肉瘤好发于鼻腔鼻窦、颞骨、下颌骨、上颌骨,以鼻腔鼻窦最常见,临床主要表现为牙齿麻木、变色、鼻部症状、面瘫、听力改变、张口受限等。头颈部软骨肉瘤以原发性中央型软骨肉瘤为主,诊断主要靠病理,治疗以手术根治性切除为主,随访显示头颈部软骨肉瘤主要死亡原因为肿瘤侵入颅内。结论头颈部软骨肉瘤是一种以局部侵袭为主的恶性肿瘤,应强调首次行根治性手术,并辅以增敏放疗。     

头颈部滑膜肉瘤13例临床分析

目的 分析和总结头颈部滑膜肉瘤的组织来源、临床表现和病理特点.方法 回顾分析我院1993～2015收治的13例头颈部滑膜肉瘤的临床表现、影像学表现和病理特点,总结其临床特点并对其误诊原因进行分析.结果 本组患者中2例病理活检得到明确诊断.其余病例分别误诊为纤维肉瘤2例,恶性外周神经鞘瘤1例,血管肉瘤l例,下颌骨骨髓炎1例,下咽癌腮腺转移1例,颈部囊肿1例.12例行手术治疗,其中4例术后联合放疗,3例联合化疗.术后易复发,随访4个月～2年,共5例复发(其中3例死亡,2例仍生存),1例死于肿瘤肺转移,1例死于咽部鳞癌,失访3例,未复发3例.结论 头颈部滑膜肉瘤是一种恶性程度较高,易发生复发及远处转移,预后较差的软组织恶性肿瘤,临床上易误诊,应加强对其临床特点和分子遗传学检测的认识,提高早期诊断和治疗效果.     

上唇横纹肌肉瘤1例及文献复习

报道近期收治的上唇横纹肌肉瘤1例,并结合复习国内外的相关文献,分析横纹肌肉瘤的临床特点、诊断及治疗。横纹肌肉瘤是一种高度恶性软组织肿瘤,临床表现多样,缺乏特征性,诊断较为困难,主要依靠病理检查,目前主要采用手术辅以放化疗的综合治疗。临床中需要提高对横纹肌肉瘤的认识和警惕性,早期诊断,制定个体化综合治疗方案,有效改善患者预后、提高生存率。     

15例头颈部梭形细胞/硬化性横纹肌肉瘤的临床病理分析

目的:探讨头颈部梭形细胞/硬化性横纹肌肉瘤（spindle cell/sclerosing rhabdomyosarcoma,SRMS/ScRMS）的临床病理学特征、免疫表型及预后。方法:回顾性分析15例头颈部SRMS/ScRMS患者临床、病理资料以及免疫组织化学检测结果。结果:15例患者（10例SRMS,5例ScRMS）中,男7例,女8例;年龄2个月~57岁,平均20.1岁。颞下窝5例,上颌骨软组织2例,舌2例,其余分别发生于咀嚼肌区、下颌软组织、翼颌间隙、腮腺区、颈部、唇部各1例。临床均表现为逐渐增大的肿块。肿块直径约1.5~20 cm,平均6.3 cm。组织学上,10例SRMS 主要由具有轻度非典型性的梭形细胞组成,呈交叉束状排列,散在于梭形细胞之间,可见少量横纹肌母细胞。5例ScRMS均以大量玻璃样变的硬化性基质为特征,类似原始的小圆细胞组成,以条索状、列兵样、梁状、微腺泡状和假血管样等多种方式排列。免疫组织化学标记显示,肿瘤细胞均弥漫表达vimentin和desmin,MyoD1呈弥漫及多灶性表达,12例表达myogenin,均不表达S100、HMB-45、Caldesmon、CK和CD34。15例患者均采用手术治疗,其中3例术后辅以化疗和/或放疗。随访4~205个月,平均59个月。5例无病生存,9例死亡,1例失访。结论:头颈部SRMS/ScRMS好发于儿童和青少年,且预后较差。熟悉其形态特征和免疫表型,有助于识别这类少见的横纹肌肉瘤亚型,并与其他梭形细胞及以间质硬化为主的肿瘤相鉴别。     

口腔颌面部横纹肌肉瘤：附10例临床病理报告

本文收集了１０例在我院诊治的口腔颌面部横纹肌肉瘤。根据ＷＨＯ关于横纹肌肉瘤病理组织诊断标准及其亚型分类标准进行观察，结合文献复习，着重探讨口腔颌面部横纹肌肉瘤的临床病理特点。本瘤在临床方面缺乏特异性表现，难以做出准确诊断，必须借助于组织病理学检查，常规切片染色诊断因难时，利用免疫组织化学染色法有助于明确诊断。     

儿童头颈部胚胎性横纹肌肉瘤

头颈部是儿童横纹肌肉瘤的好发部位,作者于1960—1975年治疗19例分化程度不同的头颈部横纹肌肉瘤患儿,年龄为13个月至17岁,平均6.7岁,受累最多的部位为眼眶9例和面部软组织5例,中耳3例,鼻咽部和喉部各1例。本组患儿处理以放疗为主,7例作手术,其中4例因复发行放疗;其余3例未能切净胚胎性横纹肌,术后立即进行放疗。除1例鼻咽部肉瘤患儿因在其它医院作过放疗,只作姑息性放疗外,余18例对原发肿瘤行根治性照射。大多数患儿照射剂量     

头颈部侵袭性纤维瘤9例及文献复习

目的:探讨头颈部侵袭性纤维瘤的临床表现、病理特征、治疗方式及预后.方法:根据2005年世界卫生组织的肿瘤类指导原则,回顾性分析2008 ～ 2012年间收治9例头颈部侵袭性纤维瘤的临床资料并复习相关文献.结果:发病部位以颈部(3例)、下颌下区(2例)最为常见,其次是上颌骨、颧部、眶下区、腮腺各1例,对9例患者都实施手术切除,4例患者因肿瘤已侵犯头颈部重要结构,无法获得阴性切缘,其中1例患者发生恶变,接受放射治疗,另2例术后未接受综合治疗,术后复发,所有病例无远处转移,随访时间为6 ～51个月.结论:头颈部侵袭性纤维瘤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病例可配合放化疗,且术后长时间密切随访.     

Postoperative adjuvant therapy for patients with loco-regionally advanced oral squamous cell carcinoma who are at high risk of recurrence

Extranodal extension (ENE) of lymph node metastasis and the presence of a positive or close margin (PCM) are major risk factors for head and neck squamous cell carcinoma recurrence. This retrospective multicentre cohort study compared the prognostic impact of postoperative radiotherapy (RT) and concurrent chemoradiotherapy (CCRT) in oral squamous cell carcinoma (OSCC) patients at high risk of recurrence. One hundred and eighteen patients with PCM and/or ENE who underwent definitive surgery plus either adjuvant RT or CCRT using cisplatin for OSCC were investigated. The cohort-wide 5-year loco-regional control (LRC), disease-free survival (DFS), and overall survival (OS) rates (the main outcome measures) were 54.3%, 35.8%, and 43.2%, respectively. Multivariate analysis showed that age ≥64 years (hazard ratio (HR) 0.584), cT3–4 stage (HR 1.927), ≥4 metastatic lymph nodes (HR 1.912), and PCM (HR 2.014) were significant independent predictors of OS. Moreover, postoperative CCRT with cisplatin was associated with a significantly improved LRC rate, but not with improved DFS or OS rates, compared to postoperative RT (HR 0.360). Given that CCRT with cisplatin does not significantly improve survival, additional clinical trials will be required to validate new regimens that further improve the outcomes of patients with loco-regionally advanced OSCC going forward.     

头颈部肿瘤调强放疗联合手术和化疗的回顾性分析

目的:回顾性分析头颈部肿瘤调强放疗(IMRT)的长期随访结果和影响预后的因素。方法:136例初治头颈部肿瘤接受全程IMRT,48例(35.3%)联合化疗。手术联合放疗平均剂量62.14 Gy(50～73 Gy),根治性放疗平均剂量68.99 Gy(56.4～77 Gy)。结果:中位随访时间19个月,3年局控率、区域控制率、无远处转移生存率、无瘤生存率和总生存率分别为83.2%、93.2%、75.8%、66.4%、67.6%。多因素分析显示临床分期、治疗模式、放疗前有无贫血是影响患者生存率的独立预后因素。急性0、1、2、3级皮肤反应发生率分别为3.7%,71.3%,24.3%,0.7%;0、1、2、3、4级黏膜反应发生率分别为4.4%,22.8%,57.4%,14.7%,0.7%。治疗结束1年后1、2、3度口干发生率分别为21.3%、16.2%、0.7%。结论:IMRT联合手术和化疗治疗头颈部肿瘤可取得较好疗效,不良反应可以耐受。临床分期、治疗模式、放疗前有无贫血是影响生存的主要因素。     

新辅助化疗治疗头颈部骨肉瘤单中心临床效果回顾

目的 评价新辅助化疗在头颈部骨肉瘤治疗中的效果。方法 回顾2007—2015年于上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科接受治疗的头颈部骨肉瘤患者的基本信息、围术期治疗信息、影像学信息、病理学信息以及随访记录,采用SPSS 17.0软件包对数据进行统计学分析。结果 最终纳入患者157例,男68例,女89例,5年总生存率（OS）为50.96%。≤30岁51例,31~60岁85例,≥61岁21例。91例肿瘤位于下颌骨,66例位于上颌骨及颅底。高级别骨肉瘤141例,低级别骨肉瘤8例,分化程度未知8例。54例肿瘤最大直径>4 cm,103例肿瘤直径≤4 cm。术区切缘阳性7例。单纯手术治疗65例,手术联合化疗15例,手术联合放、化疗23例,手术及放疗54例。单因素分析显示,肿瘤远处转移的相关因素为上颌骨及颅底肿瘤（P=0.022）、高级别骨肉瘤（P=0.011）以及阳性切缘（P=0.031）;下颌骨骨肉瘤（P=0.032）、阴性切缘（P=0.006）与患者OS相关;阴性切缘（P=0.007）与患者无瘤生存率（DFS）相关。多因素分析显示,肿瘤部位（P=0.034）、切缘（P=0.006）、化疗（P=0.025）、病理学亚型（P=0.012）与患者总体生存率相关;阳性切缘（P=0.002）、未接受放疗（P=0.005）与肿瘤局部复发相关。结论 术区安全切缘是头颈部骨肉瘤良好预后的关键,放疗在一定程度上可以抑制肿瘤复发,化疗（新辅助化疗、辅助化疗）可提高患者术后总体生存状况。     

Tumor–stroma ratio is a crucial histological predictor of occult cervical lymph node metastasis and survival in early-stage (cT1/2N0) oral squamous cell carcinoma

Occult cervical lymph node metastasis is a significant prognostic factor in patients with early-stage (cT1/2N0) oral squamous cell carcinoma (OSCC). The aim of this study was to investigate the potential value of the tumor–stroma ratio (TSR) as a histological predictor of occult cervical metastasis and survival in early-stage OSCC. This retrospective study included 151 patients who underwent excision of the primary lesion and elective neck dissection from 2013 to 2017. The clinicopathological features of the tumor, risk factors associated with occult neck metastasis, and prognostic factors for overall survival (OS) and disease-free survival (DFS) were studied. A significant correlation of TSR (P = 0.009) was found with occult neck metastasis in the multivariate logistic regression model. Multivariate Cox proportional hazards regression analysis showed that the TSR (P = 0.002) and perineural invasion (P = 0.011) were associated with OS. Occult neck metastasis (P = 0.032) was associated with DFS. These findings indicate that assessment of the TSR might be useful in prognostication for early-stage OSCC patients. Moreover, the TSR is effective in allowing an accurate evaluation of the risk of occult neck metastasis, and this may be easily applicable in the routine pathological diagnosis and clinical decision-making for elective neck dissection.     

Survival in patients under 45 years with squamous cell carcinoma of the oral tongue

Squamous cell cancer among young adults is rare and thought to have aggressive biological behaviour and poor prognosis. Clinical case records of 76 patients under the age of 45 years treated for squamous cell carcinoma (SCC) of the oral tongue were retrospectively analysed to calculate the survival rates and the predictors of survival. The overall survival (OS) and disease free survival (DFS) at 5 year were 78.0% and 57.4% respectively. Tumour status, pathological node status, surgical clearance, the selection of appropriate treatment, type of primary and neck surgery, were found to significantly influence the survival rates. Young patients with SCC of the oral tongue were seen to have a comparable outcome and prognosis compared to that in older patients.     

Clinical course and survival in patients with squamous cell carcinoma of the maxillary alveolus and hard palate: Results from a single-center prospective cohort

ObjectivesOperative management of squamous cell carcinoma of the maxillary alveolus and hard palate (MHSCC) is still a controversial issue, especially for cN0 cases. We report the survival outcomes in patients with MHSCC, including the rate of cervical occult metastasis for UICC stages I/II and survival after elective neck dissection (END).Methods77 patients with primary MHSCC were followed-up from 2 months to 14.6 years within a prospectively obtained patient cohort. Factors influencing overall survival (OS), oral cancer-specific survival (OCSS) and disease-free survival (DFS) were assessed. We estimated survival probabilities using Kaplan–Meier survival analysis by histology and stage. We used Cox's proportional hazard regression model to estimate adjusted hazard ratios for OS, OCSS and DFS.ResultsInitially, 22 patients presented with stage I, 13 with stage 2, 11 patients with stage 3 and 31 with stage 4 tumors. The presence of nodal disease rose with increased tumor size. 66.2% were treated with surgery only, 26.0% received adjuvant radiotherapy, and 7.8% received primary radiotherapy. Median overall survival was 10.9 years, and survival probabilities at 2, 5 and 10 years were 68.2%, 61.1% and 61.1%, respectively. Five-year overall survival was 72.4% in the END group and 88.9% in the non-END group. Factors influencing OS, OCSS and DFS were tumor size, the presence of metastatic disease and positive resection margins.ConclusionsMHSCC seems to show a better overall survival compared to OSCC of other locations and is less likely to develop regional and distant metastasis; END might not be necessary in early stage tumors.     

Prognostic factors in head and neck mucoepidermoid carcinoma: experience at a single institution based on 64 consecutive patients over a 28-year period

Mucoepidermoid carcinoma (MEC) is the most common malignancy of the salivary glands. The clinical behaviour of MEC is largely unpredictable, ranging from indolent tumour growth to highly aggressive metastatic spread. The objective of this study was to determine the clinicopathological predictors of recurrence and survival in patients with head and neck MEC. The medical records of 64 patients who underwent surgical treatment for head and neck MEC between 1982 and 2010 were reviewed. The main outcome measures were disease-free survival (DFS) and overall survival (OS). Clinicopathological parameters evaluated were age, sex, anatomical subsite, histological grade, tumour stage, tumour size, adjuvant therapy, and nodal and margin status. For the entire cohort, the 5-year DFS was 82.8% and the 5-year OS was 67.2%. Histological grade and tumour subsite were statistically significant predictors of OS. Furthermore, tumour stage and nodal status were statistically significant predictors with respect to OS. Advanced tumour stage, high histological grade, submandibular/sublingual localization, and positive nodal status were independent predictors of the prognosis in patients with head and neck MEC. Further studies into the molecular biology of MEC are needed in order to provide new therapeutic strategies for patients with locally aggressive and highly metastatic carcinomas.     

Prognostic significance of lymph node ratio in patients with squamous cell carcinoma of the floor of the mouth

The lymph node ratio (LNR) has been proposed as an independent prognostic factor for survival in patients with oral squamous cell carcinoma (OSCC). However, little attention has been paid to its role in the specific subsite of the floor of the mouth (FOM). The purpose of this study was to evaluate the prognostic significance of the LNR in patients with FOM SCC. A retrospective analysis of 92 patients with FOM SCC who were treated with primary curative resection and neck dissection was conducted. Overall survival (OS) and disease-free survival (DFS) were used to evaluate the prognostic significance of the LNR. Both of these parameters were significantly worse (P < 0.001) in patients with neck metastases. The mean LNR was 0.145 in patients with positive lymph nodes. A LNR <0.145 was predictive of longer DFS, while the receiver operating characteristic curve analysis demonstrated that a LNR ≥0.175 indicated a significantly lower OS. This study confirms that metastatic cervical lymph nodes correlate with an adverse prognosis in patients with FOM SCC, and specifically, a LNR ≥0.145 is predictive. Therefore, the LNR in patients with FOM SCC may be a predictor of survival in these patients.     

Oral and oropharyngeal cancer in the West of Scotland—long-term outcome data of a prospective audit 1999-2001

We prospectively studied patients from the west of Scotland who presented with a primary cancer of the oral cavity or oropharynx over a period of 24 months from November 1999, and report long-term outcomes and prognostic factors. A total of 481 patients had squamous cell carcinoma (SCC), 5-year disease-specific survival (DSS) was 50%, and overall survival (OS) was 35%. One hundred were not suitable for treatment with curative intent, and factors other than stage were important in this decision. Of those treated with curative intent, 249 had SCC of the oral cavity (5-year DSS 67%; OS 42%), and 132 had SCC of the oropharynx (5-year DSS 62%; OS 42%). Multivariate analysis showed that pathological nodal stage (p = 0.051, 95% CI 0.998-1.955), and perineural invasion (p = 0.001, 95% CI 0.186-0.666) were prognostic indicators. Improved results using intensive treatment protocols that have been seen in trials are not likely to translate directly into a general population of patients with head and neck cancer. Algorithms that allow several pathological prognostic indicators to be incorporated into decisions about adjuvant treatment should be used.     

口腔颌面-头颈部黏膜相关淋巴组织边缘区淋巴瘤临床及预后分析

目的：探讨口腔颌面-头颈部黏膜相关淋巴组织边缘区淋巴瘤（MALT淋巴瘤）的临床特征、分期、治疗方法及预后,为临床治疗提供更强的循证医学证据。方法：回顾分析上海交通大学医学院附属第九人民医院口腔颌面-头颈肿瘤科收治的105例原发于口腔颌面-头颈部的MALT淋巴瘤患者的临床资料,采用SPSS 22.0软件包对其预后进行分析。结果：105例MALT淋巴瘤患者中,男女比例为1∶2.5,中位年龄56岁（18~86岁）。主要临床表现为单纯渐增性肿块,52%的患者有长期口干症状或曾被诊断为自身免疫性疾病或慢性腮腺炎。根据Ann Arbor分期,Ⅰ期48例,Ⅱ期25例,Ⅲ期7例,Ⅳ期25例;有B症状者仅1例,无骨髓受侵的患者。最常见的部位为腮腺、腭、下颌下腺。50例接受单一治疗,49例接受联合治疗。中位随访时间60个月,8例失访,初治后完全缓解率为80%;疾病进展12例,死亡14例。5年总生存期和无进展生存期分别为89%、88%;全身治疗（化疗或利妥昔单抗）可显著提高晚期患者的无进展生存期（P<0.05）,MALT淋巴瘤的国际预后指数（MALT-IPI）是影响总生存期的独立预后因素,Ann Arbor分期是影响无进展生存期的独立预后因素。结论：口腔颌面-头颈部MALT淋巴瘤初治疗效良好,进展缓慢;晚期患者应辅助全身治疗。MALT-IPI和Ann Arbor分期是影响预后的独立因素。