鼻腔鼻窦非肠型腺癌6例临床分析

目的 以临床病例结合文献分析原发性鼻腔鼻窦非肠型腺癌（non-ITAC）的临床特性和病理免疫学特征,以期给临床医生在今后的诊疗中提供参考。方法 回顾性分析2014—2020年于北京协和医院经手术病理确诊的6例鼻腔鼻窦non-ITAC患者的临床资料和影像学、病理免疫学特点。结果 6例患者中男4例,女2例,年龄29~72岁,平均年龄54.8岁,其中4例为高级别non-ITAC,2例为低级别non-ITAC。临床表现以涕中或痰中带血、鼻塞等为主。4例男性均有吸烟史,其中1例工作中接触木屑较多。治疗方案为手术或术后加放疗或放/化疗。免疫组化结果：6例患者CK7均为阳性,其中4例患者P63为阴性;4例高级别non-ITAC患者Ki-67增殖指数为60%~90%,低级别non-ITAC患者Ki-67增殖指数1%~2%。随访至今,1例患者因肺转移死亡,其余患者均未有复发或转移。结论 鼻腔鼻窦non-ITAC患者临床表现及影像学对诊断有重要意义,病理免疫学对分级、治疗方案和预后有指导作用。     

鼻腔鼻窦低级别非肠型腺癌的临床特征分析

目的　分析鼻腔鼻窦低级别非肠型腺癌（nonintestinal adenocarcinoma,non-ITAC）的临床特点。方法  回顾性分析2014年2月～2020年2月住院的12例确诊鼻腔鼻窦低级别non-ITAC患者的临床资料,男6例,女6例,年龄28～79岁。分析其好发部位、主要症状、治疗方法、随访结果等特征。结果　好发单侧鼻腔鼻窦（12例）,症状以单侧鼻塞（12例）、涕血（4例）为主。所有患者均行手术,术后随访17～74个月,2年生存率为100%,2年复发率为37.5%。结论　鼻腔鼻窦低级别non-ITAC好发于鼻腔、筛窦和上颌 窦,确诊依靠病理,手术治疗复发率不高,不易发生转移,但复发会出现高级别恶变。彻底切除病变预后较好。术前若是考虑病变范围过大,手术不能彻底切除,可尝试行诱导化疗,术后辅助放疗。     

鼻腔低级别非肠型腺癌1例并文献复习

目的 探讨鼻腔低级别非肠型腺癌的临床特点及诊疗方法。方法 回顾性分析1例鼻腔低级别non-ITAC患者的临床资料,并复习相关文献,总结其临床表现、诊断、治疗方法及预后。结果 术后9个月随访,患者无鼻塞、脓涕及涕中带血等症状,蝶窦口及其下方可见肉芽组织。结合本例患者的临床资料,同时回顾既往中英文数据库关于鼻腔鼻窦低级别non-ITAC的报道,共计18例;患者CK7为阳性(94.4%)、CK20为阴性(61.1%)、CDX-2为阴性(61.1%),Ki-67增殖指数1%～15%;仅予以手术治疗(77.8%),手术后辅以放疗(16.6%),先放疗后予手术切除(5.6%);患者密切随访,时间为9个月至6年,平均随访时间为35.8个月,随访患者中有10例未见复发。结论 鼻腔鼻窦non-ITAC患者临床表现及影像学对诊断有重要意义,病理免疫学对分级、治疗方案和预后有指导作用。     

原发性鼻腔-鼻窦砂粒体型脑膜瘤1例报道并文献复习

目的 通过报道1例原发于鼻腔-鼻窦砂粒体型脑膜瘤患者的临床资料及相关文献复习，以提高临床医师对该病的认识及诊疗水平。方法 患者，女，50岁，因"渐进性右侧鼻塞1年余"入院。术前影像学检查提示右侧鼻腔及筛窦内不规则占位性病变，遂在全麻鼻内镜下行右侧鼻腔筛窦肿物切除术。结果 肿物完全切除，术后病理及免疫组化确诊为砂粒体型脑膜瘤（WHO I级）。患者鼻塞及头痛症状消失，术后2个月复诊见鼻腔术区黏膜上皮化完成，鼻腔无粘连，暂未见肿物复发。结论 原发于鼻腔-鼻窦的异位砂粒体型脑膜瘤少见，缺乏特异性临床表现，容易误诊。诊断主要依靠病理检查，治疗上应争取完全切除肿物及周边受侵犯组织，以达到根治，防止复发。     

晚期鼻腔鼻窦腺样囊性癌12例临床分析

目的总结原发于鼻腔鼻窦的晚期腺样囊性癌(ACC)的临床特征,探讨切缘病检结果以及复发或转移与预后的关系。方法回顾性分析12例晚期鼻腔鼻窦ACC患者的临床资料、治疗方法及预后。其中T3和T4期病变患者各6例,均无局部淋巴结及全身转移。首次治疗均以手术为主,9例联合术后放疗,4例联合化疗,复发及转移的治疗方式各异,仍以再手术为主。结果在20次首发及复发病灶的手术中,切缘阳性率高(75%,15/20),切缘阳性者生存时间最长达32年;随访期间局部复发7例(2例合并远处转移),单纯远处转移1例,局部复发并远处转移患者最长生存达32年。结论晚期鼻腔鼻窦ACC手术切缘阳性率高,局部复发率高,但局部复发或远处转移后仍能带瘤生存较长时间,在行根治性手术以及复发或转移治疗决策时应充分考虑患者的生存质量。     

儿童筛窦尤文氏肉瘤1例并文献复习

目的 探讨儿童鼻窦尤文肉瘤(ES)的临床特征、诊疗方法及预后。方法 回顾性分析1例筛窦ES的临床资料,并结合相关文献进行讨论。结果 该患者为原发性筛窦ES,术前诊断缺乏特异性临床表现和影像学特征。最终通过组织病理及免疫组织化学确诊。患者接受手术联合化疗、放疗的综合治疗,随访8个月无复发和转移。结论 儿童筛窦ES极罕见,临床表现无特异性,病理特征与鼻腔其他小圆细胞肿瘤相似,建议采用免疫标记物CD99+NKX2.2的方法诊断。鼻窦ES多采用综合治疗方案,预后较好。     

鼻腔鼻窦孤立性纤维性肿瘤1例报道及文献复习

目的 探讨发生于鼻腔鼻窦的孤立性纤维性肿瘤（SFT）的临床诊治经验。方法 结合1例鼻腔及鼻窦SFT患者的病例资料,并回顾性分析国内外文献,对SFT的临床特点、诊断与治疗进行讨论。本例患者术前CT提示：左侧鼻腔鼻窦肿物性质不明,鼻腔息肉可能。予以鼻腔新生物活检后鼻腔出血量大,随即全麻下行鼻内镜左侧鼻腔鼻窦新生物切除术,并给予止血、填塞等对症处理。结果 术后病理证实为鼻腔、鼻窦肿物符合SFT。患者恢复良好,术后半年复查未见复发。结论 SFT是一类好发于胸膜的间叶性肿瘤,但发生于鼻腔鼻窦的非常少见。影像学对于鼻腔鼻窦新生物的诊断只有辅助性意义,临床医生需要结合患者病史、临床表现、病理检查结果正确诊断与治疗。     

14例阿司匹林耐受不良三联征临床分析

目的 探讨阿司匹林耐受不良三联征(AIT)的临床特点,治疗方法,总结最新诊疗经验。方法 采用回顾性研究方法, 对14例AIT患者的用药特点、临床特点、鼻窦CT、鼻镜、鼻腔鼻窦手术史等结果及治疗情况进行统计分析,并进行相关文献复习。结果 14例患者中1例患者因哮喘急性发作,经术前评估后暂缓手术治疗;13例患者行手术治疗,术后6个月随访,其中11例未见复发,病情基本得到控制;2例患者鼻部症状复发,1例再次行手术治疗,另1例继续药物综合治疗。结论 AIT因其伴有哮喘及相关药物过敏情况,鼻窦炎症状较重,多为全组鼻窦炎,易复发,远期失嗅,早期临床多误诊。能够早期明确诊断,早期围术期规范用药,早期进行彻底清除病灶,并进行规律术后药物治疗是本病的治疗关键。     

鼻腔鼻窦窦组织细胞增生症伴巨大淋巴结病的临床病理特征分析

目的 探讨发生在鼻腔鼻窦的窦组织细胞增生症伴巨大淋巴结病(SHML)的临床、病理特征。方法 报道3例鼻腔鼻窦SHML患者的临床资料,总结分析其临床病理特征并进行相关文献复习。结果 发生在鼻腔鼻窦的SHML临床以鼻塞为主。病理组织学特点为在淋巴、浆细胞背景下,可见弥漫浸润的窦组织细胞,局部见“封入”现象。免疫组织化学染色示肿瘤细胞表达CD68、S-100。结论 SHML是一种组织细胞增生性病变,发生在鼻腔鼻窦的SHML非常罕见,临床表现缺乏特异性。病理学诊断为金标准,以手术治疗为主,但效果不佳。     

鼻腔鼻窦腺样囊性癌放疗后诱发未分化多形性肉瘤1例报道

目的 总结鼻腔鼻窦腺样囊性癌放疗后诱发未分化多形性肉瘤（UPS）的诊疗经验。方法 回顾性分析1例鼻腔鼻窦腺样囊性癌术后4年放疗诱发未分化多形性肉瘤患者的临床资料,并结合文献进行分析。结果 患者于2015年1月行鼻侧切开上颌骨大部分切除术,术中完整切除肿瘤,术后病检结果提示为左侧上颌窦腺样囊性癌,术后规律行放化疗,2018年12月患者考虑肿瘤复发,行鼻侧切开左侧上颌骨次全切除术及鼻腔鼻窦肿瘤切除术,完整切除肿瘤,术后病理结果提示为UPS。结论 UPS是一种罕见的异质性实体肿瘤,其发病率低,恶性程度高,预后差,手术完整切除肿瘤仍然是目前最有效的治疗方法,多学科综合治疗对其预后有积极作用。     

Intestinal and non-intestinal nasal cavity adenocarcinoma: Impact of wood dust exposure

The aim of the present study was to investigate the role of wood dust exposure in intestinal (ITAC) and non-intestinal type (non-ITAC) nasal adenocarcinoma, so as to improve understanding of the oncogenic mechanisms in the light of the recent literature and of evo-devo concepts.Materials and methodsAll consecutive patients operated in our institution for nasal adenocarcinoma diagnosed on anatomopathology between May 2004 and February 2014 were included. Surgical specimens were examined twice by independent pathologists, blind to wood dust exposure status. Clinical and demographic data, including wood dust exposure, were collected for the two groups (ITAC and non-IATC).Results90 patients (84 ITAC, 6 non-ITAC) were included. No non-ITAC patients had history of wood dust exposure, versus 83/84 cases (99%) in ITAC (mean exposure duration: 30 ± 16 years; range 2–65 years). Only 12 ITAC patients (18%) were still exposed at diagnosis. ITAC may develop long after the end of wood dust exposure (up to 60 years). Eight patients (12%) had exposure durations of less than 5 years. Latency between onset of exposure and onset of disease did not decrease with exposure duration.ConclusionExposure to wood dust, even for short periods of time, incurs a risk of developing ITAC, usually after a long latency period. Any exposure requires lifetime follow-up, to ensure prompt treatment. Factors leading to the development of nasal ITAC and non-ITAC are probably different. The analogy with Barret's esophagus and esophageal adenocarcinoma may shed light on the oncogenesis of nasal ITAC.     

Immunohistochemical distinction of intestinal-type sinonasal adenocarcinoma from metastatic adenocarcinoma of intestinal origin

OBJECTIVES: Distinction of intestinal-type sinonasal adenocarcinoma (ITAC) from adenocarcinoma of intestinal origin metastatic to the sinonasal cavity may be extremely difficult on histologic grounds alone. We studied the role of cytokeratin (CK) and mucin (MUC) expression in differentiating ITAC, metastatic adenocarcinoma of intestinal origin, and non-intestinal-type sinonasal adenocarcinoma (non-ITAC). METHODS: We stained specimens from 5 cases of ITAC and 4 cases of non-ITAC, along with 4 colonic and 3 duodenal adenocarcinoma controls, with CK7 and CK20, MUC2 and MUC5, neuron-specific enolase (NSE), chromogranin (CHR), and carcinoembryonic antigen (CEA) in order to examine the possible combinations of markers that best aid in the diagnosis of these lesions. We also performed a retrospective review of our clinical experience with these rare lesions. RESULTS: CK7 staining was positive in all ITAC and non-ITAC cases, whereas all cases displaying gastrointestinal-type differentiation (ITAC and metastatic intestinal cases) stained positive for both CK20 and MUC2. Staining for MUC5, NSE, CHR, and CEA was variable. CONCLUSIONS: Tumors with the CK7+, CK20+, MUC2+ immunophenotype are likely primary sinonasal lesions, whereas tumors with the CK7-, CK20+, MUC2+ profile warrant further clinical evaluation to exclude metastatic disease from the gastrointestinal tract. Complete surgical resection of ITAC remains the mainstay of therapy.     

Management of coexistent sinonasal pathology in patients undergoing endoscopic cerebrospinal fluid rhinorrhea repair

IntroductionPatients who undergo endoscopic cerebrospinal fluid rhinorrhea repair may occasionally present with coexistent sinonasal pathology which may or may not need to be addressed prior to surgical repair. Some patients may develop new onset nasal morbidity related to endoscopic repair.ObjectiveTo study the prevalence and management of additional sinonasal pathology in patients who undergo endoscopic repair of cerebrospinal fluid rhinorrheaMethodsA retrospective review of patients who underwent endoscopic cerebrospinal fluid leak repair was conducted to note the presence of coexistent sinonasal morbidity preoperatively and in the followup period.ResultsOf a total of 153 patients who underwent endoscopic closure of cerebrospinal fluid leak, 97 (63.4%) were female and 56 (36.6%) males. Most patients (90.2%) were aged between 21 and 60 years, with a mean of 40.8 years. Sixty-four patients (41.8%) were found to have coexistent sinonasal morbidity preoperatively, the commonest being symptomatic deviated nasal septum (17.6%), chronic rhinosinusitis without polyps (11.1%) and chronic rhinosinusitis with polyps (3.3%). Rare instances of septal hemangioma (0.7%) and inverting papilloma (0.7%) were also seen. Postoperatively, there was cessation of cerebrospinal fluid rhinorrhea in 96.7% which rose to 100% after revision surgery in those with recurrence. Resolution of coexistent sinonasal pathology occurred in all patients with followup ranging from 10 to 192 months. New onset sinonasal morbidity which developed postoperatively included synechiae between middle turbinate and lateral nasal wall (5.9%) and sinonasal polyposis (1.3%).ConclusionPatients who undergo endoscopic cerebrospinal fluid leak repair may have coexistent sinonasal pathology which needs to be addressed prior to or along with repair of the dural defect. New onset sinonasal morbidity, which may arise in a few patients postoperatively, may require additional treatment. A protocol for the management of coexistent sinonasal conditions ensures a successful outcome.     

Effects of unilateral sinonasal surgery on sleep-disordered breathing

Abstract

Aims/objectives: To examine the effects of surgery for unilateral sinonasal lesions on sleep-disordered breathing (SDB).

Material and methods: Oxygen desaturation index (3%ODI) as a marker of SDB and bilateral/unilateral nasal resistance were measured before and after surgery for 18 patients with unilateral sinonasal lesions. Various parameters were compared between those who achieved 60% or less decrease of 3%ODI and those who did not.

Results: Bilateral nasal resistance as well as that of the surgical side five days after surgery was significantly lower than those of pre-operative value. Preoperative 3%ODI (times/hour) was 10.08?±?7.32, which significantly decreased to 7.67?±?5.79 five days after surgery. Even in unilateral patients, sinonasal surgery could reduce the bilateral nasal resistance, resulting in a decrease in 3%ODI. Age was younger and postoperative nasal resistance of the surgical side was significantly lower in the group who achieved 60% or less decrease in 3%ODI than those who did not.

Conclusions and significance: SDB is influenced by even unilateral nasal obstruction. Surgery for unilateral lesion can improve the respiration during sleep as well as bilateral nasal resistance. Favorable outcome by surgery could be brought about in younger patients and those for whom sufficient improvement of nasal resistance was expected by surgery.     

Papilomas invertidos rinosinusales. Revisión de 61 casos

IntroductionInverted papillomas are benign sinonasal lesions that arise primarily from the lateral nasal wall which are characterized by their tendency to recur and propensity to be associated with malignancy. The aim of this work is to analyze our experience in the treatment of these lesions, especially through the endoscopic approach.Material and methodWe present 61 cases of sinonasal inverted papillomas that were treated at our hospital since 1983. The patients were studied by age, gender, site of presentation of the tumor, symptoms, radiologic studies, surgical treatment and evolution. The mean follow-up was 51 months (range 6–228 months).ResultsThirty eight of the patients (62%) were males and 23 females (38%). The average age was 58 years (range 22–80). The most common symptom was unilateral nasal obstruction in 91% of the cases, followed by rhinorrhea in 46%. The most frequent location was the ethmoid region in 51% of cases, followed by the maxillary sinus in 28% and the lateral nasal wall in 21% of the cases. 78% of cases had associated sinusitis and the 43% polyposis. Tumours were classified with the Krouse staging system as follows: 35% stage I, 37% stage II, 18% stage III and 8% stage IV. 37% of the patients had bony erosion in the CT scan. Six of the 42 patients treated by endoscopic procedures presented recurrence (14%), compared with 6 of the 9 patients (67%) who were treated by open approaches. The average time to the recurrence was 41 months. 17% of the patients had malignant inverted papillomas, nine of them diagnosed from the beginning as malignant neoplasies and one which became malignant during follow up.Discussion and conclusionsThe endoscopic approach is the method of choice for the treatment of the majority of inverted papillomas. The close follow up of the patient for a large period of time is necessary for the early detection of recurrence and to allow for surgical salvage.     

Sarcomatoid carcinoma in the sinonasal cavity: A retrospective case series from a single institution

ObjectivesSarcomatoid carcinoma (SC) is rare in the sinonasal cavity with poor prognosis. This study aimed to summarize the clinical characteristics, treatment and prognosis of patients with sinonasal SC from a single tertiary medical center.MethodsRetrospective review of the clinical data of seven patients diagnosed with sinonasal SC from May 1988 to February 2021.ResultsSeven patients with SC in nasal cavity and paranasal sinus, including six males and one female, aged 34-77 years (mean, 58.3 years), were included in this case series. The common presenting symptoms included epistaxis and nasal obstruction. The tumor usually occurred in nasal cavity, maxillary sinus and ethmoid sinus. Most patients were diagnosed at advanced stages with the involvement of orbit and skull base. Open and endoscopic surgery were performed in five and two patients, respectively. The follow-up period ranged from 3 to 26 months. Four patients died of disease within one year, though two received adjuvant chemotherapy and one received radiotherapy. Two patients survived without recurrence at the end of follow-up, both of them underwent adjuvant radiotherapy. One patient received palliative re-irradiation due to local recurrence of the tumor three months after the surgery, however, this patient was lost to follow-up.ConclusionsSinonasal SC is a rare entity with an aggressive behavior and poor prognosis. Early diagnosis and multimodality treatment are recommended to improve the survival.     

Prospective evaluation of clarithromycin in recurrent chronic rhinosinusitis with nasal polyps

IntroductionThe antiinflammatory effects of macrolides, especially clarithromycin, have been described in patients with chronic rhinosinusitis without polyps and also other chronic inflammatory airway diseases. There is no consensus in the literature regarding the effectiveness of clarithromycin in patients with chronic rhinosinusitis with sinonasal polyposis and the national literature does not report any prospective studies on the efficacy of clarithromycin in chronic rhinosinusitis in our population.ObjectiveTo evaluate the effect of clarithromycin in the adjunctive treatment of recurrent chronic rhinosinusitis with sinonasal polyposis refractory to clinical and surgical treatment.MethodsOpen prospective study with 52 patients with chronic rhinosinusitis and recurrent sinonasal polyposis. All subjects received nasal lavage with 20 mL 0.9% SS and fluticasone nasal spray, 200 mcg / day, 12/12 h for 12 weeks; and clarithromycin 250 mg 8/8 h for 2 weeks and, thereafter, 12/12 h for 10 weeks. The patients were assessed by SNOT 20, NOSE and Lund-Kennedy scales before, immediately after treatment and 12 weeks after treatment. The patients were also evaluated before treatment with paranasal cavity computed tomography (Lund-Mackay) and serum IgG, IgM, IgA, IgE and eosinophil levels. The outcomes evaluated were: SNOT-20, NOSE and Lund-Kennedy.ResultsMost patients were women, aged 47 (15) years (median / interquartile range), and 61.5% (32/52) had asthma. All patients completed the follow-up after 12 weeks and 42.3% (22/52) after 24 weeks. Treatment resulted in a quantitative decrease in the SNOT-20 [2.3 (1.6) vs. 1.4 (1.6); Δ = ?0.9 (1.1); p < 0.01]; NOSE [65 (64) vs. 20 (63); Δ = ?28 (38), p < 0.01] and Lund-Kennedy [11 (05) vs. 07 (05); Δ = ?2 (05); p < 0.01] scores. SNOT-20 showed a qualitative improvement (>0.8) in 54% (28/52, p < 0.04) of patients, a group that showed lower IgE level [108 (147) vs. 289 (355), p < 0.01]. The group of patients who completed follow-up 12 weeks after the end of treatment (n = 22) showed no worsening of outcomes.ConclusionLong-term adjuvant use of low-dose clarithromycin for chronic rhinosinusitis patients with recurrent sinonasal polyposis refractory to clinical and surgical treatment has resulted in improved quality of life and nasal endoscopy findings, especially in patients with normal IgE levels. This improvement persisted in the patient group evaluated 12 weeks after the end of the treatment.     

Results of preoperative chemoradiotherapy for patients with advanced cancer of the nasal cavity and paranasal sinuses

Objectives: Curative treatment of nasal cavity and paranasal sinus cancer is challenging due to the proximity to critical anatomical structures. The purpose of this study was to analyze the impact of trimodality therapy with preoperative chemotherapy and reduced-dose radiotherapy followed by organ-preserving surgery for treating patients with nasal cavity and paranasal sinus cancer.

Methods: This retrospective study included all 156 patients diagnosed with sinonasal cancer in western Sweden between 1986 and 2009. We determined the treatment selection pattern and treatment outcomes for 79 patients treated with preoperative chemoradiotherapy.

Results: Squamous cell carcinoma was the most common histology. The five-year overall survival was 54%, and 85% of these patients had T3 or T4 tumors. The five-year cumulative incidence rate of local recurrence was 32%. The five-year overall survival in patients with squamous cell carcinoma and adenocarcinoma was 45% and 76%, respectively. The median preoperative radiation dose was 48?Gy. Orbital exenteration was performed in 7% of patients.

Conclusions: Preoperative chemoradiotherapy may be beneficial for patients with advanced sinonasal cancer when primary radical surgery is challenging. Survival outcomes were comparable to outcomes reported in the literature despite conservative surgery and relatively low radiation doses in patients with locally advanced tumors.     

Predictors of nodal metastasis in sinonasal squamous cell carcinoma: A national cancer database analysis

ObjectiveWe present the largest population based study of sinonasal squamous cell carcinoma (SCC) to identify risk factors for presentation with nodal metastasis.MethodsThe National Cancer Database (NCDB) was used for this study. Location codes corresponding to the nasal cavity and paranasal sinuses and histology codes representing SCC malignancy were queried. Logistic regression analysis was performed to identify factors associated with presentation with nodal metastasis.Results6448 cases met inclusion criteria. Nodal metastasis at presentation was seen in 13.2% of patients, with the sinus subsite (19.3%) being a significant risk factor for nodal metastasis at presentation when compared to the nasal cavity (7.9%). Logistic regression analysis showed black, uninsured and Medicaid patients were more likely than white and privately insured patients, respectively, to present with nodal metastasis.ConclusionsIn sinonasal SCC, the sinus subsite has a significantly increased risk of nodal metastasis compared to the nasal cavity. Black race, uninsured and Medicaid patients are more likely to have nodal metastasis at presentation.