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。     

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。     

鼻腔鼻窦侵袭型真菌病(附2例报告及文献复习)

目的探讨鼻腔鼻窦侵袭型真菌病的发病机理、临床表现及分类、诊断和治疗。方法报告2例鼻腔鼻窦侵袭型真菌病的临床资料，复习相关文献。结果①鼻腔鼻窦侵袭型真菌病发病率低，但破坏性强，易迅速向周围邻近组织扩散，造成功能障碍。②临床分类：根据其临床表现和过程分为慢性、急性爆发性及肉芽肿性侵袭型鼻腔鼻窦真菌病。③诊断：主要依据临床表现、影像学、病理学及微生物学检查，真菌培养阳性可以确诊。④治疗：尽早、合理的手术治疗是关键，辅以抗真菌、纠正全身疾病、提高机体免疫等综合治疗。⑤2例病人鼻内镜下鼻窦根治，感染控制，病灶吸收，功能恢复。结论侵袭型鼻腔鼻窦真菌病是一种少见的真菌性疾病，病情凶险，预后差。早期诊断、以手术为主的综合治疗能有效提高病人的生存率、降低致残率。     

鼻腔鼻窦肠型腺癌中表皮生长因子受体蛋白表达及相关基因突变分析

目的　研究鼻腔鼻窦肠型腺癌组织中表皮生长因子受体（epidermal growth factor receptor,EGFR）蛋白的表达及KRAS（kirsten rat sarcoma viral oncogene）和 BRAF（B-Raf proto-oncogen）基因的突变及临床意义。方法　免疫组化分析EGFR蛋白在肿瘤组织胞内和胞外的表达,并分析磷酸化EGFR（p-EGFR）蛋白的表达情况;以直接测序法和热熔曲线法测定EGFR、KRAS和BRAF基因在肿瘤组织中的突变情况。结果　11例鼻腔鼻窦肠型腺癌组织中EGFR蛋白的阳性表达率为72.7%,细胞内、外EGFR蛋白的表达有显著相关性;p-EGFR的阳性表达率为9.1%。EGFR的突变率为9.1%,发生于第19外显子,KRAS和BRAF基因的突变率均为9.1%,分别发生于第2和第15外显子。EGFR蛋白表达及EGFR、KRAS和BRAF基因的突变与患者无进展生存期或总生存期的长短无相关性。结论　鼻腔鼻窦肠型腺癌组织中有EGFR蛋白的过表达,显示了肿瘤细胞的异质性;且存在EGFR、KRAS和BRAF基因的突变,突变率相对较低。     

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...     

鼻腔鼻窦Masson瘤一例报道及文献复习

Masson瘤,也被称为血管内乳头状内皮细胞增生症,是一类血管组织来源的良性增生性病变,鼻腔鼻窦发病罕见。本文报道了一例鼻腔鼻窦Masson瘤。患者单侧鼻塞伴溢泪6月,肿物内镜下外观光滑界清,CT呈膨胀性生长,MR下肿物信号混杂,不均匀强化,血液学检查提示D-二聚体增高。经柯陆氏及鼻内镜径路切除肿物,经术后病理明确诊断。结合既往文献报道,发现鼻腔鼻窦Masson瘤多发生于上颌窦、筛窦和鼻腔,均为单侧发病但缺乏典型发病特征,一般经病理学检查确诊。其治疗以手术为主,预后较好。     

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...     

非侵袭型鼻腔及鼻窦真菌病14例报告

报告１４例非侵袭型鼻腔及鼻窦真菌病患者的临床资料。全部采用手术治疗，均经病检所证实，其中８例术后加用局部抗真菌药物治疗，发现其疗效明显高于单纯手术组。提示手术彻底清除病灶加上局部使用抗真菌物是目前治疗非侵袭型鼻及鼻窦真菌病的理想方法。     

Sinonasal adenocarcinomas: Retrospective analysis from an oncology center

Introduction and objectivesSinonasal adenocarcinomas are rare and heterogeneous tumors and for this reason remain understudied. Our purpose is to analyze clinical presentation, outcomes and factors affecting survival of patients with sinonasal adenocarcinomas, treated at our institution.Material and methodsRetrospective review of clinical records of patients with sinonasal adenocarcinoma, treated at a tertiary oncology institution (January 2010 to December 2019).ResultsSixty patients diagnosed with adenocarcinoma were included, with male preponderance (54.90%) and a mean age of 57.5 ± 14.9 years. Adenocarcinoma was the most frequent type of sinonasal cancer, contrary to previous reports from our institution. In most patients, it was detected at an advanced disease stage, significantly decreasing their survival rate when compared to patients diagnosed at initial stages (p < 0.029). Intestinal type adenocarcinoma was established in 47 patients, the most frequent being the colonic subtype (24%). Tumors with good/moderate histologic differentiation presented a survival advantage over those that were poorly differentiated (p = 0.043). The most common treatment modality was surgery followed by radiotherapy. Endoscopic resection was performed in 53% of the patients and an external approach was used in 40% of the patients. Estimated overall survival rates at 3 and 5-years were 64% and 53%, respectively, and cancer specific survival 72% and 65%, for the same period. Recurrence rate was 32% and occurred mainly locally. Disease Free Survival rate was 71% at 3 years and 65% at 5 years.ConclusionsDespite being the most common sinonasal malignancy in our sample, in contrast to other series, sinonasal adenocarcinomas are still rare tumors with a substantial local failure rate of around 30%. Advanced stage at diagnosis and histologic differentiation grade negatively affected prognosis of these tumors.     

Retrospective study and review of polymorphous low-grade adenocarcinoma

OBJECTIVES/HYPOTHESIS: We review a single institution's experience with polymorphous low-grade adenocarcinoma. To our knowledge, this is the largest patient series of polymorphous low-grade adenocarcinoma with clinical follow-up in the otolaryngology literature. STUDY DESIGN: We retrospectively identified 19 patients with polymorphous low-grade adenocarcinoma who had adequate clinical follow-up and pathologic specimens available for examination. METHODS: All pathologic materials were reviewed by one head and neck pathologist. RESULTS: The median follow-up was 9.6 years. The most frequently affected sites were the hard palate (12 patients) and soft palate (9 patients). Fifteen patients had their initial treatment at our institution, and four patients presented with a recurrent tumor. Five patients had a local recurrence after surgery; of those patients, two had initially presented with recurrent tumors. Local tumors recurred as late as 15 years after the initial surgery. One patient had regional nodal disease 20 years after the initial procedure, and another had lung metastasis. No patients received chemotherapy. The most common initial diagnoses were polymorphous low-grade adenocarcinoma, adenoid cystic carcinoma, and pleomorphic adenoma. CONCLUSIONS: Polymorphous low-grade adenocarcinoma is an increasingly recognized malignancy that originates predominantly in the minor salivary gland. Our experience confirms good local control after a wide local excision is performed, but local recurrences can occur despite having negative margins after surgery. The delayed local recurrences and regional nodal metastases noted in our series show that prolonged follow-up is needed. This series also reports one of the first pathologically confirmed cases of distant metastasis.     

Sinonasal mucosal melanoma: A 44-case study and literature analysis

Objectives

Sinonasal mucosal melanoma is a rare disease, representing 4% of nasal cavity and paranasal sinus malignancies. The aim of this study was to assess the clinical characteristics, progression and treatment of this disease and to identify prognostic factors.

Sinonasal inverted papilloma associated with small cell neuroendocrine carcinoma: A case report and literature review of rare malignancies associated with inverted papilloma

We report a rare case of sinonasal inverted papilloma (IP) associated with small cell neuroendocrine carcinoma (SNEC). To our knowledge, this is the first report to describe SNEC found during the treatment of sinonasal IP. Surgery and five cycles of cisplatin plus etoposide with concurrent intensity modulated radiation therapy were performed. Neither local recurrence nor distant metastasis was noted during 6 years of post-diagnostic follow-up. The prognosis of SNEC is very poor. Treatment planning for sinonasal IP should consider a possible association with this rare but aggressive malignancy, whose treatment is completely different from that of squamous cell carcinoma, a malignancy which is commonly associated with IP.We also performed a PubMed review of the literature to identify the incidence and pathological diagnosis of associated malignancy. Among a total of 5286 cases of sinonasal IP (61 studies), the incidence of associated malignancy was 8.02% in squamous cell carcinoma, 0.19% in transitional cell carcinoma, 0.04% in mucoepidermoid carcinoma, 0.02% in verrucous cell carcinoma and 0.02% in adenocarcinoma. The incidence of associated malignancy was significantly higher in East and Southeast Asia (11.0%) and North America (10.4%) than in Europe (3.9%) (p = 0.04 and p = 0.03, respectively; T-test).     

Sinonasal intravascular papillary endothelial hyperplasia successfully treated by endoscopic excision: a case report and review of the literature

BACKGROUND: Intravascular papillary endothelial hyperplasia (IPEH) is an abundant, usually intravascular, benign endothelial proliferation that may mimic angiosarcoma clinically and histopathologically. Its occurrence in the sinonasal cavity is extremely rare. To the best of our knowledge, we present the first case with the most extensive IPEH ever found within the sinonasal cavity. METHODS: A 42-year-old man of intravascular papillary endothelial hyperplasia in the sinonasal cavity was reported. He complained of a 1-year history of left-sided nasal obstruction accompanied by unilateral rhinorrhea, repeated epistaxes and frontal headache. Anterior rhinoscopy demonstrated a smooth-surfaced reddish mass occupying the left nasal cavity. Endoscopic surgery was used to clear this uncommon tumor in the sinonasal cavity. A review of the pertinent literature was also presented. CONCLUSIONS: IPEH may be mistaken for an angiosarcoma clinically and histopathologically. Complete endoscope-guided surgical excision is the best choice of therapy for patients with IPEH and is both diagnostic and curative. Awareness of this lesion will prevent incorrect diagnosis and overly aggressive treatment.     

Management of a pediatric patient with aggressive low-grade sarcoma of the maxillary sinus: Case report and literature review

Pediatric sinonasal tumors are characterized by histological diversity, nonspecific clinical presentations, and variable recurrence and metastatic potential. We describe a rare low-grade, undifferentiated sarcoma with bland/benign histopathology but an aggressive course in a 10-year-old boy with nasal obstruction, rhinorrhea, and left eye proptosis. Imaging revealed a large sinonasal soft tissue mass completely obstructing the left nasal cavity, without intraorbital involvement or distant metastasis. The mass stained positive for S100, vimentin, glial fibrillary acidic protein, and Ki-67 and negative for desmin and EGFR (epidermal growth factor receptor). The patient underwent neo-adjuvant chemoradiation therapy, and definitive surgical resection via a left lateral rhinotomy/maxillectomy approach. Post-treatment results were excellent, and the patient has remained disease free at one-year follow-up based on clinical, radiographic, and direct endoscopic visualization examinations with minimal morbidity or functional disability.     

Genetic and clinical aspects of wood dust related intestinal-type sinonasal adenocarcinoma: a review

Intestinal-type sinonasal adenocarcinoma (ITAC) is a rare epithelial cancer of the nasal cavities and paranasal sinuses. Exposure to wood dust particles is a strong etiological factor making it a professional disease. These tumors are locally aggressive with frequent local recurrences in up to 50% of cases. Metastasis to regional lymph nodes and distant metastasis are less frequent (10%). Invasion of the duramater and local recurrence are frequent and the major cause of death. Standard therapeutic modalities include surgery followed by radiotherapy in advanced stages, sometimes with chemotherapy treatment. The molecular genetic mechanisms underlying the development and progression of this tumor is not understood. Histopathologically, ITAC resembles colorectal adenocarcinoma and have directed early genetic studies to search for similar genetic alterations. Recently, genome-wide studies have identified a recurrent pattern of chromosomal aberrations. This review aims to describe the clinico-pathological characteristics of this relatively unknown tumor and to summarize the knowledge on genetic and chromosomal analyses up to the present time.     

Sinonasal intestinal-type adenocarcinoma: report of 7 cases

The intestinal-type adenocarcinoma of the nasal cavity and paranasal sinuses are uncommon tumors, with less than 4% of the total of malignancies of this region. They have histological similitudes with the glandular estructure of the intestinal mucosa. In some aspects they are similar to others tumors of this area, symptoms, an etiological relation with the exposure to wood dust ... but they have differences in the local aggressivity, this is important for the tractament, evolution and survival. The authors present a revision about clinic characteristics, diagnostic and tractament of seven cases of nasosinusal intestinal type adenocarcinoma.     

Kikuchi-Fujimoto disease: Report of 4 cases and review of the literature

IntroductionKikuchi-Fujimoto disease or histiocytic necrotizing lymphadenitis is a benign disease of unknown origin predominantly affecting young women and presenting in the form of cervical lymphadenopathy and/or prolonged fever.Material and methodsThe authors report 4 cases of Kikuchi-Fujimoto disease diagnosed in the Internal Medicine Department of Ibn Sina university hospital in Rabat between 2009 and 2010.ResultsThese 4 women with a mean age of 27 ± 8.6 years [16–37] were admitted with febrile syndrome and cervical lymphadenopathy. The diagnosis was based on histological examination of a lymph node biopsy. The disease was associated with systemic lupus erythematosus in one case and actinomycosis in another case. A favourable course was observed in response to corticosteroid therapy in two patients, antibiotic therapy in one patient and antipyretic treatment alone in the fourth patient.ConclusionIn the light of these four cases, the authors discuss the diagnostic difficulties, the modalities of treatment of Kikuchi-Fujimoto disease and its clinical course.