嗅神经母细胞瘤16例临床分析

目的：回顾性分析嗅神经母细胞瘤的临床表现、治疗经过及预后。方法：16例嗅神经母细胞瘤，A期4例，B期6例，C期6例。其中单纯放疗或加化疗6例，手术加术后放疗9例，单纯手术1例。放疗剂量DT40Gy-70Gy，均为常规分割照射。颈部未行预防照射。结果：本组病例一、三和五年生存率分别为75%、75%和69%。随访中发现颈部淋巴结转移由初次确诊时的19％上升至44％，再次放疗敏感。结论：嗅神经母细胞瘤对放疗敏感，A期单纯放疗疗效好，B、C期宜手术加术后放疗。     

53例嗅神经母细胞瘤的临床分析

背景与目的：嗅神经母细胞瘤患者预后不良,如何提高治疗效果、延长患者的生存期是临床研究中亟需解决的问题。本研究探讨影响嗅神经母细胞瘤预后的因素及合理的治疗方法。方法：回顾性分析1980～2003年间我院收治的53例嗅神经母细胞瘤患者的临床资料,采用Kaplan—Meier法分析嗅神经母细胞瘤的临床特征、治疗方式与预后的关系,并比较不同治疗方式的疗效。结果：本组患者总5年生存率为41％。单纯手术组、单纯放疗组、单纯化疗组、手术联合放化疗组和放疗联合化疗组患者的5年生存率分别为42％、38％、0％、56％、0％,采用不同治疗方式患者的生存率比较差异有统计学意义（P〈0．05）。Kadish临床分期A期、B期、C期5年生存率分别为100％、37％、31％,不同临床分期患者生存率比较差异有统计学意义（P〈0．05）。复发率51％,复发组、无复发组5年生存率分别为42％、48％。结论：嗅神经母细胞瘤复发率高,以手术为主的综合治疗方案是较优的治疗模式。预后与临床分期、治疗方式有关。     

嗅神经母细胞瘤：（附6例报告）

嗅神经母细胞瘤是一种罕见的恶性肿瘤,我院自1978年～1988年共收治6例。例1,男,43岁,因恶心、呕吐20天,于1980年11月急诊。CT示:前颅凹占位病变。行开颅肿瘤切除。病理为:前颅凹星形细胞瘤。术后~(60)C_0前颅凹D_T45Gy/7周。1981年5月因左鼻塞就诊,检查:左鼻腔后部暗红色新     

30例嗅神经母细胞瘤的临床分析

目的 探讨嗅神经母细胞瘤的临床特征、治疗方法与预后的关系.方法 回顾性分析1992年1月至2007年5月间我院收治的30例嗅神经母细胞瘤患者的临床资料.采用Kaplan-Meier法分析全组临床分期、颈部淋巴结转移情况、治疗方式、眼眶内颅内侵犯情况和年龄与预后的关系.结果 本组患者总5年生存率为43.3%.临床分期A+B期、C期5年生存率分别为80%、25%,两组患者生存率比较差异有统计学意义(P＜0.05);颈部淋巴结转移阳性组与阴性组的5年生存率分别为25%、55.6%,两组生存率比较差异有统计学意义(P＜0.05);单纯手术组、单纯放疗组、单纯化疗组、手术联合放化疗组、放疗联合化疗组患者的5年生存率分别为0%、33.3%、33.3%、52.9%、33.3%(P＞0.05);有眼眶内颅内侵犯与无眼眶内颅内侵犯的患者5年生存率分别为21.4%、62.5%,两组生存率差异有统计学意义(P＜0.05);发病年龄≥50岁与发病年龄＜50岁的患者5年生存率分别为23.1%、58.8%,两组生存率差异有统计学意义(P＜0.05).结论 嗅神经母细胞瘤预后与年龄、临床分期、有无颈部淋巴结转移以及眼眶内颅内侵犯有关.     

嗅神经母细胞瘤25例临床分析

1资料与方法1.1一般资料我院1995年5月至2004年4月收治25例嗅神经母细胞瘤。男性11例、女性14例,年龄10～78岁,中位年龄42岁。主要临床症状为鼻衄、鼻塞23例,头痛7例,眼球突出、眶内疼痛、视力下降5例;首次确诊时颈淋巴结转移2例,首次确诊时未发现远处转移。根据改良Kadish分期A期4例,B期10例,C期9例,D期2例。1.2治疗方法全组单纯放疗9例,综合治疗16例。其中改良Kadish A期单纯放疗3例,手术+放疗1例;B期单纯放疗2例,手术+放疗8例;C期单纯放疗4例,手术+放疗3例,手术+放疗+化疗1例,放疗+化疗1例;D期手术+放疗+化疗1例,放疗+化疗1例。手术方式包括鼻腔肿物切除术2例,鼻侧壁切开肿瘤切除术7例,上颌骨切除术3例,开颅肿瘤切除术2例,同期颈淋巴结清扫术1例。放疗根据病变范围设鼻前野、正侧两野成角楔形照射或面颈联合野,筛窦区电子线补量,1例行颈部预防照射。单纯放疗剂量60～72Gy,(中位剂量70Gy),术后放疗原灶剂量50～70Gy,(中位剂量60Gy),颈部预防剂量50Gy。化疗多采用CAV方案(CTX、ADM、VCR)或AI方案(ADM、IFO),化疗1～6周...     

嗅神经母细胞瘤9例临床分析

目的 探讨嗅神经母细胞瘤的临床特点、诊断及治疗.方法 回顾性分析经病理证实的9例嗅神经母细胞瘤的临床资料.结果 按Kadish分期法,A期0例,B期7例,C期2例;9例中,2例行放疗+化疗,7例行手术+放疗,结果 显示含有手术的综合治疗方案疗效较好.结论 嗅神经母细胞瘤的发病率极低,因无特异性临床症状或体征而容易误诊,...     

嗅神经母细胞瘤5例临床病理分析

嗅神经母细胞瘤颇为罕见。现将我科近20临床诊治年来外检中所遇5例报告如下。临床资料患者年龄最大者7O岁，最小者22岁。男4例，女1例。主要症状有单侧性鼻阻、反复鼻出血。双侧鼻阻1例，头痛1例，视力下降、咽痛、发音障碍1例。局部检查：多数为单侧鼻腔内出现息肉状肿物，色淡红，表面光滑或有糜烂、出血、质脆、触之易出血，单侧鼻腔中鼻甲以上3例，位于鼻咽部2例，经CT检查1例示左鼻腔及上颌窦内占位病变。5例均未发现颈部淋巴结肿大。临床诊断鼻息肉3例，恶性肿瘤1例，待诊1例。病理观察送检标本大者1．scmXI．scmX0·6cm，小者0．s…     

嗅神经母细胞瘤诊断与治疗的临床分析

目的探讨鼻腔嗅神经母细胞瘤的诊断治疗方法及预后的相关因素。方法回顾性分析我院1994年1月-2010年5月收治的嗅神经母细胞瘤12例,改良Kadish分期A期1例,B期5例,C期3例,D期3期。含手术的综合治疗6例,单独手术4例,单独放疗1例,单独化疗1例。结果全组患者5年总生存率（OS）为90.9%,5年无病生存率（DFS）为74.1%。A、B期和C、D期患者的5年OS分别为100%和80.0%（χ²=1.20,P=0.27）,5年DFS分别为100%和44.4%（χ²=4.07,P=0.04）。有淋巴结转移和无淋巴结转移患者的5年OS分别为66.7%和100%（χ²=2.67,P=0.10）,5年DFS分别为33.3%和87.5%（χ2=4.88,P=0.03）。综合治疗与单一治疗的5年OS分别为100%和80.0%（χ²=1.20,P=0.27）,5年DFS分别为83.3%和62.5%（χ2=0.35,P=0.88）。结论嗅神经母细胞瘤的早期诊断较难,该病的治疗以综合治疗疗效较好,放疗是该病重要的治疗手段,该病预后与疾病分期及淋巴结转移情况相关。     

鼻腔嗅神经母细胞瘤11例临床分析

目的 回顾分析鼻腔嗅神经母细胞瘤的放疗疗效.方法 1974～1994年鼻腔嗅神经母细胞瘤11例,其中B期6例,C期5例.单纯放疗6例,术后放疗5例.3例术后无肿瘤肉眼残留者照射剂量D_T45Gy～55Gy/23～28次共4.5～5.5周.其余8例照射剂量D_T均在60Gy/30次共6周以上.3例颈淋巴结转移者照射剂量D_T60Gy～65Gy/30～33次共6～7周.结果 本组病例三、五年生存率分别为55%、36%,单纯放疗组分别为33%、17%,术后放疗组分别为80%、60%.P值0.2727,二组在统计学上无显著差异.全组病例中3例局部复发,2例血行转移.结论 B、C期患者根治性手术加术后放疗为局部控制的较好治疗方案.     

加减八珍汤辅助治疗小儿神经母细胞瘤的效果观察

目的:观察加减八珍汤辅助治疗小儿神经母细胞瘤的临床疗效。 方法:将80例小儿神经母细胞瘤患者按照随机数字表法分为2组,每组各40例。实验组在基础治疗上给予加减八珍汤治疗,对照组在基础治疗上予以安慰剂,均连续治疗4周,同时观察治疗期间中医证候变化、治疗效果、生活质量、肿瘤进展、不良反应等情况。4周后,以生活质量评分表、中医症状量表进行问卷调查,统计药物干预前后各组患者评分并进行数据分析。结果:两组患儿治疗前各症状程度比较,差异无统计学意义;服加减八珍汤治疗后,实验组疗效评价较治疗前均改善（P<0.05）,且实验组总体生活质量评分均优于对照组（P<0.05）。结论:加减八珍汤辅助治疗小儿神经母细胞瘤有显著效果,可提高患者生存质量,且疗效安全、可行。     

嗅神经母细胞瘤和嗅神经上皮瘤的临床特点和治疗评价

背景与目的：随着电镜和免疫组化诊断技术的提高，关于嗅神经母细胞瘤和嗅神经上皮瘤的报道逐渐增多。本研究回顾性分析嗅神经母细胞瘤和嗅神经上皮瘤的临床特点和治疗效果，探讨其治疗方法。方法：嗅神经母细胞瘤和嗅神经上皮瘤在临床上被认为是同一种疾病，故将经病理证实的19例嗅神经母细胞瘤和10例嗅神经上皮瘤共同分析。其中单纯放疗10例，术前放疗15例，术后放疗4例。根据Kadish分期标准，A期3例，B期8例，C期18例。生存分析采用Kaplan—meier法及Logrank检验：结果：患者表现为单侧鼻塞、单侧鼻衄或两者兼有者有26例，为89．66％。初次就诊时有颈淋巴结转移者5例，为17．24％。全组5年生存率、5年无瘤生存率分别为70．76％、66．48％：病理诊断为嗅神经母细胞瘤和嗅神经上皮瘤患者5年生存率及5年无瘤生存率比较差异无显著性。A／B期、C期5年生存率分别为100．00％、54．32％：有颈淋巴结转移者（N＋）和无颈淋巴结转移者（N0）4年无瘤生存率分别为60．00％、71．05％（P〈0．05）：结论：淋巴结转移是一个预后不良的因素，随着内镜手术的使用，放疗成为嗅神经母细胞瘤（嗅神经上皮瘤）的主要治疗方法。     

儿童神经母细胞瘤36例临床特点及预后因素分析

目的:分析儿童神经母细胞瘤的临床特点、疗效及预后。方法:回顾分析2010年1月至2016年6月我院血液肿瘤科收治的36例神经母细胞瘤患儿的临床资料,采用Kaplan-Meier法进行生存分析。结果:36例患儿男20例,女16例,平均年龄25个月（5~47个月）。随访至2017年6月30日,中位随访时间:21.5个月（5~81个月）,2年总生存率（OS）61.9%,II期、III期、IV期分别为100%、65%、11.1%。结论:尽早明确神经母细胞瘤分期,给予合适的综合治疗方案,能明显减少化疗毒副反应、延长生存率,提高临床治愈率。     

儿童多发性神经母细胞瘤7 例临床分析*

目的：总结儿童多发性神经母细胞瘤（neurobl astoma ,NB）的临床特征及生物学行为,以期对这类肿瘤的治疗及预后判断提供依据。方法：回顾性分析天津医科大学肿瘤医院儿童肿瘤科1995年1 月至2015年12月收治的7 例多发性NB患儿的临床资料并对其随访。结果：共收治360 例NB患儿,其中多发NB仅7 例（1.9%）。 患儿平均年龄（19.4 ± 11.9）个月;原发部位胸腹多发3 例,双肾上腺多发4 例;临床分期4S 期4 例（57.1%）,3 期2 例（28.6%）,4 期1 例（14.3%）。 7 例共15个原发灶：14个手术完整切除;组织学预后良好型（favorable histology ,FH）12个（85.7%）,组织学预后不良型（unfavorable histology,UH）2 个（14.3%）;13个（92.9%）MYCN 基因无扩增。随访满3 年的5 例患儿,3 年总生存率（overall survivalrate ,OS）为100% 。结论：多发性NB极其罕见,具有初次诊断年龄较低、以4S 期为主、手术易完整切除的临床特征,肿瘤细胞分化较成熟、FH居多、少见MYCN 基因扩增等生物学行为。该病多数预后较好,应避免过度治疗。     

嗅神经母细胞瘤的CT表现

目的探讨CT在嗅神经母细胞瘤的诊断和分期中的价值。方法回顾性分析15例嗅神经母细胞瘤的CT表现。男10例,女5例。平扫8例,增强扫描7例。结果肿瘤中心位于筛窦10例,鼻腔5例;9例呈膨胀性生长,6例浸润性生长,侵犯范围为鼻腔及筛窦各14例,眼眶及上颌窦各9例,蝶窦、额窦、脑实质及鼻咽各3例,海绵窦及颞下窝各1例。14例伴有相邻骨质破坏。7例增强扫描中,轻度强化4例,中度强化3例,1例出现钙化。结论CT扫描可清楚显示肿瘤大小与部位,冠状面及矢状面扫描能更好地显示侵犯范围,具有重要意义。     

手术加放射治疗嗅神经母细胞瘤14例

目的：评价手术加放射,治疗嗅神经母细胞瘤的疗效。方法：１９７５年１月－１９９８年２月,收治嗅神经母细胞瘤１４例,按Ｋａｄｉｓｈ分期Ａ期１例,Ｂ期８例,Ｃ期５例,采用手术加放射治疗,原发灶平均剂量６０００ｃＧｙ。结果：现仍生存６例,已生存时间为２～２４年,死亡８例,存活时间为６个月～４年。５例死于局部复发,３例死于远处转移。结论：（１）嗅神经母细胞瘤理上易误诊。免疫组化攻电镜有助于确诊;（２）本病不仅极易局部复发,也易发生远处转移;（３）手术加放射是治疗本病的适宜方法,方向治疗对减少术后复发,提高手术切除率、控制转移灶均有较大价值。     

嗅神经母细胞瘤15例临床病理及免疫组化分析

Objective: We studied the clinicopathological and immunohistochemical features of olfactory neuroblastoma (ONB). Methods: The clinic pathological data and immunohistochemical features of 15 cases with ONB were analyzed ret- rospectively, and the related literatures were reviewed. Results: The tumors were all located in the nasal cavity in 15 cases. According to Kadish's staging system, 7 cases were in stage A, 5 cases in stage B, and 3 cases in stage C. The morphological features showed small round or ovoid tumor cells divided into pieces or trabeculae by connective tissues which were rich in capillaries. The tumor cells had round or oval nuclei and fine chromatins and lack of cytoplasma. Flexner rosette and Homer- Wright rosette were found in some cases. Acidophilic fibrins were composed of cytoplasmic projection among tumor cells. All cases were positive for NSE, Syn, CgA, 1 case was positive for Vimentin, 2 cases were positive for S-100, while CKpan, LCA and HMB45 were all negative. Conclusion: ONB is a type of very rare malignant tumors, which could be diagnosed by pathology, and immunohistochemistry is helpful in the diagnosis and differential diagnosis.     

Nonplatinum-based chemotherapy with irinotecan plus docetaxel for advanced or metastatic olfactory neuroblastoma: a retrospective analysis of 12 cases

BACKGROUND: The efficacy and safety of chemotherapy with irinotecan plus docetaxel were retrospectively evaluated for olfactory neuroblastoma. METHODS: Twelve patients with histologically proven advanced or metastatic olfactory neuroblastoma were treated with chemotherapy with irinotecan plus docetaxel at the study institution between 2001 and 2005. Of these, 7 patients with locoregional disease and no prior radiotherapy received irinotecan plus docetaxel followed by definitive radiotherapy, 1 with photon radiotherapy and 6 with proton radiotherapy, whereas 3 patients with distant metastases and 2 with locoregional disease who had received prior radiotherapy received irinotecan plus docetaxel only. RESULTS: The most common toxicities of >or=grade 3 among the 12 patients receiving irinotecan plus docetaxel were leukopenia (33%), neutropenia (50%), febrile neutropenia (8%), and diarrhea (25%), all of which were manageable. Partial response was achieved in 3 patients, giving an overall response rate of 25%. The response rate was higher in patients aged <50 years (3 of 4 patients) compared with those aged >50 years (0 of 8 patients) (P = .018). With a median follow-up period of 22.2 months, the median progression-free survival and overall survival were 13.6 months and 36.6 months, respectively. Of the 7 patients with locoregional disease also receiving definitive radiotherapy, the 2-year survival rate was 100% and 6 patients were alive at the time of last follow-up. CONCLUSIONS: Chemotherapy for olfactory neuroblastoma with irinotecan plus docetaxel is safe and manageable. Patients aged <50 years may be sensitive to chemotherapy. Induction chemotherapy followed by definitive radiotherapy may represent a promising option for patients with locally advanced olfactory neuroblastoma.     

Recurrent olfactory neuroblastoma treated by radiation, surgery, and chemotherapy: "a case report"

Olfactory neuroblastoma is a rare tumor. The role of CT Scan, morphological details and the electron microscopic findings have been highlighted in the diagnosis of the lesion. The present patient, in which the tumor had recurred following surgical excision, was treated successfully with a combination of radiation, further surgery, and maintenance chemotherapy. We also feel that such a planned multidisciplinary approach is justified as the initial treatment for all advanced lesions in view of high recurrence rates reported following use of radiotherapy or surgery alone.     

Chemotherapy in the treatment of advanced or recurrent olfactory neuroblastoma

Background: Olfactory neuroblastoma is a rare sino‐nasal tumor arising from the olfactory epithelium and is often characterized by local invasion or metastasis. The role of chemotherapy in the treatment of this tumor is unclear. The purpose of this study was to review our institution’s experience of chemotherapy for advanced or recurrent olfactory neuroblastoma. Methods: Twenty‐one patients with histologically proven olfactory neuroblastoma were treated at our institution between 1992 and 2002. Twelve of these patients received chemotherapy in the setting of unresectable or recurrent disease and were retrospectively reviewed for clinical characteristics, treatment outcome or survival. Results: Eight patients of the 12 patients received cisplatin‐based chemotherapy and the remaining four patients received chemotherapy consisting of docetaxel plus irinotecan (three patients) or cyclophosphamide, doxorubicin, and vincristine (1 patient). A partial response was achieved in five patients, with an overall response rate of 42%, although the chemotherapeutic regimens were heterogeneous. Two partial responses were obtained among the three patients who received docetaxel plus irinotecan. The response rate to chemotherapy was 83% in the younger age group (<40 years), as opposed to 0% in the older age group (≥40 years), and the difference between the two groups was statistically significant (P = 0.02). Conclusion: Our study indicated that olfactory neuroblastoma would be sensitive to chemotherapy, especially with young patients. Docetaxel plus irinotecan has the possibility of showing favorable response, and warrants further investigation.