复发性鼻腔及鼻窦恶性黑色素瘤的治疗

目的 探讨鼻腔及鼻窦恶性黑色素瘤复发的原因和治疗方法.方法 回顾性分析1993～2003年我科收治的11例复发性鼻腔及鼻窦恶性黑色素瘤患者的临床资料.结果 临床误诊或误治引起的复发患者4例,病理明确手术治疗后复发7例,其中5例为单纯手术后复发,2例为手术加术后放疗后复发.11例复发病例中,局部复发局限于鼻腔及鼻窦7例;局部复发侵犯眼眶、颅底、软腭及硬腭1例;局部复发侵犯腮腺和皮肤1例;面部、眼眶及颅底广泛侵犯者1例;颈部淋巴结转移复发1例.首次复发时间最短为治疗后10个月.所有病例再次手术治疗后的累积生存率为:1年生存率72.7%,2年生存率18.2%,3年生存率9.1%.结论 减少误诊,选择适当的治疗方法是提高复发性鼻腔及鼻窦恶性黑色素瘤治愈的主要手段.     

鼻腔鼻窦恶性黑色素瘤17例临床分析

目的探讨鼻腔鼻窦恶性黑色素瘤的诊断和治疗,提高本病的诊疗水平。方法回顾性分析1980年3月-2011年6月收治的17例鼻腔鼻窦恶性黑色素瘤的临床资料,分析其临床病理特点、诊断、治疗及预后。结果患者的主要症状为单侧鼻塞,涕中带血,头痛;病灶位于鼻腔外侧壁和鼻中隔。17例患者中1例放弃治疗,2例行单纯放疗,14例行手术治疗,3、5年生存率分别为36．4％和22．2％。患者死亡原因为局部复发和远处转移。结论鼻腔鼻窦恶性黑色素瘤恶性程度高,易复发和转移,预后差,临床上尽早确诊。广泛彻底手术切除肿瘤,并辅以放疗、化疗、免疫治疗等综合治疗,是提高本病生存率的关键。     

鼻腔鼻窦恶性黑色素瘤24例报告

目的：探讨鼻腔鼻窦恶性黑色素瘤的病理、临床特点及治疗方法。方法：鼻腔鼻窦恶性黑色素瘤24例中，鼻侧切开鼻腔鼻窦肿瘤切除术18例，颈外动脉结扎上颌骨大部分切除术2例，上颌窦根治术径路鼻腔鼻窦肿瘤切除术2例，颅面联合径路前颅底鼻腔鼻窦肿瘤切除术2例，同期行颈淋巴清扫术4例；单纯手术14例，手术加术后放疗8例，手术加放疗和化疗2例，其中1例加免疫治疗（IL-2和IFN）。结果：24例患者中随访22例，失访2例，3年生存率为54．5％（12／22），5年生存率为31．8％（7／22）。结论：鼻腔鼻窦恶性黑色素瘤恶性程度高，易复发，早期诊断、根治性手术切除能提高本病生存率。     

鼻腔鼻窦粘膜恶性黑色素瘤的治疗选择和生存

鼻腔鼻窦粘膜恶性黑色素瘤较少见，其发病率占全身恶性黑色素瘤的1％以下，占鼻腔鼻窦肿瘤的4％以下，不管选择何种治疗方法，其预后均较差，文献报告5年生存率为6．5％～34％。作者早期的治疗经验提示手术加放疗可能会增加生存期，但未得到大组病例长期随访的统计学分析证实。为此，作者对1963年至1996年期间治疗的鼻腔鼻窦恶性黑色素瘤72例中资料完整的58例进行回顾性分析，旨在确定选择手术加放疗是否比单纯根治性手术对生存期有所增加。58例中男性30例，女性28例，年龄39岁～90岁，平均年龄64岁。生存分析用Kaplan－Meter法。临床表现…     

鼻内镜下切除鼻腔鼻窦黏膜源性恶性黑色素瘤

目的探讨鼻腔鼻窦黏膜源性恶性黑色素瘤的临床特点及治疗方法。方法分析3例鼻腔鼻窦黏膜源性恶性黑色素瘤的临床特点,总结鼻内镜下手术加放疗的临床疗效。结果3例患者均在鼻内镜下一次性完全切除肿瘤,术后放疗,随访11—19个月肿瘤元复发。结论对鼻腔鼻窦黏膜源性恶性黑色素瘤,如未发现远处转移,可通过鼻内镜下行肿瘤切除术,术后辅以放射治疗能达到临床治愈的目的。     

鼻腔鼻窦恶性黑色素瘤19例分析

目的 :提高对原发于鼻腔、鼻窦恶性黑色素瘤的诊疗水平。方法 :对本院 1992～ 2 0 0 0年收住的 19例鼻腔、鼻窦原发恶性黑色素瘤的临床表现、诊断、治疗方法和随访结果进行分析。结果 :该病病理与临床易与鼻息肉、鼻腔癌、血管瘤相混淆 ,彻底的手术切除及良好的局部控制与生存时间有关 ,放疗、化疗与免疫治疗的疗效有待进一步验证。结论 :鼻腔、鼻窦原发恶性黑色素瘤为临床难治疾病之一 ,预后较差 ,早期诊断及彻底手术切除是提高疗效的首选措施。     

鼻腔鼻窦恶性黑色素瘤的临床诊疗进展

鼻腔鼻窦恶性黑色素瘤(SMMM)是临床少见的鼻腔鼻窦恶性肿瘤,发病症状通常不典型,就诊时多数患者已属晚期,5年生存率仅为20%~30%。以病理诊断为金标准,组织细胞内发现黑色素颗粒有助于明确诊断,对于无色素型恶性黑色素瘤,免疫组化可帮助鉴别诊断。与Ballantyne分期相比,第7版美国癌症联合委员会(AJCC)头颈黏膜恶性黑色素瘤TNM分期更适用于SMMM。目前,早期SMMM的治疗仍以手术为主,手术加放疗以及生物化疗的综合治疗模式有助于提高患者的生存率。     

鼻腔鼻窦非上皮源性恶性肿瘤7例报告

报告鼻腔鼻窦非上皮源性恶性肿瘤7例,其中恶性黑色素瘤2例,恶性神经鞘瘤2例,非霍奇金氏淋巴瘤2例,平滑肌肉瘤1例.因鼻腔鼻窦恶性黑色素瘤罕见,故易误诊,其中1例因高度疑为此病,经病检证实,及时准确切除了瘤体,术后存活5年余;恶性神经鞘瘤临床确诊主要依靠病检,诊断平滑肌肉瘤则需结合免疫组化检测,随免疫组化的开展,非霍奇金氏淋巴瘤的临床与病理诊断符合率已有提高,前三者治疗以手术切除为主,非霍奇金氏淋巴瘤则可先化疗再局部放疗.     

鼻腔鼻窦腺样囊性癌88例临床分析

目的 总结鼻腔鼻窦腺样囊性癌的临床特征和治疗方法,探讨影响鼻腔鼻窦腺样囊性癌患者预后的因素.方法 总结中山大学肿瘤防治中心头颈外科1975年11月至2003年8月收治的88例原发于鼻腔鼻窦的腺样囊性癌患者的临床及病理资料,回顾性分析单纯手术、单纯放疗与放疗结合手术、化疗等治疗方式的疗效.对其治疗与预后进行统计分析.生存分析采用Kaplan-Meier法,组间比较采用Log-rank检验,多因素分析采用Cox比例风险模型.结果 就诊时56例鼻腔鼻窦腺样囊性癌患者为Ⅲ、Ⅳ期病变,治疗多采用手术+放疗.全组患者的5年、10年、15年生存率分别为0.640、0.341、0.190;Ⅲ期患者的5年、10年生存率分别为0.833、0.221;Ⅳ期患者的5年、10年生存率分别为0.323、0.145.手术+放疗组、单纯手术组及单纯放疗组的5年、10年生存率分别为0.761、0.415,0.750、0.367,0.286、0.143.结论 鼻腔鼻窦腺样囊性癌的治疗应以综合治疗为主,临床分期及治疗方式是影响鼻腔鼻窦腺样囊性癌患者预后的独立因素.     

髓外浆细胞瘤的诊断和治疗(6例报告及文献复习)

目的探讨髓外浆细胞瘤(Extra-medullary Plasmacytoma,EMP)的诊断及治疗。方法回顾性分析我科10年来收治的3例鼻腔、2例鼻咽、1例鼻窦EMP的临床资料,结合文献对其临床表现、病理特点、鉴别诊断及治疗进行探讨。结果6例病人中3例鼻腔EMP病人单纯手术治疗,2例鼻咽部EMP病人行单纯放疗,1例鼻窦EMP病人手术后放疗。随访2~11年,其中1例鼻咽部病人放疗后6年死于肺部感染,1例鼻窦EMP病人于放疗1年后失访,其余4例随访至今未见复发。结论EMP是少见的低度恶性肿瘤,依据临床和组织学特点,结合免疫组化可明确诊断,放疗和手术是治疗EMP主要方法。     

喉小涎腺癌15例临床分析

目的 探讨喉小涎腺癌的临床特点、治疗及预后.方法 回顾性分析中国医学科学院肿瘤医院头颈外科1959至2005年收治的15例喉小涎腺癌患者的临床资料.11例(73.3%)病变位于声门上区,4例(26.7%)病变位于声门下区.其中腺样囊性癌10例,腺癌2例,黏液表皮样癌、恶性混合瘤、基底细胞腺癌各1例.单独手术7例;手术+放疗7例,其中6例患者行术后放疗,1例行术前放疗;1例行放疗+化疗.治疗后4例局部复发患者行手术挽救,其中l例肺转移后行转移灶切除;1例治疗后颈转移患者行放疗.结果 随访时间2～16年,中位数为8年.治疗后局部复发4例次,颈淋巴转移1例次,远处转移5例次,其中局部复发伴远处转移3例.7例患者存活3～16年,其中5例为无瘤生存,1例行单纯手术治疗,4例行手术+放疗;4例远处转移于治疗2～10年后死亡;其余4例随访2～16年后失访.结论 喉小涎腺癌很少见,其易发生局部复发和远处转移,远处转移是最主要的死亡原因.外科手术是喉小涎腺癌的主要治疗方法 ,手术结合放疗可望提高肿瘤局部控制率.     

儿童侧颅底肿瘤的诊断和治疗

目的:探讨儿童侧颅底肿瘤的临床特点、诊断及治疗方法.方法:回顾性分析8例儿童侧颅底肿瘤的临床表现、影像学特点及治疗方法.其中颞部并中、后颅窝巨大三叉神经鞘膜瘤1例.颞部并中、后颅窝黑色素神经外胚层肿瘤1例,婴幼儿颞骨纤维瘤病1例,斜坡脊索瘤1例,鼻咽部胚胎型横纹肌肉瘤2例,神经母细胞瘤2例.1例经颞下窝Fisch C型及迷路上联合径路、1例经岩骨径路、4例经颞下窝Fisch C型径路将肿瘤全切除,1例经腭径路行肿瘤次全切,1例单纯行化疗未做手术.7例手术患儿中4例术前、术后化疗,3例术后化疗.结果:除1例脊索瘤患儿术后5个月复发死亡外,其余7例患儿均存活(6例手术、1例未手术),其中2例鼻咽部胚胎型横纹肌肉瘤术后小灶复发,1例神经母细胞瘤化疗后未行手术的患儿,6个月后复发.除术前1例外展神经、3例三叉神经受累及外,术后2例出现短暂脑脊液漏,1例同侧听力丧失.1例声嘶(同侧声带外展麻痹),2例吞咽困难,术后3～4个月渐恢复.7例手术患儿均无伤口感染裂开及皮瓣坏死,未出现面瘫、脑膜炎及偏瘫、死亡等严重并发症.结论:儿童侧颅底肿瘤生长部位深在、临床症状复杂多样、隐匿、不典型,往往确诊时已为晚期,积极行CT和MRI检查有助于提高早期诊断率.手术切除肿瘤仍为首选,手术前后配合放、化疗.手术径路的选择应依据病变的部位和范围,颞骨和颞下窝联合径路可以最大限度切除侵及该区域的肿瘤,保存脑神经功能,减少并发症.     

Craniofacial resection for malignant melanoma of the skull base: report of an international collaborative study

OBJECTIVE: To report postoperative mortality, complications, and outcomes in a subset of patients with the histologic diagnosis of malignant melanoma extracted from an existing database of a large cohort of patients accumulated from multiple institutions. DESIGN: Retrospective outcome analysis. SETTING: Seventeen international tertiary referral centers performing craniofacial surgery for malignant skull base tumors. PATIENTS: A total of 53 patients were identified from a database of 1307 patients who had craniofacial resection for malignant tumors at 17 institutions. The median age was 63 years. Of the 53 patients, 25 (47%) had had prior single modality or combined treatment, which included surgery in 22 (42%), radiation in 11 (21%), and chemotherapy in 2 (4%). The margins of resection were close or microscopically positive in 7 (13%). Adjuvant radiotherapy was given in 22 (42%), chemotherapy in 3 (6%), and vaccine or interferon therapy in 2 (4%). Complications were classified into overall, local, central nervous system, systemic, and orbital. Overall survival (OS), disease-specific survival (DSS), and recurrence-free survival (RFS) were determined using the Kaplan-Meier method. Predictors of outcome were identified by multivariate analysis. RESULTS: Postoperative mortality occurred in 3 patients (6%) and postoperative complications were reported in 14 patients (26%). Local wound complications occurred in 6 patients (11%), central nervous system in 7 (13%), systemic in 3 (6%), and orbital in 1 (2%). With a median follow-up of 10 months (range, 1-159 months), the 3-year OS, DSS, and RFS rates were 28.2%, 29.7%, and 25.5%, respectively. The extent of orbital involvement and adjuvant postoperative radiation therapy (PORT) were independent predictors of DSS and OS on multivariate analysis, whereas only PORT was an independent predictor of RFS. Patients treated with PORT had significantly better 3-year OS (39% vs 18%; relative risk, 2.9; P = .007), DSS (41% vs 19%; relative risk, 3.0; P = .007), and RFS (39% vs 15%; relative risk, 4.2; P = .001). CONCLUSIONS: Craniofacial resection in patients with malignant melanoma of the skull base has mortality (6%) and complication rates (26%) comparable to other malignant tumors of the skull base. However, malignant melanoma is associated with a much poorer OS, DSS, and RFS. Adjuvant PORT correlated with improved 3-year OS, DSS, and RFS on multivariate analysis. These factors must be taken into account when considering craniofacial resection in a patient with malignant melanoma invading the skull base.     

Clinical analysis of 16 cases of malignant head and neck melanoma

Subjects were 16 patients--5 men and 11 women aged 46-82 years (mean: 61 years)--with malignant melanoma of the head and neck treated at our clinic from 1972 to 1988. Histologically, 1 subjects was amelanotic and 15 melanotic type. Primary lesions were 10 involving the nasal cavity, 2 the paranasal sinus, 2 the gingiva, 1 the lip, and 1 primary unknown. They were treated with or without multimodal surgery, radiation, chemotherapy, and immunotherapy. Of 12 treated using local surgery, local recurrence was seen in 6 in 7 areas. Two-year survival was 44% and 5-year survival 22%. The prognosis of malignant head and neck melanoma is poor but has gradually improved due to preoperative decisions on disease spread and the introduction of multimodal therapy.     

Olfactory neuroblastoma: the Hokkaido University experience

Olfactory neuroblastoma is such a rare malignancy that no consensus has been reached on its management. We analyzed 17 patients with olfactory neuroblastoma treated between April 1980 and March 2004--9 men and 8 women, aged 16 to 76 years old(mean: 50.4 years). Follow-up of current survivors was 1 year 8 months to 16 years 6 months (average: 7 years 9 months). Initially, 2 were treated with surgery alone, 5 with surgery and radiotherapy, and 2 with a combination of these and chemotherapy. Without surgery, radiotherapy alone was conducted in 3 and combined of radiation and chemotherapy in 5. Three of the 5 patients treated with surgery and radiotherapy survive without locoregional recurrence as do 2 with chemotherapy added. All 5 initially treated with craniofacial resection survived more than 5 years. Combined radiotherapy and chemotherapy without surgery was effective in 2. 5- and 10-year overall survival for all patients were 75.5% and 64.7%. Overall 5-year survival of 8 patients with low-grade tumors was 87.5% and of 6 with high-grade tumors 33.3%. In conclusion, combined craniofacial resection plus radiotherapy and chemotherapy seemed to improve survival. Histopathological grading is a prognostic factors.     

Management Options and Survival in Malignant Melanoma of the Sinonasal Mucosa

Objective/Hypothesis: To determine whether surgery combined with radiotherapy confers any survival benefit on radical local excision alone in the management of mucosal malignant melanoma of the nose and sinuses. Study Design: Retrospective review. Methods: From a cohort of 72 patients treated between 1963 and 1996 within a single unit, complete data were available for 58 individuals who were examined to determine whether there was any significant statistical difference in local control and/or survival between those receiving surgery and those receiving combined surgery and radiotherapy. Results: There were 30 men and 28 women. Their ages ranged from 39 to 90 years (mean, 64 y). Twenty-nine patients underwent surgery alone; 23 patients underwent surgery and radiotherapy; 6 patients received surgery and chemotherapy; and 3 patients received surgery, chemotherapy, and radiotherapy. Primary surgery included lateral rhinotomy (42 cases), maxillectomy (8 cases [orbital clearance in 3 cases]), craniofacial resection (3 cases), total rhinectomy (3 cases), and endoscopic clearance (2 cases). Survival ranged from 1 to 228 months with rapid patient loss due to local (and/or systemic) disease during the first 36 months, irrespective of the treatment modality. Overall 5-year actuarial survival was 28%, and overall 10-year actuarial survival 20%, with a median survival of 21 months. There was no statistical difference in local control or survival between patients receiving surgery alone and those receiving surgery and radiotherapy, irrespective of whether this treatment was given in the early part of the series (i.e., before 1983) or thereafter. The addition of chemotherapy had no impact on survival, nor did the site of the tumor, the surgical procedure, the presence of lymph node metastases or the age of the patient. Conclusion: In this large cohort of sinonasal melanoma, overall survival was poor and did not appear to be improved by the addition of radiotherapy.     

The role of radiation therapy in the treatment of head and neck cutaneous melanoma

Introduction: This study was undertaken to elucidate the efficacy of external beam irradiation in the treatment of head and neck malignant melanoma, in comparison with the efficacy of surgical excision and the efficacy of surgical excision combined with external beam irradiation.Materials and Methods: A retrospective chart review of all patients seen at the Department of Radiation Oncology at the University of California at Los Angeles Medical Center with the diagnosis of head and neck melanoma from 1973 to 1992 was conducted. Using χ² analysis, survival rates were examined for patients treated with excision alone, irradiation alone, and both excision and irradiation.Results: Patients were treated with primary excision (96%), salvage excision for recurrent or residual disease (47%), primary irradiation (9%), post-surgical-planned irradiation (22%), adjuvant chemotherapy (45%), and adjuvant immunotherapy (36%). The overall survival rate for the entire group was 29%. For patients with recurrent or regional disease, survival rates were 37% for those treated with excision in combination with irradiation, 20% for those treated with irradiation alone, and 19% for those treated with excision. Whereas the differences among groups were not statistically significant, the biologic implication is that external beam irradiation is effective in controlling regional disease, since the tumors treated with irradiation alone or excision in combination with irradiation were larger and more aggressive than those treated with excision alone.Conclusion: External beam irradiation is of benefit in treating patients with melanoma who are ineligible for salvage surgery or as an adjunctive modality in situations where the risk of racurrence is high.     

Metastases in both internal auditive meatus of nasosinusal melanoma

Primary malignant melanoma of the nasal and paranasal sinusses is rare and represent 3% of malignant nasosinusal tumors. The prognosis of this tumor is poor. The evolution is unpredictable; there is very commonly a series of local recurrences which may be well controlled, followed by remote metastases remaining long quiescent. There are no statistical difference in local control or survival between patients receiving surgery alone and those receiving surgery and radiotherapy. Chemotherapy is used for metastatic forms. We present a case of a patient with a primary malignant melanoma of the nasal mucosa. Tree years after diagnosis we found intracranial metastases in left and right auditive internal meatus.