Extracranial head and neck schwannomas--a 10-year review

OBJECTIVES: To present our experience in managing a large case series of extracranial schwannomas highlighting presenting features, diagnostic difficulties, and outcomes associated with surgical treatment of these tumours. METHOD: A retrospective case note study of 31 patients with a diagnosis of extracranial schwannoma seen in the Department of Otolaryngology, Head and Neck Surgery at Southmead Hospital, a tertiary referral centre and University hospital between 1 June 1993 and 30 May 2003. RESULTS: The commonest anatomical location was in the neck (42%) and an isolated neck lump was the commonest presentation (77%). Pressure symptoms were the next most common mode of presentation, and were often a helpful indicator of the nerve of origin. The nerve of origin was identified in 47% of patients who underwent surgery. Immunohistochemistry was a useful tool in the diagnosis of these tumours and magnetic resonance imaging was the preferred imaging technique to delineate their extent. The most significant postoperative morbidity was associated with the schwannomas of the vagus nerve, sympathetic chain, hypoglossal nerve, glossopharyngeal nerve and the facial nerve. CONCLUSION: Schwannomas can present in a wide variety of sites within the head and neck region and therefore it is important that otolaryngologists and head-neck surgeons are familiar with the more common sites of presentation and the potential difficulties associated with the diagnosis and management of these tumours. Adequate imaging should be carried out preoperatively to gain as much information as possible about the individual tumour and allow informed patient counseling regarding to potential risks and morbidity of surgical intervention.     

头面部颅外脑膜瘤11例分析

目的 探讨头面部颅外脑膜瘤的临床特征及诊断、治疗特点。方法 对11例头面部颅外脑膜瘤的临床资料进行回顾性的分析、总结。结果 肿瘤病变部位：眶内5例，鼻腔2例，颞窝、颞下窝各1例，顶枕部2例。手术入路颅面联合入路2例，眶外侧入路1例，鼻侧切开术2例，眶内容物剜除术1例，颞窝、颞下窝入路各1例，额眶入路1例，顶枕部肿瘤表面入路2例。11例均行手术治疗。全切肿瘤9例，近全切肿瘤2例，无手术死亡及严重并发症发生。1例术后行放疗，1例2年后复发行X刀治疗。随访期6个月至7年均存活。结论 头面部颅外脑膜瘤是少见的病例，主要表现出所在部位的局部症状，对不易活检的部位，术前定性诊断困难。治疗以手术为主，全切预后较好。     

Extracranial head and neck schwannomas.

Extracranial head and neck schwannomas are usually benign and rarely malignant tumors of peripheral nerve sheath origin. The clinical presentation of these tumors is reviewed in our series of 21 patients, emphasizing the diagnosis with dynamic infused CT scans. Management is suggested and outcome analysis is provided.     

Familial head and neck cancer: molecular analysis of a new clinical entity

OBJECTIVE: The tumor suppressor gene p16 encodes a cyclin-dependent kinase inhibitor that normally inhibits cell proliferation by causing a G1 cell cycle arrest. The p16 gene is frequently mutated in a variety of somatic tumors, as well as in familial melanoma and familial pancreatic carcinoma. We identified a family with a high incidence of head and neck squamous cell carcinoma (HNSCC) and melanoma. Molecular analyses of the p16 gene locus in blood and tumor DNA from this family was performed to determine whether an association between germline p16 gene mutation and HNSCC exists. STUDY DESIGN: Molecular pedigree analyses. METHODS: Exon 2 of p16 was polymerase chain reaction amplified from blood, tumor, or nontumor DNA isolated from affected and unaffected members, then directly sequenced and compared with consensus p16 sequence. Cell cycle position of cells expressing wild-type or mutant p16 was determined by flow cytometry. RESULTS: Molecular analyses revealed a nonfunctional germline point mutation within exon 2 of the p16 gene that encodes a mutant p16 protein substituting proline at amino acid position 87 for the wild-type arginine (p16R87P). Relative to wild-type p16, p16R87P lost ability to cause a growth arrest following ectopic expression. The mutant (p16R87P) allele segregated with cancer predisposition in tested family members, and analyses of HNSCC tumor tissues demonstrated universal loss of wild-type allele. CONCLUSIONS: Significance of the mutant p16 (p16R87P) in HNSCC tumorigenesis is strongly suggested by its loss of cell cycle arrest activity and its retention in tumor tissue with simultaneous loss of the wild-type allele. Further, the germline p16 mutation segregated with cancer predisposition within the family. In aggregate, these data suggest that there is a direct causal relationship between the germline p16 mutation in this family and HNSCC tumorigenesis. Based on our observations, the spectrum of familial cancers associated with p16 mutations should include a new clinical entity, familial HNSCC.     

Extracranial head and neck schwannomas: a review of 8 years experience

Schwannomas of the head and neck are uncommon tumors that arise from cranial, peripheral or autonomic nerves. In this study we review a series of 52 cases of schwannoma originating in the head and neck region over an 8-year period. All the tumors were benign, with the exception of one malignant schwannoma. The age range of the patients studied was 13-76 years and there was a predilection for males. Twenty-five schwannomas occurred in the scalp, face and external ear canal, 9 in the oral or nasal cavity and 18 in the neck. Seven cases of neck schwannoma originating from the major nerve system were found in the parapharyngeal space, all of which were located in the post-styloid compartment. Cervical plexus schwannomas originated either in the peripheral nerves or in an unidentified area of the nervous system; seven tumors were found in the posterior triangle of the neck and two in the anterior triangle. Two of the tumors originating in the brachial plexus were located in the posterior neck and one in the anterior neck. Tumors originating in the vagus nerve or sympathetic chain were all located in the anterior triangle of the neck.     

Fibromatosis of the head and neck

Fibromatosis is a locally infiltrative fibrous tissue proliferation with a tendency to recur locally. From a large series of head and neck patients treated between 1977 and 1994 in our institute, we retrieved the records of nine adult patients diagnosed with this disease. They serve as examples to demonstrate this rare entity in the head and neck. Five out of nine lesions were localized in level V (posterior triangle of the neck). The majority of patients were treated by surgery in combination with radiotherapy. None of the patients died of the disease.     

Extracranial head and neck schwannomas: a study of the nerve of origin

Schwannoma is a type of benign nerve sheath tumour arising from the Schwann cell. Because of the close relationship between the tumour and the nerve of origin (NOO), the operation of extracranial head and neck schwannoma may lead to palsy of major nerve. For this reason, an accurate diagnosis of schwannoma with the identification of the NOO is crucial to the management. The aim of this review was to find out the distribution of the NOO and the usefulness of the investigations in the diagnosis of schwannoma. Medical records of the patients who underwent operation of the extracranial head and neck schwannoma in our division were reviewed. Between January 2000 and December 2009, 30 cases of extracranial head and neck schwannoma were operated. Sympathetic trunk (10, 33%) and vagus nerve (6, 20%) were the two most common NOOs. In five (17%) cases, the NOO was not found to be arising from any major nerve. For these 30 patients, 20 received fine needle aspiration cytology (FNAC) and 26 underwent imaging studies (computed tomography or magnetic resonance imaging) before operation. The specificity of FNAC and imaging studies in making the diagnosis of schwannoma was 20 and 38%, respectively. For the patients who had nerve palsies on presentation, their deficits remained after operation. The rate of nerve palsy after tumour excision with division of NOO and intracapsular enucleation was 100 and 67%, respectively. The diagnosis of schwannoma is suggested by clinical features and supported by investigations. Most of the time, the diagnosis can only be confirmed on the histological study of the surgical specimen. Sympathetic trunk and vagus nerve are the two common NOOs. MRI is the investigation of choice in the diagnosis of schwannoma and the identification of NOO.     

Gastrostomy tubes in patients with advanced head and neck cancer

OBJECTIVES: To measure the percentage of patients requiring gastrostomy tubes (G-tubes) and the timing of their placement, in addition to studying whether pretreatment variables (T stage, tumor site, N stage) and intratreatment variables (weight loss during treatment) are valid predictors for the need for G-tube placement. STUDY DESIGN: Retrospective case control study. METHODS: Retrospective review of a prospectively collected database with chart reviews was carried out on 477 patients treated on a regimen of intraarterial cisplatin and concurrent radiation. The likelihood of requiring a G-tube was calculated by studying several independent variables using chi-square analysis and the unpaired t test. RESULTS: Two hundred twenty (46%) patients had G-tubes placed, with mean time of placement being on day 20 after the initiation of treatment. Patients with T4 disease were statistically more likely to require a G-tube (50% vs. 37%, P < .05). Patients were less likely to require a G-tube when the primary site of tumor involved the larynx or paranasal sinuses (P < .05). There were no statistically significant differences between the two groups with regards to patient age, sex, N stage, and weight changes over the course of treatment. The odds of requiring a G-tube were highest for tumors involving the hypopharynx (80%), oral cavity (52%), and oropharynx (49%). CONCLUSIONS: Although clinical judgment appears to be sufficient for deciding when G-tube placement is warranted in our population, the threshold for placing a tube should be lower in those patients who had a higher likelihood of requiring a G-tube during the course of treatment, including those with advanced disease of the oral cavity, oropharynx, and hypopharynx.     

Survival of adult patients with head and neck soft tissue sarcomas

A retrospective review and survival analysis is presented of 58 adult patients referred between 1963 and 1993 for treatment of a head and neck soft tissue sarcoma. The 5 year overall and disease-free survival was 60% and 52% respectively. Univariate analysis indicated that age, positive margins. bone invasion, distant metastases, single modality treatment, histological grading, sites below the deep fascia and size might be predictors of survival. On multivariate analysis, age, histological grade, distant metastases, site and referral for recurrent disease emerged as prognostic factors known before treatment. By including variables known after treatment, age remained the most important independent prognostic factor followed by the presence of free margins, histological grading and distant metastases at presentation.     

Kimura病33例临床分析

目的:探讨Kimura病(KD)的临床特征、病理学表现和并发症等,提高对该病的认识及诊疗水平.方法:回顾性分析33例确诊为KD患者的临床资料.结果:22例表现为头颈部肿块,11例表现为身体其他部位肿块,如腹股沟、腋窝、肺门及肠系膜根部等;21例累及淋巴结,8例发生于大唾液腺;23例外周血嗜酸性细胞升高,2例行血清总IgE检测,均明显升高;7例尿常规异常,其中3例为大量蛋白尿(+++),2例为血尿(+);6例行骨髓细胞学检查,其中2例嗜酸性细胞升高;2例并发肾病综合征;6例并发头颈部局限性炎性疾病,2例并发恶性肿瘤.结论:KD常表现为头颈部肿块,多累及淋巴结和大唾液腺.不明原因外周血嗜酸性细胞升高的患者应考虑到KD的可能,血清总IgE和肿块病理检查有助于KD的早期诊断.     

Quality of life of patients with head and neck cancer

Patients with head and neck cancer have to deal with the impact of treatment on its functional and aesthetic aspects, and its self-report enables improvements in clinical and social support.ObjectiveTo evaluate the quality of life of patients dealing with squamous cell carcinoma of the head and neck.MethodA prospective analytical study. Twenty nine patients with mean age of 57 years answered at three stages: onset, middle and end of treatment, the questionnaires: Quality of Life Core Questionnaire - Cancer 30 and the Quality of Life Questionnaire - Head and Neck, the European Organization for Research and Treatment of Cancer. We used the Friedman test at: 0.05.ResultsThere were high mean values concerning physical, cognitive, social functions; improvements in general health and social function decline during treatment; and a significant difference in taste and smell (p = 0.020), swallowing (p = 0.040), cough (p = 0.013) and weight loss (p = 0.011).ConclusionThere was a significant reduction in the quality of life for some common symptoms resulting from cancer treatment, which was not seen in the evaluation of the aspects related to physical, cognitive and social functions, and general health.     

Gene expression: a review of clinical applications in otorhinolaryngology‐head and neck surgery

Tissue engineering is a multidisciplinary area of research aimed at regeneration of tissues and restoration of function of organs through implantation of cells/tissues grown outside the body or stimulating cells to grow into implanted matrix. In this short review, we aim to examine current techniques in gene expression analysis and their relevant clinical applications to the field of otorhinolaryngology‐head and neck surgery.     

Clinicopathological study of patients with head and neck sarcomas

Sarcomas are rare tumors, mainly stemming from the embryonic mesoderm, with a high grade of morbidity and mortality.ObjectiveTo carry out a retrospective study of head and neck sarcoma cases between 1999 and 2008 in three specialized centers in the city of Fortaleza.Materials and MethodsData collection was based on the charts of the patients in the study. For statistical analysis purposes we used the chi-square associations and the z test for proportions.ResultsWe found records of 36 patients, and the most affected ones were adult brown males, in the age range between 20 and 59 years - mean age of 39.7. The man/woman ratio was 1.76:1. The most prevalent histological type was the rhabdomyosarcoma and their most common locations were the face and the neck. Most of the sample was made up of live patients without evidence of the disease in the last visit - 41.6% of the cases. The most common treatment modes were the combination of surgery + radiotherapy + chemotherapy and surgery + radiotherapy, with 27.8% of the cases each.ConclusionSarcomas have a great histological variability and may have numerous locations. Since these are rare and not well-known lesions, new epidemiological studies must be carried out in order to enhance our understanding of the disease.     

Squamous carcinoma of the head and neck: the untreated patient

One thousand, nine hundred and seventy-seven patients with squamous carcinoma of the head and neck are reviewed with particular reference to the patients not treated. The commonest reason for not treating the patients was that the tumour was too extensive (55%). A further 36% were too old or infirm to be considered fit for radical treatment, and 9% of patients refused treatment. Treated and untreated patients were compared for age, sex, site of the primary and TNM stage. The proportion of patients untreated varied according to site of the primary tumour, with 25% of pharangeal tumours being the highest proportional representation. Median survival for untreated patients was 88 days; survival time was significantly related to sex, T stage of primary tumour and patient's general condition.     

Demands on caring relatives of head and neck cancer patients

BACKGROUND: Relatives of cancer patients experience high levels of stress that influence the quality of life of these individuals. To investigate whether there is a necessity for simultaneous supportive care of patient relatives, we performed for the first time a study asking the closest relatives of head and neck cancer patients about their needs during and after the treatment to consider how to optimize the situation for such patient groups. MATERIAL AND METHODS: Patients' relatives were assessed using an anonymous self-report questionnaire that was established in our department by expanding on a questionnaire for cancer patients' relatives from the psycho-oncologic society in Switzerland. The evaluation was multidimensional, cancer specific, and relative based. RESULTS: Relatives feel confronted themselves with cancer, although indirectly. The majority of the respondents were of the opinion that simultaneous psychological care of the patients and for the caring relatives would be helpful to cope with the situation. CONCLUSION: This study shows the significant impact of cancer on caring relatives of head and neck cancer patients. In our opinion, health services should become more aware of this potential to ensure that the needs of the involved patient relatives are met as well as those of the patients.     

头颈部神经鞘瘤33例临床分析

目的:探讨头颈部神经鞘瘤的诊断和治疗。方法:回顾性分析1980~2004年收治的33例头颈部神经鞘瘤的临床资料。结果:全部患者均行手术治疗,术后经病理学检查确诊良性32例,恶性1例。术后30例患者获随访,其中27例治愈,1例恶性肿瘤患者死于复发,2例死于其他疾病;3例失访。结论:手术彻底切除肿瘤是治疗该病最有效的方法,手术尽可能保留来源神经的完整性。该病预后良好,对恶变者应行扩大切除。     

Life-satisfaction in patients with head and neck cancer

Life-satisfaction is a measure of a patient's perception of the difference between his reality and his needs, or wants. This study reports the results of a longitudinal survey of patients’self-reported life-satisfaction following treatment for head and neck cancer. Life-satisfaction scores improved with time, and were related to pain, speech difficulty, and dysphagia. Lack of adequate family support was also important, although an uncommon problem. Treatment modality did not emerge as a significant determinant of life-satisfaction; speech difficulties were more likely to be due to articulation problems than voicing difficulty.     

The clinical implications of antitumor immunity in head and neck cancer

Recent developments have renewed interest in understanding the interaction between transformed cells and the immune system in the tumor microenvironment. Here, we provide a comprehensive review addressing the basics of tumor immunology in relation to head and neck cancer and the cellular components potentially involved in antitumor immune responses. In addition, we describe the mechanisms by which head and neck cancer cells escape immune-mediated killing and progress to form clinically significant disease. Further, we detail what effects standard anticancer therapies may have on antitumor immune responses and how these responses may be altered by current and investigational immunotherapies. Finally, we discuss future directions that need to be considered in the development of new immunotherapeutics designed to durably alter the immune response in favor of the host.