Primary undifferentiated carcinoma of the trachea.

BACKGROUND: Primary undifferentiated or lymphoepithelial carcinoma mainly occurs in the nasopharynx. Tracheal localization is exceedingly rare. To the best of our knowledge, only four cases have been reported previously, all in Asian patients. CASE REPORT: A 61-year-old male European patient presented with hemoptysis and cough for several months. The workup revealed a primary tracheal tumor without regional or distant metastasis. The patient was treated with tracheal resection followed by end-to-end reconstruction. Pathologic analysis of the tumor, including immunohistochemistry, confirmed the diagnosis of lymphoepithelioma-like carcinoma. Testing for Epstein-Barr virus by hybridization in situ was massively positive. With a follow-up at 15 months, the patient is alive and free of disease. CONCLUSION: We report the first case of lymphoepithelioma-like carcinoma in the trachea in the European population. Treatment possibilities are discussed. They should be based on each patient's clinical presentation and the results of their preoperative workup.

An innovative approach to tracheotomy in patients with major obstruction of the upper airway.

BACKGROUND: Patients with severe dyspnea consecutive to locally advanced obstructive head and neck squamous cell carcinoma (HNSCC) or subglottic stenosis requiring definitive or temporary tracheotomy are frequently difficult to ventilate and intubate. MATERIALS AND METHODS: We describe a new procedure to perform tracheotomy easily and safely in patients with major obstruction of the upper airway. A catheter, specifically designed for cricothyroidotomy, was inserted into the trachea under local anesthesia. Then, general anesthesia was induced and the catheter was used as a guide for dilatation tracheotomy. From November 2009 to March 2013, the procedure was successfully used in 13 consecutive patients. Twelve out of 13 patients presented severe inspiratory dyspnea and stridor. RESULTS: During and after the procedure, no complications were reported. CONCLUSIONS: The reported technique is quickly performed and is a safe way to ensure short and long time ventilation of patients with major obstruction of the upper airway.

Pitfalls in preoperative work-up of parotid gland tumours: 10-year series.

PROBLEMS/OBJECTIVES: Preoperative fine-needle aspiration cytology (FNAC) and magnetic resonance imaging (MRI) are the two most widely accepted diagnostic techniques used for the assessment of parotid gland tumours. We retrospectively evaluated the ability of FNAC and MRI to predict malignancy in parotid gland tumours. METHODOLOGY: Over a period of 10 years (2002-2011), parotidectomy for primary parotid gland tumours was performed in a consecutive series of 178 patients. Preoperative MRI was performed in 75% (133/178) of cases, and preoperative FNAC was performed in 70% of cases (124/178). Both modalities were applied in 53% (94/178) of patients. Sensitivity, specificity, and accuracy were analyzed retrospectively for each subgroup of patients. RESULTS: The sensitivity, specificity, and accuracy for predicting malignancy were 45%, 89%, and 84%, respectively, for FNAC (including only diagnostic cytology), and 40%, 88%, and 81%, respectively, for MRI. In the subgroup of patients who underwent both MRI and FNAC, sensitivity, specificity, and accuracy were 50%, 85%, and 80%, respectively. Preoperative MRI values improved significantly after introduction of diffusion-weighted (DW) acquisition in 2007 (71%, 93%, and 91%, respectively). CONCLUSIONS: Compared to previously published results, the high number of nondiagnostic smears and the low sensitivity rates in our series were disappointing, In part, this can be explained by the low percentage of malignant tumours and the high number of low-grade tumours among these. We discuss possibilities for improving preoperative performance, such as ultrasound-guided FNAC.

Quality assurance in head and neck surgical oncology: EORTC 24954 trial on larynx preservation.

BACKGROUND: The Head and Neck Cancer Group (HNCG) of the EORTC conducted a quality assurance program in the EORTC 24954 trial on larynx preservation. In this multicentre study, patients with resectable advanced squamous cell carcinoma of the larynx or hypopharynx were randomly assigned for treatment with sequential or alternating chemoradiation. The need for a quality assurance program is the evaluation and prevention of differences in treatments between centres in this multidisciplinary study. METHOD: The surgical subcommittee of the HNCG prepared a questionnaire, and clinical records of all patients were verified during audits of independent teams. Data relating institutional practices were collected during a face to face interview with members of the local team. RESULTS: 271 clinical records from the nine main contributing centres were reviewed. The main difference between centres was the time interval between first consultation and treatment initiation, with a mean of 45 days. On the pathology report the nodal involvement was described by level in 36% of the cases according to the American Academy of Otolaryngology-Head and Neck Surgery classification. Extranodal spread was not always described in neck dissection specimens. CONCLUSION: The EORTC 24954 trial on larynx preservation was the first prospective trial with a quality assurance program in head and neck surgical oncology. The analysis shows similarities in practices, but also points out some important differences between centres. Operation reports were fairly complete, but uniformity in pathology reports should be improved.

Tumour response and safety of cetuximab in a window pre-operative study in patients with squamous cell carcinoma of the head and neck.

BACKGROUND: To investigate the safety and activity of cetuximab in the pre-operative treatment of squamous cell carcinoma of the head and neck (SCCHN). PATIENTS AND METHODS: Cetuximab was administered for 2 weeks before surgery to 33 treatment-naïve patients selected for primary surgical treatment. Tumour biopsies, 2-[fluorine-18]-fluoro-2-deoxy-d-glucose positron emission tomography ((18)FDG-PET) and imaging were carried out at baseline and before surgery. The primary aim of the study was safety and the secondary aims included metabolical, radiological and pathological tumour response. Five untreated patients were included as controls. RESULTS: Cetuximab given 24 h before surgery was safe. Ninety percent of patients had (18)FDG-PET partial response (EORTC guideline) in the cetuximab group versus 0% in the control group. Delta maximal standardized uptake values (ΔSUVmax) were correlated with tumour cellularity on the surgical specimens (P < 0.0001). For patients with ΔSUVmax less than -25% or less than -50%, Ki67 was significantly decreased by cetuximab (P = 0.01 and 0.003). Cetuximab induced down-regulation of pEGFR (P = 0.0004) and pERK (P = 0.003). CONCLUSIONS: Short-course pre-operative administration of cetuximab is safe and shows a high rate of (18)FDG-PET response. (18)FDG-PET response was correlated with residual tumour cellularity suggesting that (18)FDG-PET deserves further investigation as a potential early marker of cetuximab activity in SCCHN.

Seeding of the percutaneous endoscopic gastrostomy site from head and neck carcinoma: case report and review of the literature.

BACKGROUND: Percutaneous endoscopic gastrostomy (PEG) is a relatively safe procedure and is an important supportive treatment for patients with advanced head and neck cancer. Although tumor seeding has been reported in various sites, seeding at the PEG exit site is a rare complication. METHODS AND RESULTS: We describe a clinical case in which squamous cell carcinoma of the hypopharynx implanted at the site of PEG insertion and was successfully removed by surgery. PEG was previously placed by the "pull" technique. A review of the literature, discussion of the mechanism of spread, and recommendations to avoid this complication are discussed. CONCLUSIONS: To avoid this rare and poor prognostic complication, the "pull" technique should be avoided for PEG placement in any patient with head and neck squamous cell carcinoma. An alternative method such as the "push" technique should be preferred.

Prevalence and spectrum of SDHx mutations in pheochromocytoma and paraganglioma in patients from Belgium: an update.

Since the early 2000s, the prevalence and spectrum of mutations in genes encoding subunits of succinate dehydrogenase (SDHx) were reported in large cohorts of patients with pheochromocytoma (PC) and paraganglioma (PGL) from most Western countries. Unfortunately, in Belgium, no equivalent work was performed thus far. Therefore, the aim of the work was to look for mutations in SDHx genes and genotype-phenotype correlations in patients with PC and/or PGL from Belgium. Screening of the coding parts of SDHx genes and deletion search were performed in all patients with PC and/or PGL referred to the -Cliniques Universitaires Saint-Luc from 05/2003 to 05/2011. Genetic screening was performed in 59 unrelated head and neck (hn)PGLs (8 fami-lial) and 53 PCs (7 extra-adrenal; 3 metastatic). In hnPGLs, 10 different SDHD mutations (3 substitutions, 5 deletions, 2 splice site mutations) were detected in 16 patients, including 7 familial cases and 9 apparently sporadic cases. In the same subset, we found 8 different SDHB mutations (5 substitutions, 1 splice site mutation, 1 deletion, 1 duplication) in 10 patients with sporadic hnPGL without evidence of malignancy. No SDHx mutation was detected in patients harboring PCs and no SDHC mutation whatsoever. In conclusion, in our multicentric database of PC-PGLs from Belgium, (i) the prevalence of SDHx mutations was high in hnPGLs (44% in the whole subset, 37% of apparently sporadic cases); (ii) in sporadic cases, the prevalence of SDHB mutations was high (20%), similar to that of SDHD (18%); and (iii) no SDHx mutation was found in a subset of mostly adrenal, benign PCs.

Phase II study of figitumumab in patients with recurrent and/or metastatic squamous cell carcinoma of the head and neck: clinical activity and molecular response (GORTEC 2008-02).

BACKGROUND: Preclinical studies suggest that insulin-like growth factor-1 receptor (IGF-1R) blockage could be a promising therapeutic target in squamous cell carcinoma of the head and neck (SCCHN). Therefore, we investigated the efficacy and toxicity of figitumumab, an anti-IGF-1R monoclonal antibody, in palliative SCCHN. PATIENTS AND METHODS: Patients with palliative SCCHN progressing after platinum-based therapy were treated with figitumumab i.v. 20 mg/kg, every 3 weeks. The primary end point was the disease control rate at 6-8 weeks after treatment initiation. Tumor biopsies and plasma samples were collected before and after figitumumab administration to monitor the molecular response. RESULTS: Seventeen patients were included. Only two patients achieved stable disease at 6-8 weeks. Median overall survival and progression-free survival were 63 and 52 days, respectively. The main grade 3-4 adverse event was hyperglycemia (41%). Translational research showed that figitumumab downregulated IGF-1R at the surface of tumor cells with activation of the epidermal growth factor receptor (EGFR) pathway, as shown by the upregulation of p-EGFR in tumor cells (P=0.016), and an increase in the plasma level of tumor growth factor-alpha (P=0.006). CONCLUSION: Figitumumab monotherapy has no clinically significant activity in unselected palliative SCCHN.

Prognostic factors and assessment of staging systems for head and neck soft tissue sarcomas in adults.

OBJECTIVES: The primary objectives of this study were to analyse the outcome of patients diagnosed with head and neck soft tissue sarcomas (HNSTS) and to identify relevant prognostic factors. As well as this, we compared the prognostic value of two staging systems proposed by the American Joint Committee on Cancer (AJCC) and the Memorial Sloan-Kettering Cancer Center (MSKCC). METHODS: From 07/1988 to 01/2008, the charts of 42 adult patients were retrospectively reviewed. Potential prognostic factors were analysed according to overall survival (OS), disease-free survival (DFS) and disease-specific survival (DSS). RESULTS: At 5 years, OS was 57%, DFS 47% and DSS 72%. On univariate analysis, statistically significant prognostic factors were for OS, distant or lymph node metastasis at diagnosis (p=0.032), for DFS, margins after surgery (p=0.007), for DSS, regional or distant metastasis at diagnosis (p=0.002), initial AJCC and MSKCC stage (p=0.018 and p=0.048) and margins after surgery (p=0.042). On multivariate analysis, margins remained statistically significant for DFS (p=0.039) when there was a trend with the initial AJCC stage (p=0.054) for OS. The AJCC staging system was of more prognostic value than the MSKCC staging system. CONCLUSIONS: Achieving clear margins after surgery is vital for improved local control and the best chance of survival. Adjuvant chemotherapy and radiotherapy were not shown to provide additional benefit. To better identify prognostic factors, it seems essential to set up national and international databases allowing multicenter registration for those patients.

[Neck dissection following chemoradiation for node positive head and neck carcinomas]. / Place du curage ganglionnaire après chimioradiothérapie dans les carcinomes épidermoïdes des voies aérodigestives supérieures avec atteinte ganglionnaire initiale (nasopharynx exclu).

The optimal timing and extent of neck dissection in the context of chemoradiation for head and neck cancer remains controversial. For some institutions, it is uncertain whether neck dissection should still be performed upfront especially for cystic nodes. For others, neck dissection can be performed after chemoradiation and can be omitted for N1 disease as long as a complete response to chemoradiation is obtained. The question is debated for N2 and N3 disease even after a complete response as the correlation between radiological and clinical assessment and pathology may not be reliable. Response rates are greater than or equal to 60% and isolated neck failures are less than or equal to 10% with current chemoradiation protocols. Some therefore consider that systematic upfront or planned neck dissection would lead to greater than or equal to 50% unnecessary neck dissections for N2-N3 disease. Positron-emission tomography (PET) scanning to assess treatment response and have shown a very high negative predictive value of greater than or equal to 95% when using a standard uptake value of 3 for patients with a negative PET at four months after the completion of therapy. These data may support the practice of observing PET-negative necks. More evidence-based data are awaited to assess the need for neck dissection on PET. Selective neck dissection based on radiological assessment and peroperative findings and not exclusively on initial nodal stage may help to limit morbidity and to improve the quality of life without increasing the risk of neck failure. Adjuvant regional radiation boosts might be discussed on an individual basis for aggressive residual nodal disease with extracapsular spread and uncertain margins but evidence is missing. Medical treatments aiming at reducing the metastatic risk especially for N3 disease are to be evaluated.

Solitary fibrous tumour of the soft tissue of the face: a case report.

INTRODUCTION: Solitary fibrous tumour (SFT) is a rare mesenchymal neoplasm described first in the pleura. Recently, SFTs have been found in various extra-serosal locations, including the head and neck region. CASE PRESENTATION: We report a case of SFT originating from the periorbital region of the face. Preoperative cytological examination by fine needle aspiration biopsy diagnosed a mesenchymal tumour. The patient underwent surgical resection. The mass was completely resected. Definitive histopathologic and immunohistologic examination confirmed the diagnosis of SFT. DISCUSSION: The rare localisation in extrapleural sites and the multiplicity of histological patterns can explain the difficulty in arriving at a definitive diagnosis in SFT. Usually, SFT is a benign tumour, although malignant variants exist. Clinical behaviour is unpredictable and recurrence or malignant transformation can also occur, especially in cases with macroscopically or microscopically invaded margins. Complete surgical excision and long follow-up is therefore always recommended. The recent increase in reports of extrapleural SFT indicates that this rare tumour should be included in the differential diagnosis of soft tissue head and neck tumours.

Cancer of the nasal vestibule, nasal cavity and paranasal sinuses.

The usual clinical presentation of sinonasal tumours includes symptoms that are indistinguishable from inflammatory sinus disease, namely nasal airway obstruction, pain, and epistaxis. Abnormal V1 and/or V2 sensations are a strong indication of the possibility of tumour. Computed tomography is the most reliable and informative imaging tool for evaluating the cancers of the paranasal sinuses. Magnetic resonance imaging is essential for tumour mapping because of the excellent tissue characterisation and the possibility of differentiating between neoplasms and retained secretions. A wide variety of histologies may be encountered, although squamous cell carcinoma (SCCA) is the most common. Radiation is a common adjuvant to surgery. The response of sinonasal tract tumours to radiation therapy varies with the stage and histology of the tumour. Rehabilitation after surgical resection may be accomplished with prosthodontics or reconstructive flaps. Bony erosion of the orbital walls does not constitute an indication for orbital exenteration. Patients with tumour involvement of the skull base, either in the infratemporal fossa or at the fovea ethmoidalis and cribriform plate, should be considered for craniofacial resection. Management of these tumours requires a multimodal approach, involving surgery, radiation therapy and, increasingly in recent years, chemotherapy. Management should therefore be entrusted to multidisciplinary teams only.

Initial assessment of a neck mass in children.

OBJECTIVE: To propose national recommendations for the initial assessment of a mass in the neck in children. METHODS: Comprehensive review of the available literature and consensus discussion with national experts in the field. RESULTS: Consensus guidelines are proposed concerning the work up of children presenting with a mass in the neck.

Initial work-up in head and neck squamous cell carcinoma.

OBJECTIVE: To propose national guidelines for the initial assessment of head and neck squamous cell carcinoma. METHODS: Comprehensive review of the literature and consensus discussion with national experts in the field. RESULTS: Consensus guidelines are proposed concerning the work-up of patients with a presumed diagnosis of a squamous cell carcinoma of the upper aero-digestive tract.

Management of neck masses in adults.

Initial management of a neck mass in adults is a frequently encountered problem in ENT practice. The differential diagnosis with regards to clinical presentation, localization, imaging studies and cytology and/or histology is reviewed. An algorithm is provided to help the practioner.

Carotid stenting for impending carotid blowout: suitable supportive care for head and neck cancer patients?

BACKGROUND: Carotid blowout (CB) represents a dramatic end-of-life situation for palliative head and neck cancer patients, their relatives and caregivers. Recently, endovascular therapy has been proposed for head and neck surgical patients. Preliminary reports showed a better outcome with less morbidity compared to the previous treatment modalities. However, the specific use of such techniques for palliative head and neck cancer patients has not been previously described. METHOD: Retrospective review of two cases of head and neck cancer patients receiving palliative care, presenting with a CB, managed with endovascular stent placement. RESULTS: Bleeding was effectively stopped by the procedure in both cases. Both patients developed a post-procedure thromboembolism, which was immediately treated by appropriate anticoagulation therapy. Neurological symptoms resolved within 24 hours allowing rapid hospital discharge. One patient died at home seven months later. The second patient is alive five months after the procedure. No recurrence of CB occurred in either patient. CONCLUSIONS: Endovascular stent placement for CB allows a rapid arrest of bleeding and permits the use of anticoagulation therapy in order to avoid long-term neurological injury. In our view, carotid stenting should be considered as valid supportive care for palliative head and neck patients presenting with a CB.

The role of nasal cavity disinfection in the bacteriology of chronic sinusitis.

OBJECTIVES: Several factors may influence the results of bacteriological studies in chronic rhinosinusitis. We investigated the potential role of nasal cavity disinfection in the bacteriology of the bulla ethmoidalis in patients suffering from chronic sinusitis. MATERIAL AND METHODS: Bacteriology of the bulla ethmoidalis was studied in 176 consecutive adult patients presenting a chronic sinusitis refractory to standard medical treatment who underwent functional endoscopic sinus surgery. Two different techniques were used: (A) a technique with nasal vestibule and facial disinfection with chlorhexedin (N = 89 patients and 165 samples) vs. (B) a technique with facial, nasal vestibule and nasal cavity disinfection with a povidone-iodine solution followed by a cleansing of the nasal cavity (N = 87 patients and 166 samples). RESULTS: Culture rate was 89.6% (183 bacterial isolates) for technique (A) vs 76.5% (164 bacterial isolates) for technique (B) (p < 0.001). Major bacteria encountered in the (A) group and in the (B) group were respectively: Coagulase Negative Staphylococcus: 77 vs 40 isolates (p < 0.001); Coagulase positive Staphylococcus: 44 vs 30 isolates (p = 0.061); Streptococcus pneumoniae: 4 vs 5 isolates; Others: Streptococcus sp.: 12 vs 16 isolates; Haemophilus influenzae: 8 vs 6 isolates; Enterobacteriacea: 33 vs 53 isolates (p = 0.013) and others Gram Negative Bacilli: 3 vs 7 isolates. CONCLUSIONS: The standard (A) technique to study the bacteriology of the bulla ethmoidalis in patients with chronic sinusitis yielded a higher percentage of positive culture and of bacterial isolates than a more advanced (B) technique. This is mainly due to the higher percentage of contaminant bacteria such as Coagulase Negative Staphylococcus recovered with the standard technique. Enterobacteriacea and others Gram Negative Bacilli were more often encountered into the bulla ethmoidalis with the technique where disinfection of the nasal cavity was performed.

Post-transplant lymphoproliferative disorder with supraglottic involvement.

OBJECTIVE: Transplant patients with primary Epstein-Barr virus (EBV) infection may develop post-transplant lymphoproliferative disorder (PTLD). Since many infants are seronegative at the time of transplantation, PTLD is a major concern for paediatric transplant centres. First manifestations of PTLD are frequently observed in the ENT area with adenoidal and/or tonsillar involvement. DESIGN: Retrospective study of two cases of PTLD with confirmed supraglottic involvement, their management and outcome. Only patients with pathologically and immunologically demonstrated B-cell proliferation were diagnosed as PTLD. RESULT: Two infants developed an acute stridor during PTLD respectively 8 and 10 months after orthotopic liver transplantation (OLT). These infants were seronegative for EBV at the time of transplantation. IgM anti-EBV and/or detection of EBV genome by polymerase chain reaction were positive. Laryngeal examination revealed hypopharyngeal and/or supraglottic mucosal hyperplasia. Immunostaining of laryngeal biopsy was positive for latent membrane protein-1 (LMP1). Patients were treated by a reduction in immunosuppression as far as tolerated with the intent to recover natural immune response by the patient over the proliferation of EBV-infected cells. Complete remission of PTLD was observed in these two cases. CONCLUSION: Tonsillar hypertrophy and adenoid enlargement are the most encountered features of PTLD in OLT occurring in the ENT area. Acute stridor with supraglottic involvement may also be observed in PTLD and must be promptly diagnosed as the prognosis of this disorder is related to rapid reduction in immunosuppression and consequently to the recovering of a natural immune response against the EBV infection.

Nasal obstruction and its impact on sleep-related breathing disorders.

Upper airway patency is essential during sleep in order to avoid sleep-related breathing disorders (SRBD). Nasal obstruction may have a negative impact on sleep quality and must be considered to be a co-factor in the pathophysiology of SRBD. In this paper we will discuss the relation between nasal physiology at night and sleep quality and the possible mechanisms between nasal obstruction and obstructive sleep apnea-hypopnea syndrome (OSAS). We will review the effect of the relief of nasal obstruction (with nasal dilators, medication and/or surgery) on SRBD. Also an algorithm on the management of OSAS patients when nasal surgery is indicated will be proposed.