Urs Borner

Diagnostic value of biopsies in identifying cytoplasmic antineutrophil cytoplasmic antibody-negative localized Wegener's granulomatosis presenting primarily with sinonasal disease.

Background A substantial proportion of Wegeners disease (WG) patients present with localized disease of the upper airways, i.e., sinonasal and other ear/nose/throat (ENT) symptoms. Because of the oligosymptomatic presentation a timely diagnosis of this potentially fatal disease is challenging. This study evaluates diagnostic peculiarities between WG in its localized and generalized form of the disease. Methods Retrospective analysis was performed of 82 patients with suspected WG manifesting in the ENT region between 1989 and 2009. Comparison was performed of the clinical and laboratory results between patients with localized (n = 15) and generalized stage (n = 16) as well as non-WG patients (n = 50). Results ENT signs and symptoms were subtle, especially in the population presenting with localized disease. Therapy refractory rhinosinusitis or serous otitis media were the most frequent presentations of WG. In testing for localized WG, mucosal biopsy had the highest sensitivity (53%) compared with cytoplasmic antineutrophil cytoplasmic antibodies (c-ANCAs) with a lower sensitivity (47%) but highest specificity (96%) and highest positive predictive value (PPV; 78% versus 73%). Patients with generalized WG typically revealed a pathological urine sediment, hemoptysis, or rheumatological symptoms. In the generalized stage, c-ANCA had the highest sensitivity (81%), specificity (96% versus 95%), and highest PPV (87%). Conclusion Timely diagnosis and treatment of localized WG limited to the ENT region remains problematic. Even with adequate therapy, nearly one-half of patients with sinonasal localization suffer from relapse, at least 1 in 10 will progress to generalized disease, and up to two-thirds may develop permanent tissue damage. Unfortunately, the diagnostic usefulness of c-ANCA is significantly reduced at this early stage compared with cases with generalized disease (p = 0.04). Hence, the relative diagnostic value of mucosal biopsy increases especially for the significant proportion of c-ANCA– localized WG patients (47%). Sinonasal tissue sampling represents a cornerstone for diagnosis, which unlike c-ANCA testing can be repeated in short intervals and is associated with low morbidity.

Profiling invasiveness in head and neck cancer: recent contributions of genomic and transcriptomic approaches.

High-throughput molecular profiling approaches have emerged as precious research tools in the field of head and neck translational oncology. Such approaches have identified and/or confirmed the role of several genes or pathways in the acquisition/maintenance of an invasive phenotype and the execution of cellular programs related to cell invasion. Recently published new-generation sequencing studies in head and neck squamous cell carcinoma (HNSCC) have unveiled prominent roles in carcinogenesis and cell invasion of mutations involving NOTCH1 and PI3K-patwhay components. Gene-expression profiling studies combined with systems biology approaches have allowed identifying and gaining further mechanistic understanding into pathways commonly enriched in invasive HNSCC. These pathways include antigen-presenting and leucocyte adhesion molecules, as well as genes involved in cell-extracellular matrix interactions. Here we review the major insights into invasiveness in head and neck cancer provided by high-throughput molecular profiling approaches.

Implications of intraglandular lymph node metastases in primary carcinomas of the parotid gland

Assess the diagnostic and prognostic relevance of intraglandular lymph node (IGLN) metastases in primary parotid gland carcinomas (PGCs).

Recurrent parotid gland carcinoma: how effective is salvage surgery?

ObjectiveRecurrent parotid gland carcinomas (PGCs) are poorly characterized and studies focusing on this topic are rare due to their low incidence. The goal of this study is to analyze the therapeutic strategies, prognostic factors, and oncological outcomes of a series of patients with recurrent PGCs.Patients and MethodsRetrospective chart review (1997–2012) of patients with recurrent PGCs was initially treated with curative intent.ResultsWe identified 20 patients with recurrent PGCs. Eleven patients presented isolated local, regional, or distant metastases, while the rest had recurrences in multiple sites. Recurrent tumors tended to present more advanced T-stage (p = 0.01) and overall stage (p < 0.001), but not N-stage (p = 0.74) when compared to the initial tumors. Half the patients (50%) had distant metastases at the moment of recurrence diagnosis, and another three developed them after attempted salvage surgery. Only 8/20 patients with isolated local or regional recurrences were surgically salvaged with extended revision parotidectomy and neck dissection, respectively. The remaining 12 patients were managed on palliative basis. Overall survival (31.70 months vs. 20.73 months) and progression-free survival (28.70 months vs. 13.61 months) were not significantly different in patients managed surgically vs. palliatively.ConclusionRecurrent PGCs are aggressive neoplasms with a high rate of distant metastases. Surgical salvage can be considered in patients with limited local and/or regional recurrences. The alternative to surgical salvage is palliative management with different chemotherapeutic regimens. Survival does not differ between the two strategies in the present series.

Bilateral lingual artery stenosis: A rare, late complication of chemoradiotherapy

INTRODUCTION Carotid artery stenosis following radiotherapy (RT) is a known risk factor for the development of cerebrovascular disease with a risk of subsequent stroke or transient ischaemic attack. In contrast, small vessel disease in the neck following RT has been more rarely described. CASE REPORT The authors report the case of a 61-year-old man who developed partial lingual necrosis 4 years after surgery and postoperative chemoradiotherapy for squamous cell carcinoma of the floor of the mouth. Contrast-enhanced CT scan confirmed subtotal to total occlusion of both lingual arteries. Surgical debridement of the necrosis allowed complete cure of the lesions. DISCUSSION Small vessel disease is a possible complication in patients treated by RT for head and neck cancer. Although the risk of these complications is not directly related to the total radiation dose, higher doses appear to accelerate the development of vascular lesions. Practitioners must be aware of the possibility of these complications, especially in patients surviving more than 5 years.

Epiglottic cyst: rare, but potentially dangerous

A 34-year-old woman presented with a foreign body feeling and slight sore throat without dysphagia or dyspnoea. The symptoms had occurred a week earlier. About 2 years earlier, she had similar symptoms, which resolved spontaneously. Examination showed a round mass located on the …

Neurofibromatosis of the tongue.

45-year-old woman presented with a painless mass in the tongue that had grown gradually over Athe past 20 years (Figure, arrowheads). She had café-au-lait spots and previous neurofibroma resections. Neurofibromatosis type 1was also found inher father and two children. Recent speech problems made a resection necessary. Partial removal of the mass immediately improved communication. Pathological analysis showed plexiform neurofibroma without malignant transformation. Neurofibromatosis type 1 is an autosomal dominant disorder characterised by neurofibromas that can potentially affect every site of the body. Malignant transformation is rare and resection is indicated when functional or aesthetic impairment is associated.n