Wnt-pathway activation in intestinal-type sinonasal adenocarcinoma.

BACKGROUND: Intestinal-type sinonasal adenocarcinoma (ITAC) is an epithelial cancer of the sinonasal sinuses that shows histological similarity to colorectal cancer (CRC) and share chronic inflammation as a possible etiological factor. The Wnt-pathway is one of the most important tumourigenic pathways in CRC. The aim of this study was to investigate if the Wnt-pathway is activated in ITAC. METHODOLOGY: Protein expression profiles of E-cadherin, ß-catenin, c-myc and cyclin D1 were analysed by immunohistochemistry in 83 samples of ITAC, organized into tissue microarray blocks. RESULTS: Nuclear ß-catenin expression was observed in 31% of the cases and was twice as frequent in papillary/colonic ITAC compared to solid/mucinous subtypes. Loss of membranous ß-catenin staining occurred in 24% and loss of membranous E-cadherin in 6% of the cases and this was more prominent in mucinous types. Strong c-myc and cyclin D1 expression was observed in 30% and 4% of the cases, respectively. Nuclear ß-catenin expression was significantly related to poor clinical outcome, independent from established factors as tumour stage and histological type. CONCLUSION: The presence of nuclear ß-catenin in 31% of patients with ITACs indicated that in a subset of patients, the Wnt-pathway is active and conveys a worse prognosis.

Results of endoscopic nasal surgery in the treatment of invasive fungal sinusitis in children with cancer and immunosuppression. / Resultados de cirugía endoscópica nasal en el tratamiento de sinusitis fúngica invasiva en niños con cáncer e inmunosupresión.

BACKGROUND AND OBJECTIVE: to describe the results of the treatment of invasive fungal sinusitis with nasal endoscopic surgery in an immunocompromised paediatric oncological population. METHODS: retrospective study of all patients diagnosed with invasive fungal sinusitis operated in the National Paediatric Oncology Unit between 2012 and 2016. Data taken from their medical history included: epidemiological characteristics, oncological diagnosis, haematological data, symptoms, tomographic studies, surgical interventions, results of pathology and cultures, medications received, complications, evolution and survival. RESULTS: 18 patients were identified, 7 male and 11 female. The average age was 12 years, 13 had a diagnosis of acute lymphocytic leukemia and 5 of acute myeloid leukemia. Seventeen patients presented severe neutropenia at the time of diagnosis. The most frequently identified aetiological agent was Aspergillus in 13 patients. In 16 patients (89%) the disease was controlled with nasal endoscopic surgery. Ten patients died due to unrelated causes throughout the study. DISCUSSION AND CONCLUSIONS: Invasive fungal sinusitis should be considered a medical emergency due to its high mortality. The diagnosis is based on a high index of suspicion in patients with predisposing factors (leukaemia, neutropenia, persistent fever, nasogastric tube) and endoscopic nasal evaluation. Antifungal medical treatment and aggressive nasal endoscopic surgery is indicated regardless of the patient's condition to reduce the fungal burden and associated high mortality. The treatment must be provided by a multidisciplinary team that includes paediatrics, haemato-oncology, infectology and otorhinolaryngology.

Nasopharyngeal Angiofibroma: A Clinical, Histopathological and Immunohistochemical Study of 42 Cases with Emphasis on Stromal Features.

Nasopharyngeal angiofibroma is a benign but aggressive tumor of unknown etiology, typically occurring in adolescent males. It is described as a rare neoplasm; however, the prevalence seems to have geographic differences. All cases referred to our head and neck clinical and pathology service were reviewed. Most of the patients presented at an advanced stage. The clinical and radiographic features are presented and discussed. Histologically, the tumor shows a highly vascular fibrous proliferation with characteristic plump, angulated and stellate cells, categorized as fibroblasts. Immunohistochemistry was performed on 42 cases to further elucidate the nature of these cells. The stromal cells expressed vimentin and factor XIIIa, the latter expressed most commonly in the giant stellate cells. Inflammation was almost exclusively present in peripheral subepithelial areas. Mast cells were abundant, even in the absence of other inflammatory cells. Lymphatics were observed principally in peripheral regions. Proliferating cells (Ki-67 reactive) were restricted to endothelial cells.

[Neurolaryngology]. / Neurolaringología.

The neuroanatomy of voice and speech is complex. An intricate neural network is responsible for ensuring the main functions of the larynx: airway protection, cough and Valsalva production, and providing voice. Coordination of these roles is very susceptible to disruption by neurological disorders. Neurological disorders that affect laryngeal function include Parkinson's disease, stroke, amyotrophic lateral sclerosis, multiple sclerosis, dystonia and essential tremor. A thorough neurological evaluation should be routine for any patient presenting with voice complaints suggestive of neurogenic cause. Endoscopic visualisation of the larynx using a dynamic voice assessment with a flexible laryngoscope is a crucial part of the evaluation and ancillary tests are sometimes performed. Otolaryngologic evaluation is important in the diagnosis and treatment of neurological disorders that affect laryngeal function.

[The effect of anchor voices and visible speech in training in the GRABS scale of perceptual evaluation of dysphonia]. / El espectrograma de banda estrecha como ayuda para el aprendizaje del método GRABS de análisis perceptual de la disfonía.

INTRODUCTION: Perceptual evaluation of voice quality remains a key standard for judgment of vocal impairment. The GRABS method has become a commonly-used scale for rating severity of dysphonia, but it has no published, standardised protocol to follow. Training is important for reaching good interrater agreement for its parameters; however, the references most often cited for the GRABS provide no guidelines for clinical administration, speech material or rating calibration. This study investigated the effect of anchors (standard reference voices) and visible speech (narrow band spectrogram) in training non-expert professionals in the GRABS method. MATERIAL & METHODS: Four inexperienced listeners evaluated 107 recorded pathological voices using the GRABS scale in 2 separate sessions; at first, without a visible spectrogram and then, 6 months later, with anchors and a narrow band spectrogram as additional information. RESULTS: The results show that anchors and visible speech helped to improve the reliability of G, B, A and S parameters. Interrater agreement according to k statistics was significantly stronger with the addition of spectrographic information for rating breathiness and strain. DISCUSSION: This study found that non-expert listeners showed significant improvement after training with external anchors (standard reference voices) and a narrow band spectrogram.

Functional and oncological results of non-surgical vs surgical treatment in squamous cell carcinomas of the oropharynx.

INTRODUCTION AND OBJECTIVES: Squamous cell carcinomas of the oropharynx are aggressive tumours usually diagnosed at advanced stage. Their optimal treatment has not been established. The aim of this study was to compare the oncological and functional outcomes in patients with carcinomas of the oropharynx treated by radiotherapy (with chemotherapy in advanced stages) vs surgery (with radiotherapy in advanced stages). METHODS: A retrospective study on 50 patients with squamous cell cancer of the oropharynx treated by radiotherapy (with or without chemotherapy) at our institution between 1998 and 2008 was carried out. The oncological and functional results were compared with patients with same cancer location and stage treated by surgery (with or without radiotherapy). In both groups, the patients were classified as follows: 10% Stage I, 12% Stage II, 16% Stage III, 48% Stage IVa and 14% Stage IVb. RESULTS: The 5-year disease-specific survival was 33% in the radiotherapy group and 52% in the surgical group (P=.17). Five-year disease-specific survival for Stage I and II patients was 82% in the radiotherapy group and 70% in the surgical group. In Stage III and IV disease, 5-year disease-specific survival was higher in the surgical group (47% vs 17%). The functional results were similar; anatomical and functional preservation of the larynx was higher in the radiotherapy group but the successful return to oral food intake was higher in the surgical group. CONCLUSIONS: The prognosis of squamous cell carcinoma of the oropharynx is poor. Oncological results in Stages I and II were similar for radiotherapy and surgical treatments. In advanced stages, the prognosis was better in patients treated by surgery with or without radiotherapy. Functional results were similar in both treatment modalities.

Oncologic and functional results after transhyoid surgical approach for cancer of the base of tongue.

BACKGROUND: The optimal treatment for base of tongue cancer remains unclear, especially in advanced stages. METHODS: We retrospectively review 84 previously untreated patients that underwent a transhyoid resection of a base of tongue carcinoma. Sixty-four patients (76%) underwent postoperative radiotherapy. RESULTS: Five patients had stage II disease, 6 had stage III, 58 had stage IVA, and 15 had stage IVB. The overall recurrence rate was 68%. Five-year disease-specific survival rates by stage were 100%, 67%, 27%, and 8% for stage II to IVB, respectively (p = .0007). Multivariate analysis showed that the presence of lymph node metastases was an independent predictor of reduced disease-specific survival rates (p = .02). All patients maintained an intelligible voice, and oral alimentation was successfully recovered in 97.5% of them. CONCLUSIONS: The transhyoid approach allowed adequate resection of base of tongue cancers with low morbidity and acceptable functional results, but the oncologic outcomes in advanced stages are poor. Head Neck, 2011.

[Surgical treatment of head and neck chondrosarcomas]. / Tratamiento quirúrgico de los condrosarcomas de cabeza y cuello.

INTRODUCTION: Head and neck chondrosarcomas may adopt different locations and biological behaviour. MATERIAL AND METHOD: We present a retrospective clinical series of 17 chondrosarcomas surgically treated in our Department from 1977 until 2006. RESULTS: Chondrosarcomas were located in the nasosinusal area (n=6), larynx (n=5), petrous bone (n=3), atlas (n=1), parapharyngeal space (n=1) and trachea (n=1). All patients except for one underwent surgery with radical intention. The mean follow-up period was 84 months (median, 71 months). Six patients developed recurrent disease with a mean latency of 10 months. Two patients died due to the disease and two remained alive with evidence of tumour. Patients with grade I chondrosarcomas presented less recurrent disease than those with grade II or III chondrosarcomas (17% versus 80%, P=0.029). The estimated 5-year survival was 88% with the better survival of patients with grade I chondrosarcomas reaching statistical significance (P=0.023). In 2 patients with cricoid chondrosarcomas, the reconstruction was carried out using the Rethi-Ward technique, and they were without evidence of disease at 71 months (with no cannula) and 145 months (with cannula). Chondrosarcomas of the jugular foramen were treated using a modified infratemporal type A approach. CONCLUSION: Low grade head and neck chondrosarcomas have a good prognosis while high grade chondrosarcomas tend to recur, despite radical surgical treatment.

[Oncologic and functional results of surgical treatment for base of tongue carcinomas]. / Resultados oncológicos y funcionales del tratamiento quirúrgico de los carcinomas de base de lengua.

INTRODUCTION AND OBJECTIVES: Squamous cell carcinomas at the base of the tongue are usually diagnosed at advanced stages, and their optimal treatment has not been established. The aim of this study was to describe the functional and oncological outcomes of patients with base of tongue carcinomas treated with surgery. MATERIALS AND METHODS: A retrospective study on 132 patients with base of tongue carcinoma surgically treated in our hospital between 1990 and 2007 was carried out. One patient (0.75%) was in stage I, 8 (6%) were in stage II, 15 (11%) in stage III, 91 (69%) in stage IVA, and 17 (13%) with stage IVB. A total of 92 (70%) patients received postoperative radiotherapy. RESULTS: Eighty-seven patients (66%) had recurrence: 23 patients (17%) had local recurrence, 15 (11%) regional, 20 (15%) locoregional, 16 (12%) locoregional and distant, and 9 (7%) distant metastases. The disease-specific survival was 34% at 5 years (100% for stages I and II, 44% for stage III, 28% for stage IVA and 12% for IVB; p=0.0004). Multivariate analysis showed two variables independently associated with worse survival: lymph node metastases classified as N2-3 (p=0.016) and primary tumours classified as T3-4 (p=0.040). Adequate oral intake was achieved by 96% of the patients and 79% could be decannulated. CONCLUSIONS: The prognosis of squamous cell carcinomas of the base of the tongue is poor, especially in advanced stages. Surgical treatment provides oncological and functional results similar to other therapeutic modalities.