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Mucosal melanoma of the nasal cavity and paranasal sinuses is a rare disease, but its incidence appears to be increasing. The mean age at diagnosis is between 65 and 70 years. Unilateral nasal obstruction and epistaxis are the most common presenting complaints. Melanoma arises in the septum or lateral wall of the nasal cavity in the great majority of cases. The histological diagnosis is based on specific immunohistochemical labelling and is usually established at an advanced stage of disease: stage T3 or T4 tumours according to the 7th edition of the American Joint Committee on Cancer (AJCC) classification of tumours. First-line treatment consists of surgery. The place of intranasal endoscopic surgery remains controversial due to the difficulty of controlling surgical margins and should be reserved for experienced teams. Adjuvant radiotherapy is usually performed due to its efficacy on local and regional disease control. Five-year overall survival of mucosal melanoma of the nasal cavity and paranasal sinuses in the most recent series does not exceed 40%. Local recurrence is observed in about 50% of cases and metastatic disease is common. The quality of initial tumour resection with negative surgical margins is the most important prognostic factor for tumours confined to the nasal cavity. Hopes for improvement of survival are based on early diagnosis, progress in radiotherapy techniques and cell and gene therapy that are currently under evaluation.

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OBJECTIVES: Still today, Menière's disease (MD) can be definitively diagnosed only on post-mortem findings of endolymphatic hydrops. Otoacoustic emission (OAE) phase has been shown to be highly sensitive to intracranial pressure. Preliminary analysis of OAEs in MD patients indicated high sensitivity to slight variations in intracranial pressure. The principal objective of the present study was to confirm this specific sensitivity of OAEs in MD. PATIENTS AND METHODS: In a prospective study of 32 consecutive cases of acute MD seen in consultation or hospital, 20 patients (23 ears) underwent acoustic phase-shift test: i.e., seated vs. supine OAE phase centered around 1kHz, with results compared to controls. RESULTS: The acoustic phase-shift test was performed in 62.5% of acute patients (58.9% of affected ears). In the control group, the 95% confidence interval for phase shift was between -30° and +45°. Phase shift was significantly elevated, beyond the normal interval, in 18 of the MD patients: range, -80° to +145°. Sensitivity was 90%. Overall, in patients in whom transient evoked OAEs (TEOAEs) were present, positive predictive value was 100% and negative predictive value 92.3%. CONCLUSIONS: The acoustic phase-shift test proved useful and powerful in demonstrating pressure imbalance in acute Menière's disease.

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OBJECTIVES: To assess patient satisfaction with bone-anchored hearing aids (BAHA) and the role of preoperative audiometric testing. PATIENTS AND METHODS: A telephone satisfaction survey was conducted on all patients implanted between June 1, 2005 and February 1, 2008. Patients with unilateral total deafness underwent preoperative audiometric tests in quiet and in noise and stereoaudiometry with and without BAHA. Patients with a conductive hearing loss underwent preoperative audiometric tests in quiet and in noise and real-life testing at home using a headband. A standardized satisfaction questionnaire derived from the Entific BAHA questionnaire was used. RESULTS: Twenty-two out of 26 patients responded to the questionnaire. Ten patients were implanted for conductive hearing loss (CHL) and 12 for unilateral total deafness (UTD). Mean follow-up was 19 months in the UTD group and 21 months in the CHL group. Sixty-seven percent of UTD and 80% of CHL patients reported improved quality of life. The BAHA was worn for more than 4hours per day by 83% of UTD and 100% of CHL patients, and at least 5 days per week by 67% of UTD and 80% of CHL patients. CONCLUSION: BAHAs provided real benefit in all situations for CHL patients. In UTD, its benefit basically related to noisy environments. In UTD, satisfaction on preoperative stereoaudiometric testing in noise with and without BAHA was predictive of postimplantation satisfaction. In response to the question "Would you do it again?", 81% of patients answered "Yes".

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This article reviews the concept of selectivity in peritumoral microscopic disease to be included in the Clinical Target Volume (CTV) for elective treatment for larynx and hypopharynx squamous cell carcinoma (50 Gy or 54-60 Gy for SIB-IMRT), using the local tumoral spread. The objective of the present article is to present the different delineations of the target volumes, required for an appropriate application of 3-DCRT and IMRT (supraglottic larynx, vocal cord, subglottic larynx, pyriform sinus, lateral and posterior pharyngeal wall and postcricoid pharynx). These propositions are for the delineation of microscopic peritumoral target volumes when external beam irradiation is required. CTVs are illustrated on CT sections.

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OBJECTIVES: Lacrimal sac tumors are usually primary and of epithelial origin. Overall, 55% of lacrimal sac tumors are malignant. Mortality rates for malignant tumors depend on tumor stage and type, with a mean rate of 38%. The main objective of this study was to review our experience with these malignancies and to compare this with the published literature. PATIENTS AND METHODS: A 15-year retrospective review of all malignant lacrimal sac tumor patients was completed in our department. Seven patients were found to have malignant sac tumors. The mean age was 53 years (range, 32-74 years) with six women and one man. The patients' clinical records were reviewed for data regarding patient symptoms, preoperative investigations, pathology reports, treatment results, and outcome. These data were compared with the available literature. RESULTS: All patients presented with epiphora and a palpable lump of the internal canthus. In two cases, a history of recurrent dacryocystitis was noted. The histological types were as follows: squamous cell carcinoma (n=5), one case of adenoid cystic carcinoma, and one case of malignant lymphoma. Surgery was performed in six cases with additional radiotherapy in four. One patient was treated exclusively with external radiotherapy. The mean follow-up was 68 months (range, 6-204 months). Two patients developed recurrences: three were disease-free at the time of the study. CONCLUSIONS: Malignant epithelial lacrimal sac tumors are rare cancers with significant recurrence rates. Correct diagnosis and appropriate therapy require a multidisciplinary management approach. Treatment of these malignant epithelial tumors is first and foremost complete surgical removal with wide excision.

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OBJECTIVES: Sinonasal mucosal melanomas account for approximately 1% of all melanomas. These tumors are particularly aggressive, with a recurrence rate between 37 and 54% and a 5-year survival rate between 20 and 46%. Metastases are frequent. The main objective of this study was to analyze all of our cases of sinonasal mucosal melanomas and determine any prognostic factors. PATIENTS AND METHODS: All our cases of sinonasal mucosal melanoma over a period of 10 years were included. Ten cases were analyzed. The mean age was 71 years (range: 61-85 years) for seven women and three men. The clinical, radiological, anatomopathological, and surgical data as well as the characteristics of disease progression were analyzed. These data were compared to those reported in the literature. RESULTS: The mean follow-up was 36.3 months (range: 2-96 months). The 5-year overall survival was 40%. Seven patients developed local recurrences after a mean follow-up of 16 months (range: 2-27 months) with associated metastases in three cases. Analysis of the prognostic factors showed that tumors classified as T1 and limited to the nasal fossae had a better prognosis. CONCLUSION: Sinonasal mucosal melanomas are rare tumors with a high mortality rate. Treatment of these tumors requires extensive surgical treatment associated with external radiotherapy.

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OBJECTIVE: To determine whether surgical pitfalls can be anticipated through a preoperative CT-Scan in case of middle ear implantation with a semi-implantable middle ear ossicular stimulator from Otologics (SIMOS). A second outcome measure was to assess the auditory gain obtained in the series. MATERIALS AND METHODS: A retrospective analysis of 19 consecutive surgical procedure for a SIMOS implantations was achieved. Preoperative Ct-Scans were analyzed and several measurements of the mastoid were taken out from axial transverse views, ie. the distance between the incus and the cortical mastoid bone [d(i-c)], the distance between the sigmoid sinus and the incus [d(i-ss)], the distance between the sigmoid sinus and the cortical bone of the mastoid [d(c'-ss)]. On coronal images, the dura of the middle fossa was described as procident if it was lower than the head of malleus and/or body incus. Auditory gain was calculated on pure tone and speech audiometry with bisyllabic words. RESULTS: All cases were successfully implanted. d(i-c) was on average at 33.1 +/- 2.6 mm in cases easy to implant. One case of contracted mastoid was difficult and led to the impaction of the sigmoid sinus. In this case the distance d(i-c) was shorter at 25 mm than the average minus 2 standard deviations of the others (27.9 mm). Also d(ss-c') was lower at 7 mm than the average minus 2 standard deviations of the others (7.6 mm). Two other cases led to difficult surgical procedures because of a low middle fossa dura. This too low middle fossa dura could be identified on preoperative coronal CT-scans in these two cases. Audiometric gains were remarkable, with an average of 39 +/- 16 dB. In case of severe sensorineural hearing loss the average gain was higher at 46 +/- 9 dB. Two revision-procedures were required, one for a device failure, the second because of skin alteration due to systemic inflammatory disease requiring corticosteroids. Mean follow-up was 50 +/- 22 months. CONCLUSION: The SIMOS is a powerful middle ear implant that can undoubtedly give a huge audiometric gain. The implantation procedure requires a precise microsurgical operation that can be hampered by anatomical conditions of the petrous bone. A thorough examination of preoperative CT-scan is highly recommended in order to obtain measurements of the mastoid in axial views, and the analysis of the position of the middle fossa dura in coronal views.

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OBJECTIVES: To report a case of IgG4-related multifocal fibrosclerosis, revealed by a multifocal salivary involvement. MATERIAL AND METHODS: A 59-year-old man, with chronic pancreatitis, was managed for fibrotic involvement of the 2 submaxillary glands and the 2 parotids. Clinical, radiological, and histologic features were retrospectively studied. RESULTS: Pathological features of submandibular gland revealed a chronic sclerosing sialadenitis. Retroperitoneal fibrosis in computed tomography and high rate of IgG4 are objectived. The diagnosis of IgG4-related multifocal fibrosclerosis is evoked. The patient's state improved with corticosteroid therapy. CONCLUSION: Salivary involvement in IgG4-related multifocal fibrosclerosis must be recognized in salivary medical pathology.

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El colgajo libre microvascularizado de peroné para la reconstrucción de defectos oromandibulares ha demostrado ser una técnica excelente. Para evaluar la viabilidad de la extremidad tras la extracción del injerto, y del injerto en sí mismo, se hace necesario un correcto estudio preoperatorio. El Eco-doppler color (EDC) es un método diagnóstico no invasivo que ha demostrado ser útil en el mapeo arterial y venoso de las extremidades inferiores (MMII). Hemos realizado 21 EDC en 21 pacientes en los que se eligió el colgajo libre microvacularizado de peroné como método reconstructivo. Se diseñó un protocolo standard para evaluar la circulación de MMII e identificar los vasos perforantes septocutáneos con EDC. En todos los casos el EDC fue efectivo. A la vista del EDC no se realizó ninguna arteriografía. No se encontraron anomalías anatómicas, y tan sólo en un caso se desestimó una extremidad por arteriosclerosis significativa. Los resultados se correspondieron al 100 por ciento con los hallazgos peroperatorios y ningún paciente presentó complicación vascular o del injerto. A la vista de los resultados aconsejamos el EDC como estudio preoperatorio en los pacientes que precisan una reconstrucción con colgajo libre microvascularizado. Es una técnica no invasiva, sin morbilidad y con bajo coste que permite un mapeo vascular de MMII sin necesidad de arteriografía peroperatoria de manera rutinaria (AU)

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The synthesis and isolation of unsymmetrical porphyrazines bearing two, four, and six bis-(dimethylamino) functionalities has been achieved via the base-catalyzed cross-condensation of 1,2-dicyanobenzene 8 and bis(dimethylamino)maleonitrile 7. In addition, the benzo-fused hexaaminoporphyrazine dimer 10 was prepared from condensation of dinitrile 7 (in excess) with benzenebis(1,3-diiminopyrroline) 9. Electrochemical studies reveal that all porphyrazines may be readily oxidized. The X-ray structures of porphyrazines 2b and 5a and the cis isomer 3a are presented. The latter is the first structure of a porphyrazine having a cis-type substitution pattern. The extended pi-conjugation in dimer 10 causes a approximately 100 nm red-shifted Q-band in the electronic absorption spectrum.

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The first metalated porphyrazinediols 11 have been prepared from (L)-(+)-dimethyl tartrate via conversion into the corresponding dispoke or 2,3-dimethoxy-2,3-butanediyl protected 2,3-dihydroxymaleonitrile, Linstead macrocyclization, transmetalation, and deprotection. Their stability is very dependent on the nature of the metal ion in the cavity of the porphyrazine. Reaction of these porphyrazinediols with metallocene dichlorides led to new solitaire porphyrazines 12 while DDQ oxidation followed by trapping with diaminomaleonitrile afforded new porphyrazine dinitriles 14.

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1. A young Filipino adult who presented a third upper appendage suspended from his back since birth is reported2. The extra single limb and the mass in its axillary area are morphologically described and found to be associated with spina bifida3. Microscopic sections of the mass confirms a teratomatous growth4. Past literature is reviewed for comparison. (Summary)