Background. The incidence of head and neck cancer is increasing. To improve the survival of head and neck cancer patients, an effective program of screening and/or chemoprevention of second malignancies is essential. An analysis of the incidence, time to development, and risk factors of second malignant tumors in head and neck cancer patients can contribute to the design of effective screening and chemoprevention programs. Methods. Eight hundred, fifty‐one patients with initial squamous cell carcinoma of the larynx (n = 224), tonsils (n = 189), pyriform sinus (n = 165), oral cavity (n=129), mobile tongue (n = 72), and base of tongue (n = 72) treated from 1978 to 1990 were analyzed for the presence of a second malignancy after initial therapy. Of these 851 patients, 544 (64%) were documented smokers and 35 (4%) were nonsmokers. No smoking information was available for 272 patients. Four hundred, fifty‐four patients (53%) were consumers of alcohol and 64 patients (8%) were nondrinkers. Alcohol consumption information was not available for 333 patients. Results. One hundred, sixty‐two (19%) second head and neck carcinomas occurred in the original 851 patients. Sixty‐six patients (41 %) had synchronous tumors, and 96 patients (59%) had metachronous tumors. The probability of developing a second metachronous cancer 5‐years after undergoing treatment for the initial head and neck cancer was 22%. Borderline statistical significance was observed in the 5‐year second cancer incidence based on the site of the initial primary cancer (46% for the base of tongue, 34% for the pyriform sinus, 23% for the larynx, 18% for the oral cavity, 15% for the tonsils, and 10% for the mobile tongue). Tobacco smoking (3% for nonsmokers vs. 26% for ⩽ 20 pack‐years vs. 42% for >20 and ⩽ 40 packs/year vs. 30% for > 40 packs/year of smoking) and the consumption of alcohol (5% for nondrinkers vs. 32% for drinkers) were both statistically significant in predicting the likelihood of developing a second malignancy. Multivariate analysis revealed that the two independent variables that influenced the occurrence of a second metachronous cancer were the anatomic site of the original primary cancer and patient age. The survival rate after the second cancer was influenced significantly by the site of the second cancer (20% for a second head or neck cancer, 3% for a second esophageal cancer, and 2% for a second lung cancer). Continued smoking (20% for nonsmokers vs. 5% for smokers) and continued alcohol consumption (27% for nondrinkers vs. 6% for drinkers) also adversely influenced the survival after the occurrence of a second cancer. Conclusions. This study confirms the high rate of second cancers in patients with initial head and neck malignancies. The development of a second malignancy is almost always fatal. Screening programs and chemoprevention trials should be directed toward cancer patients with initial head and neck cancers. Only the small subset of nonsmokers and nondrinkers should be excluded from such trials.
Omission of ND based on computed tomographic scan and positron emission tomography-based complete response to chemoradiation is the most common strategy for advanced nodal disease among centers. However, neck management strategies vary among institutions, and some institutions continue advocating systematic ND before irradiation. The new treatment options and the changing epidemiology, namely docetaxel-based induction chemotherapy and human papilloma virus-related head and neck squamous cell carcinoma having better response profiles and prognosis, are adding to the nonconsensual approach. The best therapeutic index in terms of neck management remains to be defined in this evolving context.
Background Pompe disease is a rare neuromuscular disorder caused by a deficiency of a lysosomal enzyme, acid α‐glucosidase. Macroglossia is a classic clinical sign of several inherited myopathies and has also been reported to occur progressively in late‐onset Pompe disease (LOPD). Methods We describe patients with LOPD and macroglossia included in the French national Pompe disease registry. Clinical, functional, and radiological data were collected during periodic follow‐up and analyzed retrospectively. These cases were compared with 15 previously reported cases. Results Five patients, three females and two males, aged 71–88 years, were included in this study. All but one of the patients suffered from symptoms related to macroglossia before the diagnosis of Pompe disease. Three had localized tongue atrophy and one had significant localized tongue hypertrophy which led to glossectomy 10 years before diagnosis. Two patients had severe dysphagia, one of whom underwent gastrostomy for enteral nutritional support. One patient experienced the persistence of numerous sleep apneas despite nocturnal bilevel positive airway pressure (BiPAP) ventilation. All our patients had dysarthria, and two required speech therapy. Four patients had a tongue hypersignal on magnetic resonance imaging (MRI) T1 sequences. Conclusions Detection of macroglossia should be part of the clinical diagnosis and follow‐up of patients with LOPD, with a careful evaluation of its main consequences. Macroglossia can have severe functional impacts on speech, swallowing, and sleep. Whole‐body MRI with facial sections may facilitate the early diagnosis of Pompe disease with the “bright tongue sign”.
Early management in oncology is based on coordination and high-quality exchange between the various health-care partners. The present guidelines are based on a literature search with levels of evidence. Treatment waiting time can be optimized by performing assessment as early as possible (Expert opinion), to limit the interval (ideally, less than 4 weeks) between first consultation and data collection. In the first specialist consultation, diagnostic work-up should be scheduled and the data required for management should be determined (Grade B). Work-up may be conducted on a day-care basis or with conventional admission (Expert opinion). The patient's medico-social context should be taken into account from the outset, with social work involvement whenever necessary (Expert opinion). Pain and nutritional management should be planned for (Grade A) and realistic therapeutic education be provided (Expert opinion). Community-hospital teamwork for supportive care should be optimized (Expert opinion). Management should be early and multidisciplinary, to shorten delay between diagnosis and treatment initiation.
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