SUMMARY We report a rare case of Cushing’s syndrome in a 37-year-old female who initially presented with localized acinic cell carcinoma of the parotid gland. In January 2014, she underwent a right parotidectomy with facial nerve preservation and adjuvant radiotherapy. In August 2018, she presented a histologically-proven local regional relapse. The patient was considered for salvage surgery with facial nerve sacrifice and remained with no evidence of disease. One year later the patient developed pulmonary dissemination and started to gain weight and developed facial plethora and acne on the face and upper trunk. In a physical examination, the patient presented moon face, buffalo hump, acne and stage 2 hypertension. Biochemical evaluation confirmed ACTH-dependent Cushing’s syndrome. IHC for ACTH in the lung biopsy revealed strong positive staining for ACTH confirming a diagnosis of ectopic ACTH secretion by a metastatic parotid acinic cell carcinoma. Ketoconazole (600 mg/d) was started to treat the CS. In addition, as chemotherapy was initiated to treat the metastatic disease. After the fifth cycle of chemotherapy, ketoconazole was suspended and the patient remained in remission of CS for four months, when CS recurred. A unique feature of this case is related to the clinical CS relapse associated with disease progression, which needed prompt treatment with ketoconazole, resulting in a significant improvement in the patient’s condition. Although rare, should be attentive for possible CS features in patients with high-grade salivary gland carcinomas, since the diagnosis of ectopic secretion of ACTH may significantly impact their management and outcomes.

Abstract Introduction Coronavirus disease 2019 (COVID-19) is an acute infection caused by the new coronavirus (SARS-CoV-2) and it is highly transmissible, especially through respiratory droplets. To prepare the health system for the care of these patients also led to a restriction in the activity of several medical specialties. Physicians who work with patients affected by diseases of the head and neck region constitute one of the populations most vulnerable to COVID-19 and also most affected by the interruption of their professional activities. Objective The aim of the present study was to assess the impact of the COVID-19 pandemic on the practice of head and neck surgeons and otorhinolaryngologists in Brazil. Methods An anonymous online survey of voluntary participation was applied, containing 30 questions regarding demographic aspects, availability of personal protective equipment (PPE), and impact on the routine of head and neck surgeons and otorhinolaryngologists, as well as clinical oncologists and radiation oncologists who work with head and neck diseases. Results Seven hundred and twenty-nine answers were received in a period of 4 days, ∼ 40 days after the 1st confirmed case in Brazil. With professionals working in public and private services, there was a high level of concerns with the disease and its consequences, limited availability of PPE and a significant decrease in the volume of specialized medical care. Conclusion The study demonstrated a direct impact of the COVID-19 pandemic on the clinical practice of specialties related to the treatment of patients with diseases of the head and neck region already in the beginning of the illness management in Brazil.

RESUMO Introdução: O tratamento baseado em quimirradioterapia do Carcinoma Espinocelular de Orofaringe (CECOF) apresenta bons resultados no controle locorregional da doença com boas taxas de resposta clínica e patológica completas especialmente no pescoço. Objetivo: Determinar se a resposta patológica completa após quimiorradioterapia estárelacionada aos prognósticos dos pacientes com CECOF. Método: Os dados foram obtidos de maneira prospectiva da revisão de prontuários de pacientes com CECOF N2 e N3 submetidos a esvaziamento cervical planejado após quimiorradioterapia. Resultados: Um total de 19 pacientes foram avaliados. Metade dos indivíduos apresentou resposta patológica completa no pescoço. Recidiva à distância ou locorregional ocorreu em aproximadamente 42% dos pacientes e 26% deles morreram. A análise estatística demonstrou uma associação entre resposta patológica completa e menor taxa de recidiva (77,8% vs.20,8%; p = 0,017) e maior sobrevivência global (88,9% vs.23,3%; p = 0,049). Conclusão: A presença de resposta patológica completa após quimiorradioterapia influencia positivamente no prognóstico de pacientes com carcinoma espinocelular de orofaringe.

ABSTRACT INTRODUCTION: Chemoradiotherapy for squamous cell carcinoma of the oropharynx (SCCO) provides good results for locoregional disease control, with high rates of complete clinical and pathologic responses, mainly in the neck. OBJECTIVE: To determine whether complete pathologic response after chemoradiotherapy is related to the prognosis of patients with SCCO. METHODS: Data were prospectively extracted from clinical records of N2 and N3 SCCO patients submitted to a planned neck dissection after chemoradiotherapy. RESULTS: A total of 19 patients were evaluated. Half of patients obtained complete pathologic response in the neck. Distant or locoregional recurrence occurred in approximately 42% of patients, and 26% died. Statistical analysis showed an association between complete pathologic response and lower disease recurrence rate (77.8% vs. 20.8%; p = 0.017) and greater overall survival (88.9% vs. 23.3%;p = 0.049). CONCLUSION: The presence of a complete pathologic response after chemoradiotherapy positively influences the prognosis of patients with SCCO.

ResumoO câncer de pulmão é a principal causa de mortes por câncer no mundo. Recentemente, novas estratégias promissoras de tratamento foram criadas a partir do desenvolvimento de terapias de alvo molecular, particularmente aquelas que interferem em vias de transdução de sinais em células neoplásicas. Uma das vias de transdução de sinais mais estudadas é aquela ativada a partir do EGFR, que leva a perda do controle da proliferação celular, aumento da angiogênese celular e aumento da capacidade de invasão celular. Mutações ativadoras no EGFR (deleções no éxon 19 e mutação L858R no éxon 21), primeiramente descritas em 2004, foram detectadas em aproximadamente 10% dos pacientes com carcinoma de pulmão de células não pequenas (CPCNP) não escamoso em países ocidentais e são os fatores preditivos mais importantes de resposta aos tyrosine-kinase inhibitors (inibidores de tirosina quinase) do EGFR (EGFR-TKIs). Estudos de tratamento de primeira linha com esses EGFR-TKIs (gefitinibe, erlotinibe e afatinibe) em pacientes sem tratamento sistêmico prévio, em comparação com regimes baseados em platinas, têm demonstrado que os EGFR-TKIs resultam em ganhos em sobrevida livre de progressão e taxas globais de resposta, embora somente em pacientes cujos tumores alberguem mutações ativadoras no EGFR. Ensaios clínicos também mostraram a efetividade dos EGFR-TKIs como tratamentos de segunda e terceira linha de CPCNP avançado. Neste artigo, revisamos os principais aspectos da ativação da via do EGFR em CPCNP, reforçamos a importância da identificação correta das mutações ativadoras no EGFR e discutimos os principais resultados do tratamento do CPCNP com EGFR-TKIs.

AbstractLung cancer is the leading cause of cancer-related deaths worldwide. Promising new therapies have recently emerged from the development of molecular targeted drugs; particularly promising are those blocking the signal transduction machinery of cancer cells. One of the most widely studied cell signaling pathways is that of EGFR, which leads to uncontrolled cell proliferation, increased cell angiogenesis, and greater cell invasiveness. Activating mutations in the EGFR gene (deletions in exon 19 and mutation L858R in exon 21), first described in 2004, have been detected in approximately 10% of all non-squamous non-small cell lung cancer (NSCLC) patients in Western countries and are the most important predictors of a response to EGFR tyrosine-kinase inhibitors (EGFR-TKIs). Studies of the EGFR-TKIs gefitinib, erlotinib, and afatinib, in comparison with platinum-based regimens, as first-line treatments in chemotherapy-naïve patients have shown that the EGFR-TKIs produce gains in progression-free survival and overall response rates, although only in patients whose tumors harbor activating mutations in the EGFR gene. Clinical trials have also shown EGFR-TKIs to be effective as second- and third-line therapies in advanced NSCLC. Here, we review the main aspects of EGFR pathway activation in NSCLC, underscore the importance of correctly identifying activating mutations in the EGFR gene, and discuss the main outcomes of EGFR-TKI treatment in NSCLC.